Background: Calciphylaxis is a life-threatening form of metastatic calcification-induced microvascular occlusion syndrome. Although traditionally observed in patients with end-stage renal disease and/or hyperparathyroidism, the development of calciphylaxis in "nontraditional" patients having both normal renal and parathyroid function has been reported. However, to date there has been no collective analysis identifying common patient characteristics potentially predisposing to the development of calciphylaxis in nontraditional patients.Observations: A 58-year-old woman with endometrial carcinoma developed extensive calciphylaxis despite the presence of normal renal and parathyroid function. The disease resolved with rapid diagnosis, supportive therapy, and medical management. Analysis of this case and the 13 previously reported cases of nontraditional calciphylaxis identified the following patient characteristics that highlight clinical situations potentially predisposing to calciphylaxis: hypoalbuminemia, malignant neoplasm, systemic corticosteroid use, anticoagulation with warfarin sodium or phenprocoumon, chemotherapy, systemic inflammation, hepatic cirrhosis, protein C or S deficiency, obesity, rapid weight loss, and infection.Conclusions: Calciphylaxis is becoming increasingly common in patients with normal renal and parathyroid function. The observations from this study may assist dermatologists in the rapid diagnosis and prompt initiation of therapy for this devastating disease.
A 57-year-old woman presented with refractory genital erosive disease. One year earlier she experienced gingival fragility; direct immunofluorescence resulted in the diagnosis of cicatricial pemphigoid, and prednisone therapy led to initial improvement. Initial skin biopsy of her genital erosions demonstrated full-thickness ulceration with viral cytopathic change and a re-epithelializing subepidermal separation. Indirect immunofluorescence revealed intercellular IgG staining on monkey oesophagus at a titre of 1:320 consistent with pemphigus, leading to the diagnoses of pemphigus vulgaris with herpetic superinfection. Immunosuppressive treatment initially led to improvement; however, disease subsequently recurred as extensive genital erosions. We diagnosed atypical herpes simplex virus infection and oral candidiasis, discontinued all immunosuppressive medications, and initiated antiviral and antifungal therapy. Dramatic resolution was observed and the patient has remained free of disease for 13 months while taking only prophylactic famciclovir.
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