Scleroderma en coup de sabre treated with polymethylmethacrylate - Case report

Abstract The scleroderma en coup de sabre is a variant of localized scleroderma that occurs preferentially in children. The disease progresses with a proliferative and inflammatory phase and later atrophy and residual deformity, which are treated with surgical techniques such as injectable fillers, transplanted or autologous fat grafting and resection of the lesion. Among the most widely used fillers is hyaluronic acid. However, there are limitations that motivate the search for alternatives, such as polymethylmethacrylate, a permanent filler that is biocompatible, non-toxic, non-mutagenic and immunologically inert. In order to illustrate its application, a case of scleroderma en coup de sabre in a 17-year-old patient, who was treated with polymethylmethacrylate with excellent aesthetic results, is reported.

Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil

The main treatment for pemphigus vulgaris are systemic corticosteroids and immunosuppressive agents, but due to adverse reactions and therapeutic failure, new drugs such as rituximab and mycophenolate mofetil have been used. In this case report are described two cases of severe pemphigus vulgaris refractory to various treatments, with resolution after use of rituximab and mycophenolate mofetil, associated with corticosteroids. A higher-than-usual dose of rituximab was employed, without the occurrence of serious adverse reactions. Mycophenolate mofetil was added as adjunctive therapy due to lack of response to azathioprine.

Case for diagnosis

Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor of intermediate malignancy with aggressive localized growth, high recurrence rate, but low metastatic potential. It appears as a hardened plaque, with slow growth, upon which the development of nodules occurs. It predominates in the trunk and is unusual in acral locations. Histopathology reveals spindle cells with storiform pattern and cartwheel-like or whirlwind-like aspect. Immunohistochemistry shows positivity for CD34. The treatment is surgical. We report a case of long evolution, with an unusual location, that relapsed after surgery, to emphasize the importance of early diagnosis and proper treatment, avoiding aggressive resections with increased morbidity.

Case for diagnosis / Caso para diagnostico

The chondroid syringoma is a rare benign tumor, also called mixed cutaneous tumor by the presence of epithelial and mesenchymal components, consisting of sweat elements in cartilaginous, collagenous, myxoid or osseous stroma, among others. It mainly affects middle-aged men and is characterized by asymptomatic and slowgrowing, dermal or subcutaneous nodules. The most common locations are the head and neck. It is rare on the extremities. There are reports of malignant variants predominantly in women, located on the extremities. We report a case of a female patient with a lesion on the toe, with excellent outcome after surgical treatment.

O siringoma condroide é um tumor benigno, raro, também chamado de tumor misto cutâneo pela presença de componentes epiteliais e mesenquimais, que consistem em elementos sudoríparos em estroma cartilaginoso, colagênico, mixoide ou ósseo, entre outros. Acomete, principalmente, homens de meia-idade e caracteriza-se por nódulos subcutâneos ou dérmicos, assintomáticos e com crescimento lento. As localizações preferenciais são a cabeça e o pescoço, sendo raro nas extremidades. Existem relatos de variantes malignas, com predomínio em mulheres, localizadas nas extremidades. Relatamos um caso em paciente do sexo feminino, com lesão em extremidade e excelente resultado após o tratamento cirúrgico.