André Cartell schleper@terra.com

CASOS RELEVANTES DE

DERMATOPATOLOGIA

O QUE PODE CAIR NO TED a intenção não é adivinhar o que vai cair no

TED, mas dentro de diagnósticos difíceis prevenir o residente para algumas possibilidades interessantes

durante o curso foram lembrados algumas possibilidades, pois geram fotos elegantes – líquen plano, cilindroma, CBC, micoses, etc

o centerfold dos archives é sempre uma boa fonte de estudo

se eu acertar 3 questões, tem de pagar uma janta!

O QUE PODE CAIR NO TED1. porokeratosis ptychotropica

2. síndrome de Bannayan-Riley-Ruvalcaba

3. prototecose

4. amebíase

5. microscopia confocal de ocronose

6. dermatoscopia de acantoma de células claras

7. complexo de Carney (LAMB/NAME)

8. dermatopatia fibrosante nefrogêncica

9. variantes de MF – Woringer-Kollop, granulomatous slack skin e mucinose folicular

10.ATCL – flower cell

O QUE PODE CAIR NO TED11.síndrome de Birt-Hogg-Dubbé

12.síndrome de Brooke-Spiegler

13.hemangioma glomeruloide e síndrome de POEMS

14.estrongiloidíase

15.doença de Flegel

16.pyostomatitis vegetans

17.tatuagem por amálgama

18.síndrome de Sneddon

19.malacoplaquia e von Kossa

20.granuloma secundário a preenchedor dérmico

CASOS LEGAIS NA CLÍNICA COM FOTOS RUINS NA PATOLOGIA – alguns exemplos ICTIOSES – Netherton, Sjögren–Larsson, eritroqueratodermia

variabilis e pitiríase rotunda HIPERCERATOSES PALMO-PLANTARES – todas BOLHOSAS – epidermólises bolhosas genéticas, penfigoide

gestacional e penfigoide cicatricial ECZEMAS & DESCAMATIVAS – pitiríase alba, síndrome de

Gianotti-Crosti, eritrodermia INTERFACE – liquen striatus, keratosis lichenoide chronica;

atrofodermia, dermatomiosite VASCULOPÁTICAS – eritemas figurados, EPS, VLINs –

perniose, DPP, PUPPP; pioderma gangrenoso e Behçet PROLIFERATIVAS – acantose nigricante X PRCGC FANTASMAS – vitiligo, nevo de Becker, anetodermia,

hipomelanose de Ito, Ehler-Danlos, dermatocalázio

POROKERATOSIS PTYCHOTROPICAClin Exp Dermatol. 2009 Dec;34(8):e895-7.Porokeratosis ptychotropica: a lesser-known variant. Tallon B, Blumental G, Bhawan J.Abstract – We report a case of the rare porokeratosis variant porokeratosis ptychotropica (PP). A circumferential perianal plaque and the characteristic histology of multiple cornoid lamellae with underlying dermal amyloid deposition were seen. Amyloid deposition was seen in the biopsied intertriginous area of the plaque only, which, in concordance with other cutaneous amyloid deposition disorders, may suggest a role for friction in the pathogenesis of this histological finding. We review the literature on PP and summarize the poor response seen to treatments.

POROKERATOSIS PTYCHOTROPICAJ Cutan Pathol. 2010 Jul;37(7):802-7Verrucous porokeratosis of the gluteal cleft (porokeratosis ptychotropica): a rare disorder easily misdiagnosed.Takiguchi RH, White KP, White CR Jr, Simpson EL.Abstract – porokeratosis represents a heterogeneous group of disorders characterized clinically by a distinctive ridge-like border and histologically by cornoid lamellae. A verrucous variant of porokeratosis involving the gluteal cleft has been recently described. We present 5 new cases and review the current literature to highlight the clinical and histopathologic features of this disorder. Descriptive terms including hyperkeratotic porokeratosis, genitogluteal porokeratosis, porokeratoma, follicular porokeratosis, and porokeratosis ptychotropica have all been used to describe this verrucous variant of porokeratosis involving the gluteal cleft. To avoid further confusion, we propose a consolidation of terminologies and suggest verrucous porokeratosis be added to the commonly described variants of porokeratosis.

1-POROKERATOSIS PTYCHOTROPICAJ Am Acad Dermatol. 2009 Mar;60(3):501-3. Porokeratosis ptychotropica: a clinically distinct variant of porokeratosis.McGuigan K, Shurman D, Campanelli C, Lee JB.Abstract – porokeratosis represents a spectrum of clinical disease. Multiple variants have been described including porokeratosis ptychotropica, a rare subtype. The clinical presentation of porokeratosis ptychotropica frequently resembles an inflammatory perianal disease. We report a patient with porokeratosis ptychotropica with coexistent disseminated superficial actinic porokeratosis. We review the current literature on porokeratosis ptychotropica including the clinical presentation, histopathology, cause, and pathogenesis of this rare variant of porokeratosis.

2-S. DE BANNAYAN-RILEY-RUVALCABArare hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomasthe disease is inherited in an autosomal dominant form, but sporadic cases have been reportedbelongs to a family of hamartomatous polyposis syndromes, which also includes Peutz-Jeghers syndrome, juvenile polyposis and Cowden syndromemutation of the PTEN gene underlies this syndrome, as well as Cowden syndrome, Proteus syndrome, and Proteus-like syndrome; collectively, these four syndromes are referred to as PTEN Hamartoma-Tumor Syndromes (PHTS)

2-S. DE BANNAYAN-RILEY-RUVALCABAJ Pediatr Surg. 2006 Sep;41(9):1601-3.Cutaneous lipoma in children: 5 cases with Bannayan-Riley-Ruvalcaba syndrome.Buisson P, et al.Abstract – cutaneous lipoma is rare in children, but it can be part of a syndrome such as the Bannayan-Riley-Ruvalcaba syndrome (BRRS). The BRRS is a dominant autosomal disorder characterized by cutaneous lipomas, macrocephaly, intestinal polyps, and developmental delay associated with PTEN gene mutations. This syndrome is thought to represent a pediatric form of the Cowden syndrome, characterized among other features by an increased risk of cancer. We report 5 cases of BRRS, all diagnosed in children with lipoma and macrocephaly. Children presenting with lipomas need a complete physical examination to look for other signs of BRRS, because they may need further follow-up for tumor screening in adulthood.

