Cutaneous leiomyosarcoma: a 20-year retrospective study and review of the literature

Abstract Background: Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. Objectives: The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years. Methods: A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted. Results: Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (n = 7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (n = 5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months. Study limitations: This was a retrospective study based on medical and pathological records. Conclusions: Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.

Spitz nevus and infliximab: association or coincidence?

Abstract Biological therapies, including anti-TNF agents, are important in the treatment of various chronic inflammatory diseases, including psoriasis, rheumatoid arthritis or inflammatory bowel disease. The increased use of these drugs translates into an increasing awareness of its adverse effects, which include malignancy. In this paper, we describe the case of a 28-year-old woman who developed a spitzoid melanocytic tumor after starting infliximab therapy for ulcerative colitis. The evidence for causality between anti-TNF and melanocytic proliferations is still sparse; nonetheless, treatment-associated immunosuppression seems to play a key role in this phenomenon. Therefore, a regular follow-up with a rigorous skin examination is essential in these patients. Noninvasive techniques such as dermoscopy or reflectance confocal microscopy are particularly useful diagnostic tools in these circumstances.

Dermatoses associadas ao trabalho em profissionais de saúde de um centro hospitalar português / Occupational dermatoses among healthcare workers in a hospital center in Portugal

Introdução: As patologias cutâneas representam mais de 35% das doenças relacionadas com o trabalho, afetando anualmente 1/1.000 trabalhadores. Objetivos: Caracterizar as dermatoses associadas ao trabalho em profissionais de um centro hospitalar e identificar possíveis agentes desencadeantes e fatores de suscetibilidade. Métodos: Estudo transversal com recolha da informação registrada no processo clínico eletrônico dos trabalhadores observados em exame de saúde periódico no período de um ano. Resultados: Nos 1.741 trabalhadores estudados, 3,56% tinham registro de dermatoses associadas ao trabalho, a maioria mulheres (76,85%). Dos profissionais com dermatoses, 34 (54,84%) tinham dermatite de contato irritativa, 17 (27,42%) urticária ao látex, seis (9,68%) dermatite de contato alérgica e cinco (8,06%) duas dermatoses concomitantes. Encontrou-se diferença estatisticamente significativa entre os vários grupos profissionais (p=0,008), sendo os auxiliares de enfermagem os mais afetados (5,11%). Verificou-se também maior prevalência (8,47%) em profissionais de blocos operatórios (p=0,001). Os principais agentes desencadeantes foram desinfetantes cutâneos, látex, luvas de nitrilo e contato prolongado com água (4,84%). Conclusões: Por tratar-se de um estudo baseado em registros clínicos, a informação está dependente da qualidade destes. Para além disso, os auxiliares de enfermagem foram a maioria representada na amostra no que se refere à população do centro hospitalar, o que limita a extrapolação dos resultados. Este estudo encontrou prevalência de dermatoses de somente 3,56%, o que talvez se deva à implementação de medidas preventivas. Os profissionais mais afetados foram os de blocos operatórios e os auxiliares de enfermagem. Os desinfetantes cutâneos foram os principais agentes apontados como desencadeantes.

Background: Skin diseases account for more than 35% of occupational diseases, affecting 1/1,000 workers annually. Objective: To characterize occupational dermatoses affecting hospital workers and identify possible triggers and susceptibility factors. Methods: Cross-sectional study in which we analyzed information extracted from electronic medical records of workers who performed periodic examinations in the course of one year. Results: About 3.56% of 1,741 included workers had a diagnosis of occupational dermatosis, being mainly women (76.85%). Thirty-four (54.84%) of the affected workers had irritant contact dermatitis, 17 (27.42%) latex allergy, 6 (9.68%) allergic dermatitis, and 5 (8.06%) two concomitant conditions. We found significant difference in prevalence as a function of occupational group (p=0.008), being highest for nursing assistants (5.11%). Prevalence was also higher for employees allocated to surgery departments (8.47%, p=0.001). Main triggers were skin disinfectants, latex, nitrile gloves, and prolonged contact with water (4.84%). Conclusion: The quality of the analyzed data depends on the quality of the analyzed medical records. Most subjects were nursing assistants, which fact hinders the generalization of the results. The prevalence of occupational dermatosis was just 3.56%, which might be explained by previously implemented preventive measures. The employees most frequently affected were those allocated to surgery departments and nursing assistants. Skin disinfectants were the most frequent triggers.

