Segmental stiff skin syndrome (SSS): Clinical case and a brief review.

Stiff skin syndrome (SSS) is a rare, scleroderma-like condition that is commonly characterised by stony hard skin and limited joint mobility, in the absence of visceral involvement or immunologic abnormalities. Depending on the distribution of the disease, this disorder can be further categorised into classic (widespread) SSS or its newly described segmental variant. Additional features of this syndrome may include hypertrichosis, lipodystrophy, dysmetria and scoliosis. In this report, we present the case of a patient with segmental SSS and we briefly review the current literature about the topic.

Photoletter to the editor - Calciphylaxis: a diagnostic and therapeutic challenge.

Calciphylaxis is an uncommon necrotizing dermal condition, most often related to end-stage renal disease, associated with secondary hyperparathyroidism. The incidence is 1-4% per year in dialyzed patients. The condition is characterized by microcalcification of small and medium-sized blood vessels, causing cutaneous and soft tissue necrosis. The etiology of calciphylaxis is poorly understood, although many reports highlight the abnormal calcium-phosphate products as an important etiological factor. Calciphylaxis is associated with significant morbidity and mortality with estimated 5-year survival rates of less than 50%. Sepsis is the main cause of death. We report two patients of calciphylaxis treated with surgical debridement and excellent outcome in both cases.

Photoletter to the editor: Botryomycosis in an immunocompetent woman.

Botryomycosis is a chronic, granulomatous infection of the skin, soft tissue and viscera. It is uncommon and mainly occurs in immunocompromised patients. Splendore-Hoeppli phenomenon represents an immunological host reaction to antigens of infectious and non-infectious agents. We report a case of a 64-year-old immunocompetent woman presented at our department with a 2-month history of abdominal papules and discharging nodules. She was treated with several antibiotics with no success. Skin biopsy showed granular bodies in the dermis with a Splendore-Hoeppli phenomenon. Microbiologic study isolated a Staphylococcus aureus. Patient was successfully treated 4 weeks with oral clindamycin 300 mg bid. Lack of drug penetration into the "encapsulated-like" microcolonies could explain the therapeutic challenge of this case.

Um caso de liquen mixedematoso e cancro da mama / A case of lichen myxedematosus breast carcinoma

No disponible