2-S. DE BANNAYAN-RILEY-RUVALCABAGenet Med. 2009 Oct;11(10):687-94.PTEN hamartoma tumor syndrome: an overview. Hobert JA, Eng C.Abstract – PTEN hamartoma tumor syndrome (PHTS) encompasses four major clinically distinct syndromes associated with germline mutations in the tumor suppressor PTEN. These allelic disorders, Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome, and Proteus-like syndrome are associated with unregulated cellular proliferation leading to the formation of hamartomas. Thus far, an increased risk of malignancy has only been documented in Cowden syndrome; however, current recommendations advise that all individuals with PTEN hamartoma tumor syndrome follow the cancer surveillance strategies suggested for Cowden syndrome until further data indicate otherwise. Because any individual phenotypic feature of Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome are frequently present in the general population, many individuals often go undiagnosed and consequently do not benefit from available cancer surveillance strategies. Therefore, it is critical for clinicians to recognize the phenotypic features associated with these syndromes to accurately diagnose and provide preventative care. This overview details the clinical description of the PTEN hamartoma tumor syndrome and associated disorders, their diagnosis and molecular/genetic testing, as well as differential diagnosis for assessment of other hamartoma-associated syndromes.

2-S. DE BANNAYAN-RILEY-RUVALCABAHum Mutat. 2003 Sep;22(3):183-98.PTEN: one gene, many syndromes. Eng C.Abstract - PTEN, on 10q23.3, encodes a major lipid phosphatase which signals down the phosphoinositol-3-kinase/Akt pathway and effects G1 cell cycle arrest and apoptosis. Germline PTEN mutations have been found to occur in 80% of classic Cowden syndrome (CS), 60% of Bannayan-Riley-Ruvalcaba syndrome (BRRS), up to 20% of Proteus syndrome (PS), and approximately 50% of a Proteus-like syndrome (PSL). CS is a heritable multiple hamartoma syndrome with a high risk of breast, thyroid, and endometrial carcinomas. BRRS is a congenital autosomal dominant disorder characterized by megencephaly, developmental delay, lipomatosis, and speckled penis. PS and PSL had never been associated with risk of malignancy. Finding germline PTEN mutations in patients with BRRS, PS, and PSL suggests equivalent risks of developing malignancy as in CS with implications for medical management. The mutational spectra of CS and BRRS overlap, with many of the mutations occurring in exons 5, 7, and 8. Genotype-phenotype association analyses have revealed that the presence of germline PTEN mutations is associated with breast tumor development, and that mutations occurring within and 5' of the phosphatase motif were associated with multi-organ involvement. Pooled analysis of PTEN mutation series of CS and BRRS occurring in the last five years reveals that 65% of CS-associated mutations occur in the first five exons encoding the phosphatase domain and the promoter region, while 60% of BRRS-associated mutations occur in the 3' four exons encoding mainly the C2 domain. Somatic PTEN mutations occur with a wide distribution of frequencies in sporadic primary tumors, with the highest frequencies in endometrial carcinomas and glioblastoma multiform. Several mechanisms of PTEN inactivation occur in primary malignancies derived from different tissues, but a favored mechanism appears to occur in a tissue-specific manner. Inappropriate subcellular compartmentalization and increased/decreased proteosome degradation may be two novel mechanisms of PTEN inactivation. Further functional work could reveal more effective means of molecular-directed therapy and prevention.

aspectos geraisagente: Chlorella (alga verde), P. wickerhamii

1. microorganismo ubíquo

2. infecção por inoculação ou via ferimento pré-existente

3. não ocorre transmissão pessoa-a-pessoa

4. baixa virulência

5. imunocomprometidos – formas sistêmicas: pele, sangue, peritônio, TGI, fígado e meninges

6. menos de 100 casos publicados nos EUA desde 1964

7. endosporos arredondados com 6 a 10 micrômetros, intracitoplasmáticos ou livres, padrão moruliforme

8. granulomas supurativos clássicos

3-prototecose

3-PROTOTECOSEMed Mycol. 2004 Apr;42(2):95-106.Protothecosis.Leimann BC, Monteiro PC, Lazéra M, Candanoza ER, Wanke B.Mycology Service, Evandro Chagas Institute of Clinical Research, IPEC/FIOCRUZ, Rio de Janeiro, Brazil. leimann@centroin.com.brAbstractProtothecosis is an infection caused by achlorophyllic algae of the genus Prototheca which rarely affects humans. Some 100 cases have been described in the medical literature, the majority caused by the species P. wickerhamii. The skin is the organ most frequently involved. Diagnosis is performed by isolation of the microorganism in culture or by histopathology. The ideal treatment has not been defined, with amphotericin B and the azoles having been employed. Surgical excision is recommended for small, localized lesions. We describe a case of cutaneous protothecosis on the right fourth finger of a female patient 59 years old with no underlying disease. Administration of itraconazole 400 mg/day for 6 weeks failed to produce an adequate clinical response. Treatment was then changed to fluconazole 200 mg/day, with regression of the lesion.

3-PROTOTECOSEClin Microbiol Rev. 2007 Apr;20(2):230-42. Lass-Flörl C, Mayr A.Human protothecosis.Abstract – human protothecosis is a rare infection caused by members of the genus Prototheca. Prototheca species are generally considered to be achlorophyllic algae and are ubiquitous in nature. The occurrence of protothecosis can be local or disseminated and acute or chronic, with the latter being more common. Diseases have been classified as (i) cutaneous lesions, (ii) olecranon bursitis, or (iii) disseminated or systemic manifestations. Infections can occur in both immunocompetent and immunosuppressed patients, although more severe and disseminated infections tend to occur in immunocompromised individuals. Prototheca wickerhamii and Prototheca zopfii have been associated with human disease. Usually, treatment involves medical and surgical approaches; treatment failure is not uncommon. Antifungals such as ketoconazole, itraconazole, fluconazole, and amphotericin B are the most commonly used drugs to date. Among them, amphotericin B displays the best activity against Prototheca spp. Diagnosis is largely made upon detection of characteristic structures observed on histopathologic examination of tissue.

4-AMEBÍASEArch Dermatol. 2008 Oct;144(10):1369-72.Cutaneous amebiasis in pediatrics.Magaña ML, Fernández-Díez J, Magaña M.Abstract – BACKGROUND: cutaneous amebiasis (CA), which is still a health problem in developing countries, is important to diagnose based on its clinical and histopathologic features. OBSERVATIONS: Retrospective medical record review of 26 patients with CA (22 adults and 4 children) treated from 1955 to 2005 was performed. CONCLUSIONS: Cutaneous amebiasis always presents with painful ulcers. The ulcers are laden with amebae, which are relatively easy to see microscopically with routine stains. Erythrophagocytosis is an unequivocal sign of CA. Amebae reach the skin via 2 mechanisms: direct and indirect. Amebae are able to reach the skin if there is a laceration (port of entry) and if conditions in the patient are favorable. Amebae are able to destroy tissues by means of their physical activity, phagocytosis, enzymes, secretagogues, and other molecules.

