Abstract: The classification of cutaneous lymphomas is multidisciplinary and requires the correlation between clinical, histopathological, immunohistochemical, and molecular diagnostic elements. In this article, we present four different cases of CD30-positive T-cell lymphoma with cutaneous manifestations. We compare cases with definitive diagnosis of papulosis lymphomatoid type C, primary cutaneous anaplastic large T-cell lymphoma, systemic anaplastic large T-cell lymphoma with secondary skin involvement, and mycosis fungoides with large cell transformation, highlighting the importance of clinicopathological correlation to classify these cases.

Abstract Woolly hair nevus is a rare condition characterized by a structural anomaly of the hair, restricted to certain areas of the scalp. The hair becomes coiled and slightly hypopigmented. The term woolly hair refers to changes that affect all the scalp and has a hereditary character. We present a case of woolly hair nevus, that developed at the age of 2 years, associated with dental diastema and verrucous epidermal nevus.

Abstract The use of tumor necrosis factor antagonists (anti-TNF) has become a usual practice to treat various inflammatory diseases. Although indicated for the treatment of psoriasis, anti-TNF may paradoxically trigger a psoriasiform condition. We present a case of a female patient who, during the use of infliximab for rheumatoid arthritis, developed psoriasis. In an attempt to switch anti-TNF class, we observed a cumulative worsening of the lesions requiring suspension of the immunobiological agent and the introduction of other drugs for clinical control. The therapeutic challenge of this paradoxical form of psoriasis is the focus of our discussion. The use of another anti-TNF in these patients is a matter of debate among experts.

Abstract Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

We report the case of an 11-year-old male patient with a histopathological and immunohistochemical diagnosis of dermatofibroma with an atypical clinical presentation on the right forearm. Although dermatofibroma is considered a benign skin tumor, some of its differential diagnoses, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, are truly aggressive. Lesions with atypical clinical aspects and topology associated with specific histopathological variants are some of the criteria for complete tumor excision.

Os autores relatam um caso de esquistossomose cutânea ectópica em uma paciente de 35 anos que apresentou máculas e pápulas eritematosas agrupadas na nádega esquerda. O diagnostico não foi suspeitado durante a avaliação clínica, tendo sido obtido através da visualização dos ovos no exame histopatológico.

The authors report a case of ectopic cutaneous schistosomiasis in a 35 year-old female who presented clustered reddish macules and papules on the left buttock. The diagnosis was not suspected during clinical evaluation and required visualization of Schistosoma mansoni eggs on sections of tissue.

A dermatose bolhosa por IgA linear tem sido crescentemente associada com doenças inflamatórias intestinais, especialmente a retocolite ulcerativa. Relatamos o caso de um adolescente masculino, 13 anos de idade, com retocolite ulcerativa diagnosticada 11 meses antes, que desenvolveu vesículas, pústulas e erosões na pele da face, do tronco e das nádegas e na mucosa oral. A investigação revelou doença bolhosa subepidérmica rica em neutrófilos e deposição linear de IgA ao longo da junção dermoepidérmica, estabelecendo o diagnóstico de dermatose bolhosa por IgA linear. O paciente experimentou controle insatisfatório dos sintomas cutâneos e gastrointestinais apesar do uso de adalimumab, mesalazina, prednisona e dapsona por alguns meses. Após colectomia total, ele apresentou remissão completa das lesões cutâneas, sem necessidade de medicações durante os dois anos de seguimento. Neste artigo, revisamos os casos previamente relatados desta associação e debatemos o papel da retocolite ulcerativa no desencadeamento da dermatose bolhosa por IgA linear.

Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The patient experienced unsatisfactory partial control of skin and intestinal symptoms despite the use of adalimumab, mesalazine, prednisone and dapsone for some months. After total colectomy, he presented complete remission of skin lesions, with no need of medications during two years of follow-up. A review of previously reported cases of the association is provided here and the role of ulcerative colitis in triggering linear IgA dermatosis is discussed.

A dermatite seborreica é uma doença eritêmato-escamativa de caráter crônico-recidivante que acomete entre 1 e 3% da população geral dos Estados Unidos. Possui dois picos de incidência - o primeiro, durante os três primeiros meses de vida, e o segundo, a partir da puberdade, atingindo seu ápice entre os 40 e 60 anos de idade. Os indivíduos HIV positivos têm maior prevalência da doença, que apresenta maior intensidade e tendência à refratariedade ao tratamento. Doenças neurológicas e outras doenças crônicas também estão associadas ao desenvolvimento da dermatite seborreica. Como mecanismo fisiopatogênico, reconhece-se que o fungo Malassezia sp., presente na pele de indivíduos suscetíveis, leve a uma irritação não-imunogênica a partir da produção de metabólitos à base de ácidos graxos insaturados deixados na superfície cutânea. Este artigo faz uma revisão da literatura sobre dermatite seborreica, com ênfase nos aspectos imunogenéticos, formas clínicas e tratamento.

Seborrheic dermatitis is a chronic relapsing erythematous scaly skin disease, the prevalence of which is around 1 to 3% of the general population in the United States. It has two incidence peaks, the first in the first three months of life and the second beginning at puberty and reaching its apex at 40 to 60 years of age. The prevalence of seborrheic dermatitis is higher in HIV-positive individuals and the condition tends to be more intense and refractory to treatment in these patients. Neurological disorders and other chronic diseases are also associated with the onset of seborrheic dermatitis. The currently accepted theory on the pathogenesis of this disease advocates that yeast of Malassezia spp., present on the skin surface of susceptible individuals, leads to a non-immunogenic irritation due to the production of unsaturated fatty acids deposited on the skin surface. This article provides a review of the literature on seborrheic dermatitis, focusing on immunogenetics, the clinical forms of the disease and its treatment.

O perineurioma esclerosante é uma neoplasia benigna rara,constituída exclusivamente por células com diferenciação perineural, dispersas em meio a estroma fibroso denso. Acomete preferencialmente a pele dos quirodáctilos e das regiões palmares e se apresenta como pápula ou nódulo firme, normocrômico e assintomático. Apresentamos um caso de perineurioma esclerosante localizado na região palmar esquerda de uma paciente feminina de 16 anos de idade, com detalhada demonstração dos aspectos clínicos, histopatologia e revisão da literatura publicada em língua inglesa.

Sclerosing perineurioma is a rare benign neoplasm composed exclusively of perineural differentiation cells spread in a dense fibrous stroma. It affects primarily the skin of fingers and that of the palm of the hands. It appears as a hard papule or nodule, normochromic and asymptomatic. A case of sclerosing perineurioma in the left palm of a 16-year-old female is described, with detailed demonstration of clinical aspects, histopathology and literature review published in the English language.

O carcinoma de células escamosas pigmentado é variedade rara de carcinoma de células escamosas, havendo apenas 13 casos de localização na pele relatados na literatura inglesa, cinco na literatura latino-americana e nenhum caso na brasileira. Neste trabalho, os autores relatam um caso de carcinoma de células escamosas pigmentado da pele, destacando os aspectos clínicos, histopatológicos e imuno-histoquímicos.

Pigmented squamous cell carcinoma is a rare variant of squamous cell carcinoma. Only 13 cases arising in the skin have been reported in the English literature, five cases in Latin America and none in Brazil. The authors report a case of pigmented squamous cell carcinoma, with emphasis on clinical, pathological and imunohistochemical aspects.