Case for diagnosis. Single-digit clubbing

Abstract A 58-year-old female patient presented with a single-digit clubbing on the second finger of her right hand two years previously. After investigation with imaging and incisional biopsy, superficial acral fibromyxoma was diagnosed. A brief review on single-digit clubbing and its causes is presented, focusing on superficial acral fibromyxoma.

Intravascular histiocytosis: case report of a rare disease probably associated with silicone breast implant

Abstract Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.

Therapeutic failure with itraconazole in sporotrichosis due to bariatric surgery

Abstract Sporotrichosis is a deep mycosis of subacute or chronic evolution, caused by the dimorphic fungus of the genus Sporothrix. The treatment is carried out with antifungal orally or intravenously. Therapeutic success can be affected by several factors, such as altered gastrointestinal physiology by surgery. More and more patients are submitted to bariatric surgeries and the literature for the alterations of the absorption of medications in this context is very scarce. We intend to contribute to a better understanding with this case report of cutaneous-lymphatic sporotrichosis in a patient after bariatric surgery without response to itraconazole treatment, even at high doses.

Dermoscopy of squamoid eccrine ductal carcinoma: an aid for early diagnosis

Abstract: Squamoid eccrine ductal carcinoma is a cutaneous malignancy that originates from the eccrine sweat gland. A 76-year-old woman presented with an asymptomatic papule on her nose that had been present for one year. Dermoscopy showed pseudocysts, irregular linear crown vessels, and yellowish-brown globules surrounded by white halos. This is the first dermoscopic description of squamoid eccrine ductal carcinoma. Studies with more cases are needed to confirm the dermoscopic characterization of this tumor.

Dermoscopy of granuloma faciale: a description of a new finding

Abstract: Granuloma faciale is a rare, chronic dermatologic disorder, which mainly affects the face. Recently, dermoscopy has been demonstrated as an important ancillary tool on the clinical diagnosis of facial dermatoses. We report two cases of granuloma faciale with yellow areas on dermoscopy that was not yet described in the literature, corresponding to abundant hemosiderin on histopathological examination.

Do you know this syndrome? Heerfordt-Waldenström syndrome

Abstract: Heerfordt-Waldenström syndrome is a rare subacute variant of sarcoidosis, characterized by enlargement of the parotid or salivary glands, facial nerve paralysis and anterior uveitis. Granulomas with a peripheral lymphocyte deficit are found in the anatomic pathology of affected organs. It is normally self-limiting, with cure achieved between 12 and 36 months, but some prolonged cases have been reported. Diagnosis of the syndrome is clinical, and treatment depends on the degree of systemic impairment. Oral corticosteroids represent the first line treatment option. The mortality rate ranges between 1 and 5% of cases.

Hidroacanthoma simplex: dermoscopy and cryosurgery treatment

Abstract: Hidroacanthoma simplex is a rare intraepidermal neoplasia that arises from the acrosyringial portion of the eccrine duct. Malignant transformation of hidroacanthoma simplex is reported in the literature and the treatment is performed with wide excision or Mohs micrographic surgery. We report the first case successfully treated with cryosurgery with a long-term follow up.

Dermatofibrossarcoma atrófico: a importância da suspeita clínica / Atrophic Dermatofibrosarcoma: the importance of clinical suspicion

Dermatofibrossarcoma protuberante é tumor fibro-histiocítico de malignidade intermediária, relativamente incomum e recidivante. Sua variante mais rara é conhecida como atrófica, tipo morfeia ou dermatofibrossarcoma não protuberante, sendo de difícil diagnóstico clínico. Não há critérios dermatoscópicos bem estabelecidos, portanto o diagnóstico é histopatológico. Existem avanços nas áreas da imuno-histoquímica e da genética que auxiliam no diagnóstico e excisão cirúrgica completa. Relatamos um caso de dermatofibrossarcoma atrófico em paciente de 27 anos, revisado na literatura quanto aos aspectos clínicos, dermatoscópicos, histopatológicos e genéticos.

Dermatofibrosarcoma protuberans is an uncommon and relapsing fibrohistiocytic tumor of intermediate malignancy. Its rarest variant is known as atrophic, morphea type or dermatofibrosarcoma non protuberans and its clinical diagnose is difficult. There are not well established dermatoscopic criteria, so the diagnosis is histopathologic. There are advances in immunohistochemical and genetic that help the diagnosis and complete surgical excision. It is reported a case of atrophic dermatofibrosarcoma in a 27 years patient ant it is reviewed the clinical, dermoscopic, histological and genetic aspects.