4-AMEBÍASEAm J Dermatopathol. 2004 Aug;26(4):280-4.Histopathology of cutaneous amebiasis.Magaña M, Magaña ML, Alcántara A, Pérez-Martín MA.Abstract – cutaneous amebiasis (CA) is the manifestation in the skin and underlying soft tissues of the pathogenic properties of Entamoeba histolytica, which may be the only expression of the infection or may be associated with disease in other organs. So far, there have been only isolated case reports on this disease. We herein report the histopathologic findings on a series of seven cases, six adults and one child, of CA. The most common findings include ulcers, areas of necrosis, mixed inflammatory infiltrates, and the presence of trophozoites, the invasive form of the parasite. CA is a very rare and severe disease, it is progressive and destructive; erythrophagocytosis, a microscopic sign of pathogenicity, is always seen in CA.

acantamoeba

5-OCRONOSEochronosis is the syndrome caused by the accumulation of homogentisic acid in connective tissuesthe phenomenon was first described by Rudolf Virchow in 1865was named after the yellowish (ocher-like) discoloration of the tissue seen on microscopic examination. However, macroscopically the affected tissues appear bluish grey because of a light scattering phenomenon known as the Tyndall effectis most often associated with alkaptonuria but can occur from exogenous administration of phenol complexes like hydroquinonealkaptonuria is often asymptomatic, but the sclera of the eyes may be pigmented (often only at a later age), and the skin may be darkened in sun-exposed areas and around sweat glandssweat may be coloured brownurine may turn brown if collected and left exposed to open air, especially when left standing for a period of timekidney stones and stone formation in the prostate are common and may occur in more than a quarter of cases

5-OCRONOSEBr J Neurosurg. 2008 Dec;22(6):805-7.Alkaptonuria presenting with ochronotic spondyloarthropathy.Al-Mahfoudh R, Clark S, Buxton N.Abstract – alkaptonuria is a rare autosomal recessive metabolic disease that leads to the deposition of homogentisic acid. Ochronotic arthropathy is the articular manifestation of alkaptonuria with the most common clinical feature being severe spondyloarthropathy. We present the case of a 58-year-old woman with back pain. Radiographs and magnetic resonance imaging (MRI) revealed characteristic features of ochronotic spondyloarthropathy. The literature regarding management of alkaptonuria is reviewed.

5-OCRONOSEArch Dermatol. 2010 Sep;146(9):1021-5. Dermoscopic and reflectance confocal microscopic features of exogenous ochronosis. Gil I, Segura S, Martínez-Escala E Abstract – exogenous ochronosis presents as an acquired asymptomatic hyperpigmentation on photoexposed areas, predominantly over bony prominences, and is caused by the topical application of several skin-lightening agents. OBSERVATIONS: We describe a 63-year-old Hispanic woman who developed exogenous ochronosis lesions on her face after using topical bleaching creams containing hydroquinone, 2% to 3%, and oxybenzone, 2%, for several years. Dermoscopy revealed irregular brown-gray globular, annular, and arciform structures that corresponded to focal deposition of ochronotic pigment on the dermis. These deposits correlated with multiple banana-shaped nonrefractile structures seen using reflectance confocal microscopy. Histopathologic sections revealed the deposition of a banana-shaped, yellow to brown material in the papillary and middle dermis. Ultrastructural examination revealed an amorphous electron-dense material mostly located in the core of elastic fibers and also in smaller amounts in the interstitium with prominent degenerative changes in the elastic fibers. A good correlation was observed between the results of both noninvasive techniques and the diagnostic histologic features of this condition. CONCLUSIONS: We characterized by means of dermoscopy, reflectance confocal microscopy, and electronic microscopy a case of exogenous ochronosis. To our knowledge, this is the first description of reflectance confocal microscopic findings in this condition. Dermoscopy and reflectance confocal microscopy are proved to be useful noninvasive techniques for the diagnosis of this pigmentary disorder.

OCRONOSE

6-acantoma de células clarasachados histopatológicos acantoma de células claras de Degos

1. acantose com proliferação bem demarcada de células claras em relação ao epiderma normal

2. poupa o epitélio anexial

3. pode haver fusão dos cones interpapilares; predominantemente endofítico

4. pode haver erosão e crosta

5. neutrófilos infiltrando o epiderma; pode haver espongiose

6. as células claras são PAS positivas e diastáse sensíveis

aspectos clínicos MMII de paciente de meia idade ou velhos; varizes lesão eritematosa bem demarcada com colarette descamativo associação com psoríase (reacional, inflamatória)R. Degos, J. Delort, J. Civatte, A. Poires Baptista: Tumeur épidermique d'aspect particulier:

acanthome à cellules claires. Annales de dermat.et syphilig., Paris, 1962, 9: 361-371

6-ACANTOMA DE CÉLULAS CLARAShttp://dermoscopymadesimple.blogspot.com/2010/09/clear-cell-acanthoma.htmlhttp://www.dermoscopyatlas.com/index.cfm .

7-COMPLEXO DE CARNEYCarney complex (also known as "LAMB syndrome," and "NAME syndrome") is an autosomal dominant condition comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity (Carney et al., 1985; McCarthy et al., 1986)LAMB: lentigines, atrial myxomas, mucocutaneous myxomas, and blue neviNAME: nevi, atrial myxoma, myxoid neurofibroma, and ephelides it must be differentiated from Carney triad – coexistence of several neoplasms, including: gastric epithelioid leiomyosarcoma, pulmonary chondroma, and extra-adrenal paraganglioma approximately 7% of all cardiac myxomas are associated with Carney complex the majority of cases are caused by mutations in the PRKAR1-α gene on chromosome 17q24, which has been suggested to function as a tumor-suppressor gene the spotty skin pigmentation and lentigines occur most commonly on the face, especially on the lips, eyelids, conjunctiva, and oral mucosa (McCarthy et al., 1986)cardiac myxomas may lead to embolic strokes and heart failure (Reynen, 1995) and may present with fever, joint pain, shortness of breath, diastolic rumble, and tumor plop; myxomas may also occur in the skin and breastendocrine tumors may manifest as disorders such as Cushing syndrome http://emedicine.medscape.com/article/160000-overview

7-COMPLEXO DE CARNEY

8-DERMATOPATIA FIBROSANTE NEFROGÊNICA

Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy is a rare and serious syndrome that involves fibrosis of skin, joints, eyes, and internal organs. Its cause is not fully understood. However, there is much evidence to suggest that it is associated with exposure to gadolinium (which is frequently used as a contrast agent for MRIs) in patients with severe kidney failureepidemiological studies suggest that the incidence of NSF is unrelated to gender, race, or age and it is not thought to have a genetic basis.patients develop large areas of hardened skin with fibrotic nodules and plaques. NSF may also cause joint contractures resulting in joint pain and limitation in range of motion. In its most severe form, NSF may cause severe systemic fibrosis affecting internal organs including the lungs, heart and liverat the microscopic level, NSF resembles scleromyxedema – proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin; recent case reports have described the presence of sclerotic bodies (also known as elastocollagenous balls) in skin biopsies from NSF patients. While not universally present, this finding is believed to be unique to NSFmost patients with NSF have undergone hemodialysis for renal failure, some have never undergone dialysis and others have received only peritoneal dialysis. Many patients have taken immunosuppressive medications and have other diseases, such as hepatitis C. Four of the seven gadolinium contrast agents approved by the FDA have been principally implicated in NSF, including Omniscan, Multihance, Magnevist, and OptiMARK.the first cases of NSF were identified in 1997, but NSF was first described as an independent disease entity in 2000 – Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE (2000). "Scleromyxoedema-like cutaneous diseases in renal-dialysis patients". Lancet 356 (9234): 1000–1