Multifocal primary cutaneous extranodal NK/T lymphoma nasal type

Abstract Nasal type extranodal NK/T-cell lymphoma is a distinct entity according to the World Health Organization classification. Although 60% to 90% of patients with this disease present with a destructive mass in the midline facial tissues, it may also primarily or secondarily involve extranasal sites, like the skin. We report the case of a 77-year-old patient that came to our department with erythematous plaques of the right leg and eczematous lesions of the trunk. These lesions were biopsied and the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He was treated with multi-agent systemic chemotherapy but died 5 months after diagnosis. This case highlights the rarity and variability of cutaneous features of this disease and its aggressive course and poor prognosis.

Autoimmune thyroiditis presenting as interstitial granulomatous dermatitis / Dermatite intersticial granulomatosa como apresentação de tireoidite autoimune

A 54-year-old female presented with recurrent, widespread, erythematous, painful plaques, over a 3-month period. Skin biopsy was compatible with interstitial granulomatous dermatitis. Additional investigation revealed hypothyroidism and positive anti-thyroid antibodies. Normalization of thyroid function and high-potency topical corticosteroids provided only transitory improvement of the dermatosis. Interstitial granulomatous dermatitis is a histologic inflammatory reaction, with variable cutaneous expression. It has been reported in association with several drugs, lymphoproliferative diseases and autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus and vasculitis, but association with autoimmune thyroiditis is rare. Optimal therapy for this condition is yet to be established, but topical corticosteroids have been a mainstay of treatment. In most cases, this disease is characterized by flares and remissions.

Uma doente de 54 anos foi avaliada por placas eritematosas, dolorosas, disseminadas, recorrentes, com 3 meses de evolução. A biopsia cutânea foi compatível com dermatite intersticial granulomatosa. Os restantes exames laboratoriais revelaram hipotiroidismo e anticorpos anti-tiroideus positivos. Apesar da normalização da função tiroideia e de tratamento com corticóide tópico de alta potência, a dermatose melhorou apenas parcialmente. Dermatite intersticial inflamatória é um diagnóstico histopatológico, com expressão clínica variável. Tem sido associada a vários fármacos, doenças linfoproliferativas e autoimunes, nomeadamente artrite reumatóide, lupus eritematoso sistémico e vasculites, mas a associação com tireoidite autoimune é rara. Até ao momento, não foi definido nenhum tratamento específico, mas os corticóides tópicos são dos fármacos mais utilizados. A doença caracteriza-se por períodos de agravamento e remissão.

Recurrence of peripheral T-cell lymphoma as granulomas in the lower limbs / Granulomas epitelióides cutâneos, como manifestação de recidiva de linfoma de células T periférico

The presence of granulomas in the skin of T-cell lymphoma patients is a rare but well-known phenomenon. The authors present the case of a 44-year-old Caucasian male, with a previously treated peripheral T-cell lymphoma, with cutaneous infiltration and extensive dyschromia on his lower limbs. Skin biopsies revealed the presence of sarcoid granulomas associated with the recurrence of the lymphoma, confirmed by immunostaining and molecular analysis. Although a new scheme of chemotherapy was started, he died 18 months later. There are two different patterns of skin granulomas associated with lymphoma: granulomatous infiltrates admixed with neoplastic cells and cutaneous granulomas constituting a nonspecific manifestation of the underlying lymphoma, but presently there is no evidence supporting their relationship with lymphoma prognosis.

A presença de granulomas cutâneos associados a linfomas é um fenômeno raro, mas bem conhecido. Um homem, 44 anos de idade, com diagnóstico prévio de linfoma de células T periférico, foi enviado à nossa consulta por infiltração cutânea e extensa discromia nos membros inferiores. A biopsia cutânea revelou a presença de granulomas epitelioides associados à recidiva do linfoma, confirmada pela marcação imuno-histoquímica e estudo molecular. Apesar de iniciado novo esquema de quimioterapia, o doente faleceu 18 meses depois. Na literatura são descritos dois tipos de granulomas cutâneos na presença de linfomas: associados à infiltração cutânea pelo linfoma ou como uma manifestação não específica do linfoma. Contudo, atualmente não há evidência que suporte a sua relação com o prognóstico dos linfomas.

