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.
A 64-year-old woman presented at our department with a 2-month history of inflammatory papules and nodules mainly localized to the abdomen (Fig. 1). Some of these nodules showed a discharge of purulent fluid. She denied previous traumas or insect bites. Medical past history was irrelevant. HIV and HCV were negative. Patient was previously unsuccessfully treated by her assistant physician with flucloxacillin and cephradine (duration and dosages unknown). Findings from routine blood testing, including leukocytes, neutrophil count and C-reactive protein, were normal. A punch biopsy specimen from a nodule was submitted for histopathologic evaluation and another one was submitted DOI: http://dx.doi.org/10.3315/jdcr.2013.1134 29 PHOTOLETTER TO THE EDITOR AbstractBotryomycosis 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. (J Dermatol Case Rep. 2013; 7(1): 29-30) Key words: botryomycosis, actninophytosis, Splendore-Hoeppli phenomenon Figure 1Abdominal pus discharging papules and nodules.
Calciphylaxis is a rare vasculopathy that is characterized by ischemia and necrosis due to calcification, intima fibroplasia and thrombosis of dermal and pannicular arterioles. It has been called a "metastatic-like calcification". Usually calciphylaxis affects patients with end-stage renal disease, with an incidence of 1-4% of dialysisdependent individuals. 1 There are scarce reports of calciphylaxis in patients with normal renal function and with calcium and phosphorus serum levels within normal range. 2 The pathogenesis of calciphylaxis is poorly understood but hyperparathyroidism (primary or secondary to renal failure), diabetes, obesity, warfarin treatment, protein C or S deficiency are well recognized risk factors. Clinically, calciphylaxis presents as purpuric plaques with spontaneous ulceration and necrosis that often progress to deep tissue necrosis. It is associated with significant morbidity and mortality. It is estimated that 5-year survival is less than 50%. 3 Sepsis, originating in the skin lesions is the main cause of mortality. 69 PHOTOLETTER TO THE EDITOR AbstractCalciphylaxis 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. (J Dermatol Case Rep. 2013; 7(2): 69-70) Key words: calciphylaxis, calcium, kidney failure, surgical debridement, treatment, ulcer Figure 1 Extensive lesion of necrotic tissue on the left lower leg surrounded by livedo racemosa-like purpura.
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