8-DERMATOPATIA FIBROSANTE NEFROGÊNICA

Am Fam Physician. 2009 Oct 1;80(7):711-4. Schlaudecker JD, Bernheisel CR.Gadolinium-associated nephrogenic systemic fibrosis.Abstract – Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadolinium-based contrast agents used in magnetic resonance imaging. Because of this relationship between nephrogenic systemic fibrosis and gadolinium-based contrast agents, the U.S. FDA currently warns against using gadolinium-based contrast agents in patients with a glomerular filtration rate less than 30 mL per minute per 1.73 m2, or any acute renal insufficiency related to the hepatorenal syndrome or perioperative liver transplantation. There have been reports of nephrogenic systemic fibrosis developing in patients not exposed to gadolinium-based contrast agents, but most patients have the triad of gadolinium exposure through contrast-enhanced magnetic resonance imaging, renal failure, and a proinflammatory state, such as recent surgery, endovascular injury, or sepsis. Development of nephrogenic systemic fibrosis among patients with severe renal insufficiency following exposure to gadolinium-based contrast agents is approximately 4 percent, and mortality can approach 31 percent. The mechanism for nephrogenic systemic fibrosis is unclear, and current treatments are disappointing. Prevention with hemodialysis immediately following gadolinium-based contrast agents has been recommended, but no studies have shown this to be effective. Because of the large number of patients with clinically silent renal impairment and the serious consequences of nephrogenic systemic fibrosis related to gadolinium exposure, physicians should use alternative imaging modalities for patients who are at risk.

8-DERMATOPATIA FIBROSANTE NEFROGÊNICA

J Am Acad Dermatol. 2010 Sep;63(3):389-99.Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: a single-center experience.Lemy AA, del Marmol V, Kolivras A, High WA, Matos C, Laporte M, Nortier JL.Abstract – nephrogenic systemic fibrosis (NSF) is a fibrotic disorder occurring in patients with renal dysfunction. Exposure to gadolinium (Gd)-based contrast agents (GBCAs) during renal impairment is associated with development of NSF. METHODS: A cross-referenced search of kidney transplantation and radiology databases at a single institution revealed the prevalence of NSF in the transplant population. Clinical records and skin biopsy specimens from 6 patients with kidney transplant given a diagnosis of NSF were reviewed to identify contributing factors. RESULTS: Between January 1999 and December 2006, NSF was diagnosed in 6 of 705 patients with kidney transplant (0.9%). Renal function was impaired in all patients. Of 33 patients with kidney transplant exposed to GBCAs, 5 (15.2%) developed NSF. Disease onset ranged from 7 days to 11 months after exposure to GBCAs. All 5 patients exposed to GBCAs who developed NSF were also treated with a beta-blocker and clinical improvement was observed with discontinuation. The sixth case NSF appeared unrelated to Gd, without a known exposure, and testing of tissue via mass spectrometry revealed no Gd. Symptoms of NSF in this patient disappeared after administration of darbepoetin was switched from subcutaneous to intravenous injection. One patient with NSF who manifested the highest Gd level in tissue died 22 months after disease onset. LIMITATIONS: The study represents the retrospective experience of only a single center. CONCLUSIONS: NSF can develop in kidney transplant recipients with altered graft function. In these patients, exposure to GBCAs appears associated with development of NSF. The role of beta-blockers in the course of the disease merits further investigation

9-MF variantes – Woringer-KoloppSemin Cutan Med Surg. 2000 Jun;19(2):91-9.Mycosis fungoides: classic disease and variant presentations.Howard MS, Smoller BR.Abstract – mycosis fungoides is a peripheral non-Hodgkin's T-cell neoplastic process, representing the most common type of primary cutaneous malignant lymphoma. Neoplastic lesions classically show skin predilection and characteristic clinical and histologic features in patch, plaque, and tumor stages. In addition, several clinicopathologic variants of mycosis fungoides have been delineated, including poikiloderma atrophicans vasculare (parapsoriasis variegata), Sézary syndrome, granulomatous mycosis fungoides, hypopigmented mycosis fungoides, folliculocentric mycosis fungoides, syringotropic mycosis fungoides, and Woringer Kolopp disease. We will review the salient features of patch, plaque, and tumor stage mycosis fungoides in this article and follow with a discussion of these variant clinicopathologic presentations and of therapeutic modalities.

9-MF variantes – Woringer-KoloppAm J Dermatopathol. 2005 Feb;27(1):68-85. Steffen CKetron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis.Abstract – in this historical review I will synopsize the original articles by Lloyd W. Ketron and M.H. Goodman who described Ketron-Goodman disease, by Frederic Woringer and Pierre Kolopp who described Woringer-Kolopp disease, and by Otto Braun-Falco and colleagues who described pagetoid reticulosis. In their publications, each of these authors reported on one patient. I will review the clinical picture of the three patients, their histopathology, and the pathogenesis of each disease as suggested by the above authors. Then the views of others that have written on the subject recently will be reviewed particularly as to their conception of the diseases. Publications that describe the histopathology of the patch (early) stage of mycosis fungoides will be redacted to compare it to the histopathology of Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis. I will discuss whether any or all of them are diseases sui generis, whether they are one, two, or three entities, or whether any or all are but forms of mycosis fungoides.

Segundo caso observado por Frédéric Woringer em 1958 – Kollop (urologista) em 1938 mandou uma biópsia do antebraço E de um rapaz de 13 anos pensando em tuberculose cutânea.

Woringer-Kollop – CD3+

Woringer-Kollop – CD3+

Ketron-Goodman

Ketron-Goodman

9-Granulomatous slack skinDermatology. 1998;196(4):382-91. van Haselen CWGranulomatous slack skin. Report of three patients with an updated review of the literature.Abstract - PURPOSE: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin's disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. PATIENTS AND METHODS: The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. RESULTS: A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor beta genes were found in the 2 female patients; the male patient could not be tested. CONCLUSION: GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.

9-Granulomatous slack skinActa Derm Venereol. 2001 Jan-Feb;81(1):42-4. Topar GGranulomatous slack skin: a distinct disorder or a variant of mycosis fungoides?About 75% of cutaneous lymphomas belong to the group ofT-cell lymphomas . Mycosis fungoides is the most commonentity in this group. Granulomatous slack skin is a rare form ofcutaneous T-cell lymphoma closely related to mycosis fungoides.We present here a patient with areas of lax skin forseveral years who developed a generalized erythroderma withassociated immunoactivation and a deterioration in his generalcondition. This report discusses clinically and histologically thedifferential diagnoses, namely granulomatous slack skin andgranulomatous mycosis fungoides, and suggests that these2 disorders are only variants in the broad spectrum of a singledisease.

