RESUMO O angiohistiocitoma de células multinucleadas (ACM) é uma entidade rara, cuja patogenia não está completamente compreendida. É mais comum em mulheres de meia-idade e caracteriza-se por pápulas ou nódulos eritematovioláceos, habitualmente assintomáticos e localizados predominantemente nas extremidades. O diagnóstico é fundamentalmente histopatológico, consistindo em alterações típicas na derme, nomeadamente na proliferação de pequenos vasos e na presença de células gigantes multinucleadas. A sua evolução é benigna, embora persistente, havendo poucos casos relatados de remissão espontânea. Apresenta-se o caso de um homem de 48 anos com diagnóstico de ACM, manifestando-se por pápulas e nódulos assintomáticos, agrupados no dorso de ambas as mãos, com um ano de evolução. O diagnóstico foi determinado pelo exame histopatológico, excluindo-se outros diagnósticos diferenciais. Com este relato de caso pretende-se contribuir para o conhecimento sobre esta patologia e destacar a complementaridade entre a observação clínica e a análise histopatológica.

ABSTRACT Multinucleated cell angiohistiocytoma (MCAH) is a rare clinical entity whose pathogenesis is not fully understood. It is more common in middle-aged women and is characterized by erythematoviolaceous papules or nodules, usually asymptomatic and predominantly located in the extremities. Histopathology is essential for making the diagnosis, consisting of typical alterations in the dermis, namely the proliferation of small vessels and the presence of multinucleated giant cells. Evolution is benign, although persistent, with few reported cases of spontaneous remission. We present the case of a 48-year-old man with MCAH, who exhibited asymptomatic papules and nodules grouped on the back of both hands, with a year of evolution. The diagnosis was made after histopathology, which excluded other differential diagnoses. With this case report, we intend to contribute to the knowledge about this entity and highlight the interconnection of clinical observation and histopathologic analysis.

Abstract Cutaneous and systemic plasmacytosis is a rare disorder characterized by cutaneous polyclonal plasma cell infiltration frequently associated with polyclonal hypergammaglobulinemia and lymphadenopathy. We report a case of a 67-year-old woman with an inflammatory ulcerated plaque in the left masseter region. A skin biopsy showed dense perivascular infiltrate of mature plasma cells in the dermis without atypia and immunoglobulin light chain restriction. After physical examination and further investigation, we ruled out systemic disease. Our patient was successfully treated only with hydrocortisone cream application. Few cases of isolated benign primary cutaneous plasmacytosis have been described, particularly in children. After excluding the diagnosis of a reactive process to an infection, which is unlikely in this case, we suspected of a rare manifestation of primary cutaneous plasmacytosis in adults with distinct presentation and clinical course.

Abstract Metastatic ovarian cancer uncommonly presents with skin metastasis. When present, skin metastases of ovarian cancer are usually localized in the vicinity of the primary tumor. We report a case of a 58-year-old woman with a rapid growing erythematous, well-defined nodule localized on the left nasal ala. A skin biopsy was performed and histopathological and immunohistochemical findings were compatible with a cutaneous metastasis of adenocarcinoma. A systematic investigation revealed a bilateral ovarian cystadenocarcinoma associated with visceral dissemination, likely associated with nose cutaneous metastasis. We report a very uncommon case because of the presentation of ovarian carcinoma as cutaneous metastasis. To our knowledge, this atypical localization on the nose has not been described yet in the literature.

Abstract Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

Several variants of dermatofibroma have been described. They are essentially distinguished by their clinical and histopathological features. To review the mainfeaturesof these variants, a retrospective study of skin biopsies and tissue excisions of dermatofibromasperformed in the dermatology and venereology service at the Hospital Garcia de Orta between May 2007 and April 2012 was carried out. During that period, 192 dermatofibromas were diagnosed in 181 patients, the lesions being more common in women. Median age of the study population was 48 years. The most common lesion site was the limbs (74% of patients). The histopathological types found were common fibrous histiocytoma (80%) and the aneurysmal (5.7%),hemosiderotic (5.7%), epithelioid (2.6%), cellular (2.1%), lipidized (2.1%), atrophic (1.0) and clear cell (0.5%) variants. Based on these findings, this review focuses on the clinical and histological features of the various variants of dermatofibroma in terms of their clinical presentation, distinct histopathological features, differential diagnosis and prognosis.