Orlistat-induced bullous leukocytoclastic vasculitis.

Cutaneous leukocytoclastic vasculitis (LCV), also known as small-vessel vasculitis, is a process thought to be related to the presence of circulating immune complexes. Leukocytoclastic vasculitis is thought to be idiopathic in up to 50% of cases, but other common causes and associated disorders include certain medications, most frequently antibiotics; infections; collagen-vascular disease; paraproteinemias; and rarely neoplasia. We report a patient with cutaneous LCV induced by orlistat, a pancreatic lipase inhibitor that works as a weight-loss agent by decreasing the absorption of dietary fat.

Localized Darier's disease in a Blaschkoid distribution: two cases of phenotypic mosaicism and a review of mosaic Darier's disease.

Darier's disease is a rare autosomal dominant genodermatosis. Characteristic skin and nail changes consist of bilateral crusted red-brown papules and plaques in a seborrheic distribution, and V-shaped nicking or longitudinal ridging of the nails. In limited cases, a segmental distribution of this disease may be present in which localized lesions, found along the lines of Blaschko, are otherwise indistinguishable from those of generalized Darier's disease. Genomic mosaicism in localized lesions of Darier's disease is a new concept that was recently demonstrated in individuals of mosaic phenotypes. Both type 1 and type 2 mosaic phenotypes have been reported. We report two cases of type 1 localized Darier's disease in a Blaschkoid distribution and review the genetic implications of phenotypic mosaicism within the field of Darier's disease.

Mid-dermal elastolysis preceded by acute neutrophilic dermatosis.

BACKGROUND: Mid-dermal elastolysis is a rare idiopathic elastic tissue disorder that is characterized by localized patches of finely wrinkled skin and a "band-like" loss of elastic tissue in the mid-reticular dermis. Lesions may be preceded by erythema and/or urticaria, and histological examination of inflamed lesional skin may demonstrate lymphohistiocytic dermal infiltration. CASE REPORT: We report a case of mid-dermal elastolysis in a 31-year-old woman who developed multiple erythematous and urticarial plaques on the arms and trunk. Histologic examination of a representative lesion revealed a neutrophilic infiltrate and a normal pattern of elastic tissue. Several months later, the erythema and urticaria was noted to have resolved, leaving soft, pendulous plaques with overlying finely wrinkled skin. A follow-up biopsy at this time showed minimal lymphocytic inflammation but almost complete absence of elastic tissue in the mid-reticular dermis. CONCLUSIONS: To our knowledge, acute neutrophilic dermatosis resulting in mid-dermal elastolysis has not been previously described. This observation lends support to an emerging theory that the pathogenesis of mid-dermal elastolysis may be inflammatory.

Type I cryoglobulinemia presenting as hemorrhagic crusted leg ulcers.

Cryoglobulins (CGs) are serum proteins that reversibly precipitate when the serum is cooled below 37 degrees C. Cryoglobulinemias are associated with a variety of diseases, including hematologic, autoimmune, and infectious. Isolation of CGs requires handling the specimen with extreme care. We describe a 70-year-old man, recently diagnosed with chronic lymphocytic leukemia, who developed hemorrhagic crusted skin ulcers on his legs that were pruritic and painful. Results of skin biopsies showed dilated superficial, mid-dermal and deep-dermal blood vessels containing pink amorphous material and red blood cells. Cryoglobulinemia was suspected; however, an initial search for CGs was negative. There was concern about suboptimal handling of the specimen, and the test was repeated. Two percent CGs with IgM kappa specificity were detected. This case illustrates the importance of the proper handling of specimens for evaluation of cryoglobulinemia.

Calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.

Calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.