An. Bras. Dermatol. vol.82 no.5 Rio de Janeiro Sept./Oct. 2007

9-MUCINOSE FOLICULARAn. Bras. Dermatol. vol.77 no.6 Rio de Janeiro Nov./Dec. 2002 Mucinose Folicular: revisão da literatura e relato de um casoEm 1957, Pinkus3 descreveu uma nova entidade dermatológica, sob a denominação de alopecia mucinosa, apresentando a observação de seis pacientes portadores da nova enfermidade. Porém, como a alopecia só é clinicamente evidente quando se encontram acometidas áreas cutâneas com pêlo terminal, parece ser mais adequada a designação MuF, proposta em 1959 por Jablonska Chorzelski e Lancucky e adotada pela maioria dos autores que se ocuparam do assunto. A MuF tem sido observada em todas as raças, em todas as idades e igualmente em ambos os sexos. Sua causa permanece desconhecida mas a tendência atual é considerá-la um padrão de reação do epitélio folicular a diversas noxasA classificação da MuF foi ampliada recentemente para incluir três formas:1.primária, de curta evolução 2. primária, de curso prolongado 3. secundária, associada a outros processosA terceira variedade, que habitualmente incide entre os 40 e os 70 anos de idade, apresenta-se como múltiplas placas generalizadas, infiltradas, associadas a linfomas de células T, especialmente micose fungóide – 11 a 32%

9-MUCINOSE FOLICULARaspectos histopatológicos1. infiltrado folicular, perifolicular ou perivascular,

predominantemente linfocitário, mas pode haver eosinófilos ou plasmócitos

2. na micose fungoide, geralmente não há infiltrado linfocitário folicular relevante

3. acomete vários folículos ao mesmo tempo, especialmente na metade superior

4. mucinose intrafolicular, semelhante a edema, mas cora para mucina (PAS+AB, ferro coloidal)

MF hipopigmentada pediátrica

10-ATLL – LINFOMA DE CÉLULAS TInt J Dermatol. 2008 Apr;47(4):359-62. Pezeshkpoor FSpecific cutaneous manifestations in adult T-cell leukemia/lymphoma.Abstract – BACKGROUND: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive malignancy which may occur in individuals infected with human T-cell lymphotropic virus type-I (HTLV-I). HTLV-I is endemic in Khorasan, with a frequency of 2.3% in the general population. As specific cutaneous manifestations of lymphoma may occur in a significant number of patients, we studied these manifestations in ATLL patients admitted to the Hematology and Dermatology Departments of Ghaem Hospital, Mashhad, Iran, during 1995-2004. METHODS: In this descriptive study, demographic and clinical information was obtained from 23 patients suffering from ATLL with specific cutaneous lesions (atypical lymphocytes on histopathology of cutaneous lesions), and was analyzed statistically. RESULTS: Of the 23 patients, 11 were male and 12 were female. The mean age was 48.17 +/- 14.1 years. The birth place in over 85% of cases was the north of Khorasan. The most common type of specific skin lesion was a maculopapular eruption (11 cases; 47.8%); papular lesions were seen in four cases (17.4%). Other lesions included plaques, ichthyosis-like lesions, erythroderma, tumors, papules, and nodular lesions. In most patients (56.5%), the skin lesions were generalized. CONCLUSION: The most common type of specific skin lesion in ATLL was maculopapular eruption, especially with a generalized distribution. Other types of specific skin lesion, in order of frequency, were papules, plaques, ichthyosis-like skin lesions, nodules, tumors, and erythroderma.

10-ATLL – LINFOMA DE CÉLULAS TInt J Dermatol. 2010 Oct;49(10):1099-110. Cutaneous manifestations associated with HTLV-1 infection.Bittencourt AL. Laboratory of Pathology, Complexo Hospitalar Universitário Prof. Edgard Santos, Federal University of Bahia, Salvador, Bahia, Brazil. achilea@uol.com.brAbstract - Skin lesions are frequent in HTLV-1 infection and may constitute an alert for the diagnosis of this condition. The most severe skin diseases related to this virus are adult T-cell leukemia/lymphoma (ATLL), an aggressive form of leukemia/lymphoma that fails to respond to chemotherapy, and infective dermatitis associated with HTLV-1 (IDH), a severe and recurrent form of eczema occurring in childhood. ATLL affects the skin in 43-72% of cases. In this review, the clinical, histopathological and immunohistochemical aspects of ATLL and IDH will be discussed, as well as the differential diagnoses, giving particular focus to the primary cutaneous ATLL. IDH may progress to HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and to ATLL. Adult onset IDH and reactional and inflammatory dermatoses found in carriers and also in patients with HAM/TSP will be considered. Other dermatological diseases that occur more frequently in HTLV-1-infected individuals such as xerosis, acquired ichthyosis, seborrheic dermatitis and infectious and parasitic dermatoses will also be discussed.

Human T-cell leukemia virus type I (HTLV-I) infection and the onset of adult T-cell leukemia (ATL); Matsuoka, Masao; Retrovirology  Vol.  2  Issue  1, 2005

flower cells

flower cells

11-BIRT-HOGG-DUBÉBirt–Hogg–Dubé syndrome (BHD; 1977) is a human genetic disorder that involves susceptibility to renal cancer, renal and pulmonary cysts, and tumors of the hair follicles. The disorder has been reported in more than 100 families worldwide, and it is inherited in an autosomal dominant pattern. Mutations in the FLCN gene, located on the short arm of chromosome 17 (17p11.2), that makes a protein called folliculin.Clinical triad

1. The cutaneous manifestations of BHD were originally described as fibrofolliculomas, trichodiscomas, and acrochordons. The dermatologic diagnosis of BHD can be made in an individual five or more skin lesions, at least one of which must be confirmed as a fibrofolliculoma by biopsy.

2. Most individuals (89%) with BHD are found to have multiple cysts in both lungs, and 24% have had one or more episodes of pneumothorax. The cysts can be detected by chest CT scan.

3. Renal tumors can manifest as multiple types of renal cell carcinoma, but certain pathological subtypes (including chromophobe, oncocytoma, and oncocytic hybrid tumors) are more commonly seen in BHD.