Squamoid eccrine ductal carcinoma

Abstract Squamoid eccrine ductal carcinoma is an eccrine carcinoma subtype, and only twelve cases have been reported until now. It is a rare tumor and its histopathological diagnosis is difficult. Almost half of patients are misdiagnosed as squamous cell carcinoma by the incisional biopsy. We report the thirteenth case of squamoid eccrine ductal carcinoma. Female patient, 72 years old, in the last 6 months presenting erythematous, keratotic and ulcerated papules on the nose. The incisional biopsy diagnosed squamoid eccrine ductal carcinoma. After excision, histopathology revealed positive margins. A wideningmargins surgery and grafting were performed, which again resulted in positive margins. The patient was then referred for radiotherapy. After 25 sessions, the injury reappeared. After another surgery, although the intraoperative biopsy showed free surgical margins, the product of resection revealed persistent lesion. Distinction between squamoid eccrine ductal carcinoma and squamous cell carcinoma is important because of the more aggressive nature of the first, which requires wider margins surgery to avoid recurrence.

Development of exclusively cutaneous sarcoidosis in patient with rheumatoid arthritis during treatment with etanercept / Desenvolvimento de sarcoidose exclusivamente cutânea em paciente com artrite reumatoide, durante tratamento com etanercepte

Summary We report the case of a patient with rheumatoid arthritis who, after 2 months of treatment with etanercept, showed disseminated asymptomatic violaceous papules. Biopsy of the skin lesion showed chronic granulomatous dermatitis with negative staining for fungi and acid-fast bacilli (AFB). After discontinuation of etanercept, the patient's condition improved. Although apparently paradoxical, cases of cutaneous and systemic sarcoidosis after anti-TNF medications have been reported in the literature, with very few cases presenting exclusive cutaneous involvement.

Resumo Relata-se caso de uma paciente com artrite reumatoide que, após 2 meses de tratamento com o medicamento imunobiológico anti-TNF-α etanercepte, apresentou quadro cutâneo compatível com sarcoidose. Notavam-se pápulas violáceas disseminadas e assintomáticas, cuja histopatologia revelou dermatite crônica granulomatosa, com pesquisa de fungos e bacilos álcool-ácido resistentes negativa. Após suspensão do etanercepte, houve regressão do quadro cutâneo. Apesar de paradoxal, têm sido relatados na literatura casos de sarcoidose cutânea e sistêmica após uso de medicações anti-TNF, sendo raríssimos os casos com acometimento cutâneo exclusivo.

Lichen planopilaris-like eruption during treatment with tyrosine kinase inhibitor nilotinib

Abstract Tyrosine kinase inhibitors are effective as a target therapy for malignant neoplasms. Imatinib was the first tyrosine kinase inhibitor used. After its introduction, several other drugs have appeared with a similar mechanism of action, but less prone to causing resistance. Even though these drugs are selective, their toxicity does not exclusively target cancer cells, and skin toxicity is the most common non-hematologic adverse effect. We report an eruption similar to lichen planopilaris that developed during therapy with nilotinib, a second generation tyrosine kinase inhibitor, in a patient with chronic myeloid leukemia resistant to imatinib. In a literature review, we found only one report of non-scarring alopecia due to the use of nilotinib.

Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence

Abstract Frontal fibrosing alopecia is a distinctive form of scarring alopecia considered to be a clinical variant of lichen planopilaris. It predominantly occurs in postmenopausal women and has a slowly progressive course. It was first described by Kossard in 1994. Since then the number of reported cases has increased significantly. Coexistence of frontal fibrosing alopecia and autoimmune disorders - such as discoid erythematosus lupus and Sjögren's syndrome - may suggest a common pathogenic background among the diseases.

Porokeratosis of Mibelli in an HIV-positive patient

Abstract Porokeratosis represents a group of disorders of epidermal keratinization that are characterized by one or more annular plaques surrounded by a histologically distinctive hyperkeratotic ridge-like border called the cornoid lamella. Many studies showed that organ transplantation and immunosuppression were associated in a significant number of cases. Furthermore, an association with squamous cell carcinoma and basal cell carcinoma has been noted in all variants of porokeratosis. The rarity of this disorder and its atypical clinical presentation – a single lesion on the thumb of an HIV-positive male patient – motivated this report.

Primary cutaneous aspergillosis and idiopathic bone marrow aplasia

Abstract: We describe the case of a 9-year-old boy with idiopathic bone marrow aplasia and severe neutropenia, who developed skin ulcers under cardiac monitoring electrodes. The diagnosis of primary cutaneous aspergillosis was made after the second biopsy and culture. Imaging investigation did not reveal internal fungal infection. The child was treated, but did not improve and died 3 months after admission. The report highlights and discusses the preventable risk of aspergillus skin infection in immunocompromised patients.