Leiomiossarcoma cutâneo no tronco / Cutaneous leiomyosarcoma on the trunk

O Leiomiossarcoma cutâneo é um tumor maligno que representa 7 por cento dos sarcomas dos tecidos moles, afetando, mais frequentemente, homens entre os 50-70 anos. A localização, no tronco, é atípica, constituindo 10 a 15 por cento dos casos. A radioterapia e o traumatismo prévio têm sido referidos como fatores de risco. Descrevemos um homem de 57 anos, com tumor eritemato-violáceo, indolor, de consistência dura, localizado na região pré-esternal, com cerca de oito meses de evolução. A histopatologia evidenciou tumor maligno de células fusiformes, com núcleos em forma de "charuto", pleomórficos, com alto índice mitótico, ocupando toda a espessura da derme. Estas células expressaram actina do músculo liso, desmina e vimentina e foram negativas para proteína S-100 e pancitoqueratina. Foi efetuada a exérese cirúrgica radical do tumor.

Cutaneous leiomyosarcoma is a relatively uncommon tumor that accounts for 7 percent of all soft tissue sarcomas. It occurs more frequently in males between 50-70 years and only 10-15 percent of cases are located on the trunk. Radiotherapy and previous trauma have been implied as risk factors. We report the case of a 57 year-old male with an eight-month history of a hard painless erythematous-violaceous tumor on the presternal region. Histopathology evidenced a malignant spindle cell tumor, "cigar" shaped, with pleomorphic nuclei and a high mitotic index that occupied the entire dermal thickness. Immunohistochemical staining of the tumor cells was positive for smooth muscle actin, desmin and vimentin and negative for S-100 protein and pan-cytokeratin, which supported the diagnosis of dermal leiomyosarcoma. Radical surgery was performed to remove the tumor.

A importância das provas epicutâneas de contacto no diagnóstico diferencial de reações a medicamentos / The importance of patch tests in the differential diagnosis of adverse drug reactions

O eritema exsudativo multiforme é uma erupção aguda, autolimitada, frequentemente associada a infecções (geralmente virais), doenças sistêmicas e fármacos. Apresenta-se o caso de uma mulher de 39 anos, com o diagnóstico de lúpus eritematoso sistêmico, que recorreu à Urgência com quadro de eritema exsudativo multiforme, com início 10 dias após tomar amoxicilina e ácido clavulânico por amigdalite e, quase simultaneamente, receber a vacina antipneumocócica. Colocou-se também a hipótese de síndrome de Rowell. Efetuaram-se testes epicutâneos de contacto com bateria básica (portuguesa) e princípios ativos dos fármacos suspeitos (Chemotechnique®). Encontrou-se hipersensibilidade à amoxicilina 10 por cento vas (++), à ampicilina 10 por cento vas (++) e à penicilina G potássica 10 por cento vas (+), atribuindo-se à amoxicilina a causa mais provável do eritema exsudativo multiforme.

Exudative erythema multiforme is an acute self-limited skin disease often associated with infections (usually viral), and also with systemic diseases and drugs. We report the case of a 39-year-old woman diagnosed with systemic lupus erythematosus, who presented at the emergency clinic with exudative erythema multiforme which started 10 days after taking amoxicillin and clavulanic acid for tonsillitis together (almost simultaneously) with the pneumococcal vaccine. Rowell's syndrome was also considered to be a possibility. Skin patch tests were carried with the standard battery of patches (GPEDC) and the active ingredients of the suspected drugs (Chemotechnique ®), with readings at D2 and D3. The tests were positive for amoxicillin 10 percent pet (++), ampicillin 10 percent pet (+ +) and penicillin G potassium 10 percent pet (+). We accepted the diagnosis of erythema multiforme due to amoxicillin, confirmed by patch testing.