Other, less commonly associated features include a large connective-tissue nevus, parathyroid adenomas, flecked chorioretinopathy, bullous emphysema, lipomas, angiolipomas, parotid oncocytomas, multiple oral mucosal papules, neural tissue tumors, and multiple facial angiofibromas.

http://emedicine.medscape.com/article/1060579-overview

A- TRICODISCOMA B- FIBROFOLICULOMA

12-BROOKE-SPIEGLERBrooke-Spiegler syndrome is an uncommon disease with a predisposition to develop cutaneous adnexal neoplasms such as cylindromas, trichoepitheliomas, spiradenomas, trichoblastomas, basal-cell carcinomas, follicular cysts, organoid nevi, and malignant transformation of pre-existing tumors in the affected individualsBrooke-Spiegler syndrome is inherited in autosomal-dominant fashion, although expression and penetrance are variable. Lesions usually begin to appear in the second or third decades and gradually increase in number and size throughout adult lifewomen are affected more frequently than are menmutations in the CYLD tumor-suppressor gene, located at 16q12-q13, have been implicated in the phenotype diversityCylindromas, trichoepitheliomas, and spiradenomas, which are the most commonly observed tumors, are typically located on the head and neckmultiple trichoepitheliomas may be seen also in two other rare syndromes: Rombo syndrome (vermicular atrophoderma, milia, hypotrichosis, basal-cell carcinomas, trichoepitheliomas, and peripheral vasodilatation with cyanosis) and Bazex syndrome (follicular atrophoderma, hypotrichosis, occasional trichoepitheliomas, basal-cell carcinomas, and localized or generalized hypohidrosis) http://dermatology.cdlib.org/131/cases/NYUcases/111505_7.html

12-BROOKE-SPIEGLERAm J Dermatopathol. 2011 Mar 8. Michal MMultiple (Familial) Trichoepitheliomas: A Clinicopathological and Molecular Biological Study, Including CYLD and PTCH Gene Analysis, of a Series of 16 Patients.Abstract – Multiple familial trichoepitheliomas (MFT) constitute an autosomally inherited syndrome possibly related to Brooke-Spiegler syndrome (BSS). Although some early studies suggested a role for the PTCH gene on chromosome 9q22.3 in the etiopathogenesis of MFT, recent studies of occasional patients with the MFT clinical phenotype identified mutations in the CYLD gene on chromosome 16q12-q13, a gene responsible for BSS. A systematic investigation of PTCH and CYLD mutations in patients with MFT has never been performed. Our main objective was to collect a reasonably large series of patients with MFT to (1) study the clinicopathological spectrum of the disease, (2) determine whether the PTCH gene is implicated in the pathogenesis of MFT, and if so (3) determine the relative frequency of CYLD and PTCH mutations, (4) establish if there may be any possible genotype-phenotype correlations, and (5) study the spectrum of somatic mutations. Clinical analysis including family histories, histopathological investigations, and molecular genetic studies were performed. There were 9 female and 7 male patients ranging in age from 11 to 63 years. They presented with multiple, small, discrete and sometimes confluent, skin-colored to pink, asymptomatic nodules preferentially located on the face, being especially prominent and confluent in the nasolabial folds and inner aspects of the eyebrows. A total of 66 conventional trichoepitheliomas (TEs) were studied microscopically. Aside from typical features of TE, some also exhibited variant morphological patterns including areas reminiscent of other benign adnexal neoplasms and melanocytic hyperplasia. In none of the 9 patients tested was a germline mutation of the PTCH gene identified. Germline CYLD mutations were detected in 6 of 13 patients tested (identical in 2 unrelated patients) including 2 novel mutations, whereas the remaining 7 individuals showed wild-type alleles. Two patients with germline wild-type CYLD showed, however, a somatic mutation in the gene (1 duplication, 1 substitution mutation). Neither CYLD nor PTCH germline mutations were found in the 5 patients in whom both genes were analyzed. MFT seems to be a phenotypic variant of BSS. The PTCH gene is rarely, if ever, involved in the pathogenesis of MFT. Absence of a germline mutation of the CYLD gene in cases harboring a somatic mutation may be explained by large deletions in the gene or by mutation in intronic sequences or in the promoter region. Considering our 5 patients with no mutation in either gene, the final possibility is that another, as yet undescribed gene (neither CYLD nor PTCH) is implicated in the pathogenesis of some patients with MFT.

13-HEMANGIOMA GLOMERULOIDE – POEMS polineuropatia, organomegalia, endocrinopatia, proteína M e alterações cutâneas (skin changes) – Bardwick, 1980 mieloma osteoesclerótico, síndrome de Crow-Fukase e síndrome de Takatsuki síndrome de POEMS é uma entidade clínica única definida pela presença concomitante de doença monoclonal de plasmócitos, polineuropatia periférica e outras manifestações paraneoplásicas tais como organomegalias, endocrinopatias, alterações cutâneas, papiledema, lesões osteoescleróticas e sobrecarga de volume extravascular. a sobrevida média dos pacientes com síndrome de POEMS é significativamente superior àquela esperada para os portadores de mieloma múltiplo (165 e 38 meses, respectivamente), independente do número de manifestações presentes ao diagnóstico e da intensidade de infiltração da medula óssea por plasmócitos outros achados comuns relacionados à síndrome incluem lesões osteoescleróticas, doença de Castleman, derrame pleural, ascite, edema periférico, papiledema, eritrocitose, plaquetose e baqueteamento dos dedos. O quadro clínico peculiar e a maior sobrevida média dos pacientes distinguem claramente a síndrome de POEMS do mieloma múltiplo. pelo menos dois terços dos portadores de síndrome de Poems apresentam uma ou mais das seguintes anormalidades endócrinas detectadas através da avaliação clínica ou laboratorial: hipogonadismo, hipotiroidismo, diabetes melllitus, insuficiência adrenal ou hipoparatiroidismoalterações cutâneas: hiperpigmentação, hipertricose, acrocianose, pletora, hemangiomata/telangectasiaRev. Bras. Hematol. Hemoter. vol.29 no.1 São José do Rio Preto Jan./Mar. 2007http://emedicine.medscape.com/article/1097031-overview

14-ESTRONGILOIDIASEStrongyloidiasis is caused by the soil-dwelling nematode Strongyloides stercoralis. This parasite is endemic to tropical and subtropical regions throughout the world including areas of the southeastern United States and Appalachia and affects 100 million people worldwide. Humans are infected transcutaneously by filariform larvae that subsequently travel via the venous system to the lungs. There they eventually enter the pharynx and are swallowed. In the small intestine adult female worms produce eggs that hatch rhabditiform larvae. These larvae pass in the stool to continue the free-living external life cycle or develop into filariform larvae that can reinfect the host by penetration of the perianal skin or intestinal wall. This latter process is termed "autoinfection" and can occur at low levels throughout the course of infection.Strongyloidiasis has a wide range of manifestations from asymptomatic disease to disseminated infection. Acute and chronic infection, hyperinfection syndrome, and disseminated infection are described. Cutaneous manifestations of acute strongyloidiasis are not well characterized, but local urticarial reaction at the site of larval entry may occur. Pulmonary and gastrointestinal symptoms have been described, with larvae detectable in stool within three to four weeks. Chronic infection is most frequently asymptomatic. Dermatological manifestations include chronic urticarial lesions of the buttocks and waistline that last one to two days. Larva currens is pathognomonic of chronic Strongyloides infection and is caused by filiariform larval migration in the skin. This manifests as intensely pruritic serpiginous urticarial wheals that move at a rate of 5-15 cm/hour and occur mainly on the buttocks, groin, and trunk. Biopsies have not revealed larvae. Hyperinfection occurs with accelerated autoinfection, usually as a result of immunosuppression that leads to increased multiplication and migration of larvae. This increased larval burden produces exacerbated gastrointestinal and pulmonary symptoms. When larvae migrate beyond the pulmonary and intestinal systems, the term "disseminated infection" is used. Cutaneous manifestations of disseminated disease, as seen in our patient, include progressive petechial and purpuric eruptions on the abdomen and proximal extremities. As we have shown, larvae can be demonstrated on routine hematoxylin-eosin stains. The larvae migrate through blood vessel walls into the dermis causing hemorrhage and the petechial appearance of this eruption.http://anagen.ucdavis.edu/1412/case_reports/strongyloidiasis/arch.html

