Abstract: Granuloma annulare is a relatively common, idiopathic, benign inflammatory dermatosis, with a varied clinical presentation that often makes diagnosis difficult. It mainly affects the extremities, such as the dorsa of the hands and feet, forearms and legs. Palmar and plantar regions are generally spared. It occurs mainly in young female patients. The presentation of the palmar variant in an elderly patient is a rarity.

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

AbstractMuir-Torre syndrome is a rare genodermatosis characterized by the occurrence of at least one sebaceous tumor associated with visceral neoplasia, but with no predisposing factors. The sebaceous neoplasm may appear before, during or after the diagnosis of colorectal cancer. As it is regarded as a subtype of nonpolyposis hereditary colorectal cancer, it is important to evaluate the patient's first-degree relatives. The clinical course of the neoplasm is usually more indolent and the syndrome has a good prognosis. We report the case of a patient who, after a ten-year diagnosis of colorectal cancer, presented with multiple sebaceous neoplasms.

AbstractGranulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

Mulher de 57 anos, com equimose periorbitária, frouxidão cutânea nas dobras, polineuropatia e síndrome do túnel do carpo bilateral.O exame histopatológico da lesão axilar revelou fragmentação de fibras elásticas, porém a coloração de von Kossa foi negativa;o diagnóstico foi de cútis laxa. Amiloidose sistêmica primária foi confirmada pela presença de material amilóide no exame histopatológico da pálpebra, além de púrpura, polineuropatia, síndrome do túnel do carpo bilateral e gamopatia monoclonal. Foi diagnosticada como portadora de mieloma múltiplo por apresentar 40% de plasmócitos na medula óssea, componente M urinário e anemia. A paciente evoluiu com bolhas de conteúdo citrino, cujo exame histopatológico mostrou mucinose. Os diagnósticos finais foram: amiloidose sistêmica primária, cútis laxa adquirida e mucinose, todos vinculados ao mieloma múltiplo.

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Síndrome de Berardinelli-Seip é doença genética autossômica recessiva rara, caracterizada por ineficiência em metabolizar e estocar material lipídico adequadamente nos adipócitos, gerando acúmulo de gordura em órgãos não habituais, como fígado, baço, pâncreas, coração, endotélio arterial e pele. Classicamente, os portadores nascem ou manifestam lipoatrofia generalizada até os 2 anos e, geralmente na adolescência, desenvolvem marcada resistência insulínica com rápida progressão para diabetes e dislipidemia. Relatamos um caso de portadora da síndrome de Berardinelli-Seip, de 17 anos, com xantoma eruptivo associado à hipertrigliceridemia grave. Ressalta-se o xantoma eruptivo como manifestação dermatológica não enfatizada nos casos relatados sobre esse distúrbio metabólico genético.

Berardinelli-Seip syndrome is a rare autosomal recessive disease characterized by inadequate metabolism and inefficient storing of lipids in fat cells, generating accumulation of fat in organs such as the liver, spleen, pancreas, heart, arterial endothelium and skin. Classically, patients manifest generalized lipoatrophy at birth or until 2 years of age, and in adolescence usually develop marked insulin resistance with rapid progression to diabetes and dyslipidemia. We report the case of a 17-year-old Berardinelli-Seip syndrome patient with eruptive xanthoma associated with severe hypertriglyceridemia. It is worth noting Eruptive xanthoma as a dermatological manifestation that is not generally highlighted in the reports of cases of this genetic metabolic disorder.

A telangiectasia macular eruptiva perstans é uma forma rara de mastocitose cutânea, caracterizada pela presença de máculas eritematosas ou castanho-amareladas com telangiectasias, localizadas preferencialmente no tronco e membros superiores. Descrevemos um caso de telangiectasia macular eruptiva perstans enfocando nas características dermatoscópicas dessa doença.

Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis, characterized by the presence of erythematous or yellowish-brown macules with telangiectasias, preferably located on the trunk and upper limbs. We have described a case of telangiectasia macularis eruptiva perstans focusing on the dermoscopic characteristics of this disease.

Líquen escleroso é uma doença mucocutânea inflamatória, crônica, de etiologia desconhecida, que afeta mais comumente a genitália feminina. O envolvimento cutâneo com bolhas não hemorrágicas é muito raro. Descrevemos um caso de líquen escleroso bolhoso.

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Angioleiomiomas são tumores benignos raros derivados da musculatura lisa vascular, que geralmente se apresentam como nódulo doloroso solitário nos membros inferiores. Relata-se um caso de angioleiomioma no segundo quirodátilo esquerdo, localização incomum deste tumor.

Vascular leiomyoma are uncommon benign smooth muscle tumors which generally present as a single painful nodule in the lower limbs. We report a case of vascular leiomyoma on the second finger of the left hand, an unusual location for this tumor.

A sarcoidose é uma doença granulomatosa de etiologia desconhecida. A pele é comumente afetada. As manifestações cutâneas podem mimetizar outras afecções e desordens auto-imunes. Assim, o dermatologista assume papel fundamental para elucidar o diagnóstico clínico e auxiliar outros especialistas na investigação de uma doença sistêmica. Relatamos um caso de paciente com manifestação cutânea típica de sarcoidose associada com acometimento pulmonar.

Sarcoidosis is a granulomatous disease of unknown etiology. The skin is commonly affected. Cutaneous manifestations can mimic other diseases and autoimmune disorders. The dermatologist plays a critical role in elucidating the clinical diagnosis and assisting other specialists in the investigation of a systemic disease. We report a patient with typical cutaneous manifestation of sarcoidosis with pulmonary involvement.

Relatamos um caso de histiocitose cefálica benigna em uma criança do sexo masculino, de um ano e três meses de idade que desenvolveu múltiplas pápulas na região malar bilateralmente, sem outros comemorativos associados. A histopatologia caracterizou-se pelo padrão derme papilar, com imuno-histoquímica negativa para S100 e CD1a, e positiva para CD68, ficando assim estabelecido o diagnóstico desta histiocitose não- Langerhans, baseado nos aspectos clínicos, histopatológicos e imuno-histoquímicos característicos.

The present paper reports a case of benign cephalic histiocytosis in a 15-month baby boy, who developed multiple papules bilaterally in the malar region with no other associated manifestations. Histopathology revealed a papillary dermal pattern, while immunohistochemistry was negative for S100 and CD1a and positive for CD68. Therefore, diagnosis was established as non-Langerhans cell histiocytosis, based on the clinical, histopathological and immunohistochemical features present.

A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20% dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20% of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Relata-se o caso de um homem de 45 anos com dermatofitose superficial de longa data, tratado, inadvertidamente, com corticoide e antibiótico, com progressão subsequente para a forma profunda, conhecida como granuloma de Majocchi. O tratamento com terbinafina VO foi curativo

We report the case of a man of 45 with superficial dermatophytosis longtime inadvertently treated with antibiotics and corticosteroids with subsequent progression to the deep form, known as granuloma Majocchi. Treatment with orally terbinafine was successful