15-DOENÇA DE FLEGELFlegel originally described hyperkeratosis lenticularis perstans (HLP) in 1958- Flegel H. [Hyperkeratosis lenticularis perstans.]. Hautarzt. Aug 1958;9(8):363-4occurs most commonly in mid-to-older age groups; however, reports exist of occurring in patients as young as 13 yearsall reports have been in white patientssmall, red-brown, hyperkeratotic, 1-5 mm papules begin to develop symmetrically on the lower extremities are the most frequent and characteristic presentation of hyperkeratosis lenticularis perstans; involvement of the ear pinnae, arms, palms, soles, and the oral mucosa has been reported, although these reports are rare; involvement of the trunk has been reported but remains an unusual variantremoval of the scale reveals a bright red base, often with pinpoint bleedingDD: acrokeratosis verruciformis of Hopf, porokeratosis, actinic keratosis, Stucco keratosis, Darier disease, and Kyrle Diseasebiopsy: a discrete area of hyperkeratosis occurs (with areas of parakeratosis) overlying a thinned stratum malpighii and thinned-to-absent granular layer; irregular acanthosis and some vascular dilatation are peripheral; a lymphoid infiltrate with occasional histiocytes in a bandlike pattern in the papillary dermis typically is seen – pode ter padrão de DIVa muito discretohttp://emedicine.medscape.com/article/1107012-diagnosis

16-PYOSTOMATITIS VEGETANSColl Antropol. 2010 Apr;34 Suppl 2:279-82.Pyostomatitis vegetans associated with inflammatory bowel disease--report of two cases.Mijandrusić-Sincić B, Licul V, Gorup L, Brncić N, Glazar I, Lucin K.Abstract – Pyostomatitis vegetans (PV) is a rare, chronic mucocutaneous disorder associated with inflammatory bowel disease (IBD). Oral lesions of PV are distinct and present as multiple white or yellow pustules with an erythematous base that coalesce and undergo necrosis to form a typical "snail tracks" appearance. Two cases of PV associated with IBD--one with Crohn's disease (CD) and the other with ulcerative colitis (UC) are reported. In the first case, adalimumab therapy brought the oral and gastrointestinal manifestations to complete remission. In the second case, the remission was achieved with systemic steroid therapy, but the disease relapsed after therapy discontinuation. Azathioprine was added leading to sustained remission of PV. Because of persistent active intestinal manifestation of UC, in spite of immunosuppressive therapy, infliximab was introduced. With the therapy remission of intestinal manifestation of UC was achieved as well. Our cases confirm previously reported good experience with immunomodulators and biologics in the treatment of PV. But, before using them we have to exclude an infectious etiology of oral lesions.

16-PYOSTOMATITIS VEGETANSAm J Dermatopathol. 2011 Feb;33(1):e1-6.All that glitters is not pemphigus: Pyodermatitis-pyostomatitis vegetans misdiagnosed as IgA pemphigus for 8 years.Abellaneda C, Mascaró JM Jr, Vázquez MG, Pablo IM, Iranzo P.AbstractPyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis often associated with gastrointestinal disorders, especially with inflammatory bowel disease. It is clinically characterized by erythematous lesions with multiple pustules and erosions affecting the mucosal surfaces. Cutaneous lesions are characterized by exudative and vegetating plaques affecting frequently the axillae and groins. The clinical diagnosis is supported by histologic findings, whereas immunofluorescence studies (negative) are useful to rule out other entities such as pemphigus. Herein we report the case of a young man who was misdiagnosed as having IgA pemphigus for 8 years due to positive immunofluorescence findings. The clue for the final diagnosis was the diagnosis of a concomitant ulcerative colitis, which prompted us to reconsider his cutaneous disease.

16-PYOSTOMATITIS VEGETANSJ Am Acad Dermatol. 1994 Aug;31(2 Pt 2):336-41.Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory bowel disease.Storwick GS, Prihoda MB, Fulton RJ, Wood WS.AbstractIn pyodermatitis-pyostomatitis vegetans annular pustular cutaneous lesions may precede, accompany, or follow the usually extensive vegetating oral disease. Sometimes only cutaneous or only oral lesions occur and previously have been described as separate entities. Clinical, histopathologic, and immunopathologic evidence clearly indicates this is one disease and suggests that it is distinct from pemphigus vegetans. The association between pyodermatitis-pyostomatitis vegetans and inflammatory bowel disease, most commonly ulcerative colitis, has been amply confirmed. Pyodermatitis-pyostomatitis vegetans should be considered a marker for inflammatory bowel disease.

17-AMALGAMA TATTOOAmalgam tattoos are common oral pigmented lesions that clinically present as isolated, blue, grey, or black macules on the gingivae, the buccal and alveolar mucosae, the palate, and/or the tongue. They are due to deposition of a mixture of silver, tin, mercury, copper, and zinc, which are components of an amalgam filling, into the oral soft tissues. The deposition occurs after a number of different dental procedures that include diffusion through soft tissues from root-end fillings, accidental deposition of fine metallic particles into the gingiva by high-speed drills, accidental abrasion of the mucosa by high-speed rotary instruments, or deposition of amalgam scraps left behind during extraction. These tattoos do not represent a health hazard since the mercury present in amalgam is not in a free state. However, owing to its clinical appearance, amalgam tattoos can be mistaken for a number of different conditions of concern, such as melanoma, pigment-cell nevi, melanotic macules, melanoacanthoma, Kaposi's sarcoma, and physiologic pigmentation. The diagnosis is more easily determined if the lesion is in the vicinity of a large silver amalgam restoration or a gold crown. If not, a biopsy may be performed. Histopathologic features include discrete, fine, dark granules and irregular, solid fragments. They can be found along collagen bundles and vessels and also are found within macrophages, mulinucleated giant cells, fibroblasts, and endothelial cells. Treatment for amalgam tattoos was originally limited to surgery with grafting of mucosa or gingiva over the previous site of the tattoo. Advances in laser technology now allow amalgam tattoos to be removed by the Q-switched ruby laser.http://dermatology.cdlib.org/145/nyu/cases/032007_6.html

17-AMALGAMA TATTOOHell Stomatol Chron. 1989 Apr-Jun;33(2):113-20.Differential diagnosis of bluish and pigmented lesions of the oral mucosaTsiklakis K, Patsakas A.Abstract – the clinical features of the most common bluish and pigmented lesions of the oral mucosa are discussed in this paper. Considerable attention is given to the findings from the medical and dental history of the patient, in the methodology of the clinical examination (inspection, palpation, digital pressure, aspiration) in the clinical characteristics of the lesions (location, size duration, consistency, prognosis) in the laboratory findings (radiographs and other supplementary examinations) and in the differential diagnosis. The bluish and pigmented lesions which are discussed include: melanoma, Albright's syndrome, Addison's diseases, Peutz-Jeghers's syndrome, arsening poisoning, hemangioma, hematoma, petechia and ecchymosis, Sturge-Weber syndrome, amalgam tatoo, heavy metal lines, mucocele and eruption cyst.http://emedicine.medscape.com/article/1078143-media

18-SÍNDROME DE SNEDDONSneddon's syndrome, also known as "Idiopathic livedo reticularis with cerebrovascular accidents“, is a form of characterized by several symptoms, including: cerebrovascular disease, livedoid vasculitis, and hypertensionit is named for Ian Bruce Snnedon in 1965 – Sneddon IB (April 1965). "Cerebrovascular lesions and livedo reticularis“; Br. J. Dermatol. 77: 180–5 J Am Acad Dermatol. 2005 Jun;52(6):1009-19.Livedo reticularis: an update.Gibbs MB, English JC 3rd, Zirwas MJ.AbstractLivedo reticularis (LR) is a well-known, relatively common physical finding consisting of macular, violaceous, connecting rings that form a netlike pattern (Fig 1). In most cases, it is a completely benign finding related to cold exposure. However, there are many potential causes (Table I), and this can make the evaluation of a patient presenting with this finding very difficult. An excellent review of the topic by Fleischer and Resnick was published in 1990. We have endeavored to update the literature and provide clinicians with guidance regarding the evaluation and treatment of patients presenting with LR.

18-SÍNDROME DE SNEDDONJ Neurol. 2005 Oct;252(10):1155-66. Epub 2005 Aug 26.The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review.Kraemer M, Linden D, Berlit P.AbstractLivedo is a cutaneous sign of striking violaceous netlike patterned erythema of the skin. This dermatological phenomenon is of special interest in the differential diagnosis in neurological patients. In 1907 Ehrmann distinguished two different patterns of livedo: the pathological livedo racemosa and the physiological livedo reticularis. Despite important clinical differences, in the English language literature the heading livedo reticularis is still used for all types of livedo. A literature review about the spectrum of differential diagnosis in patients with livedo reticularis (especially cutis marmorata and amantadine-induced livedo reticularis) and livedo racemosa (especially Sneddon's syndrome, Divry-van Bogaert syndrome, systemic lupus erythematosus, antiphospholipid antibody syndrome, polyarteritis nodosa, cholesterol embolization syndrome, livedoid vasculopathy and haematological diseases) is provided.

19-MALACOPLAQUIAMalakoplakia is an inflammatory condition presenting as a plaque or a nodule that usually affects the genitourinary tract but may rarely involve the skin. Malakoplakia was first described in the early 1900s as yellow soft plaques that were seen on the mucosa of the urinary bladder. Microscopically, malakoplakia is characterized by the presence of foamy histiocytes with distinctive basophilic inclusions, which are known as Michaelis-Gutmann bodiesMalakoplakia is a treatable inflammatory reaction to bacteria, mycobacteria, or fungi that may occur in immunocompromised patients – a number of cases caused by Rhodococcus equi have been reported in AIDS patients also characteristic of lesions of malakoplakia are rounded intra- or extracellular inclusions representing giant lysosomes. They contain organisms and often calcify. These inclusions are known as Michaelis-Gutman bodieshttp://emedicine.medscape.com/article/1055606-overview

19-MALACOPLAQUIAArch Pathol Lab Med. 2008 Jan;132(1):113-7.Cutaneous malakoplakia.Kohl SK, Hans CP.Abstract – malakoplakia is an acquired granulomatous disorder first described by Michaelis and Gutmann in 1902. The pathogenesis of malakoplakia is poorly understood, but it is thought to be secondary to an acquired bacteriocidal defect in macrophages occurring mostly in immunosuppressed patients or in the setting of autoimmune disease. Malakoplakia has been described in numerous anatomic locations, most commonly in the genitourinary tract. Microscopically, malakoplakia consists predominantly of sheets of macrophages known as von Hansemann cells with scattered targetoid intracytoplasmic inclusions known as Michaelis-Gutmann bodies. Cutaneous malakoplakia is a rare entity with less than 50 cases reported in the literature. In this article, we review cutaneous malakoplakia including the clinical, gross, and microscopic features as well as the treatment and prognosis of 40 cases of cutaneous malakoplakia identified in the literature.

Michaelis-Gutmann bodies –von Kossa

Michaelis-Gutmann bodies are positive for calcium (von Kossa stain, original magnification, x 200).

20-GRANULOMA 2ND PREENCHEDOR DÉRMICO

Plast Reconstr Surg. 2008 May;121(5):1811-20. Salles AG, Ferreira MC.Complications after polymethylmethacrylate injections: report of 32 cases.Abstract – During the past 15 years, polymethylmethacrylate has been used as a synthetic permanent filler for soft-tissue augmentation.METHODS: This article reports 32 cases of complications seen at Hospital das Clínicas, Faculty of Medicine, University of São Paulo, for procedures performed elsewhere. RESULTS: The average age of the patients was 43.6 years (range, 22 to 70 years). Twenty-five patients were women. Sixteen injection procedures were performed by certified plastic surgeons, nine by dermatologists, two by urologists, and one by a nonphysician. Complications were classified into five groups according to main presentation as follows: tissue necrosis (five cases), an acute complication that can be related to technical mistakes but that can also be dependent on patient factors or caused by local infection; granuloma (10 cases), which usually presents as a subacute complication 6 to 12 months after the procedure; chronic inflammatory reactions (10 cases), which usually occur years later and can be related to a triggering event, such as another operation or infection in the area that was injected (these reactions are immunogenic in origin and may have cyclic periods of activation and remission); chronic inflammatory reaction in the lips (six cases), which may be present with severe symptoms, especially with lymphedema, because of mobility of the lip; and infections (one case), which are rare but possible complications after filling procedures. CONCLUSIONS: Polymethylmethacrylate filler complications, despite being rare, are often permanent and difficult or even impossible to treat. Safety guidelines should be observed when considering use of polymethylmethacrylate for augmentation.

20-GRANULOMA 2ND PREENCHEDOR DÉRMICO

CD68

http://www.ispub.com/journal/the_internet_journal_of_dermatology/volume_3_number_1_11/article/dermalive_granuloma_a_lesion_with_distinctive_histological_features.html

http://emedicine.medscape.com/article/1125066-overview