Lymphangiosarcoma of the scalp.

BACKGROUND: Lymphangiosarcoma is a rare, aggressive malignancy that originates from the endothelial cells lining lymphatic vessels and carries an extremely poor prognosis. Its clinical and histologic features are often indistinguishable from angiosarcoma. OBJECTIVE: We sought to better characterize the clinical and histologic features of lymphangiosarcoma. METHODS: Case report and review of the literature. RESULTS: A number of immunohistochemical markers, including Von Willebrand factor, Ulex europaeus agglutinin 1, CD31, VEGFR-3, D2-40, Prox-1, can be used to help differentiate lymphatic from vascular tissue. CONCLUSIONS: Recent characterization of several new biologic markers has allowed greater differentiation between these tumors and may provide new therapeutic targets for treatment.

Reticulated phototoxic eruption in a patient on long-term diltiazem therapy.

Diltiazem hydrochloride is a commonly prescribed medication in the treatment of cardiovascular disease. A case of diltiazem-induced hyperpigmentation in a patient after significant sun exposure is reported. The morphological appearance was reticulated and slate-gray to blue in color. The pathogenesis of the hyperpigmentation is discussed and possible treatment options are reviewed.

Severe radiation dermatitis is related to Staphylococcus aureus.

Acute radiation dermatitis commonly occurs following local radiation therapy for various cancers and, when severe, may necessitate disruption of treatment. Intense inflammatory reaction may result in a breakdown of the skin's barrier function and accompanying bacterial colonization. Bacterial superantigens may exacerbate inflammation through activation of T-cells and subsequent cytokine release. We report six cases of severe radiation dermatitis in cancer patients. Four of the six grew pathogenic bacteria, and three had psoriasiform or eczematous reactions at distant sites. Both the radiation dermatitis and the distant cutaneous reactions resolved rapidly on a combination of topical steroids and oral plus topical antibiotic therapy. We suggest that staphylococcal superinfection of acute radiodermatitis intensifies the inflammatory process and hinders repair of the epidermal barrier. Patients with acute radiation dermatitis should be investigated for secondary infection. We emphasize the importance of including topical and oral antibiotic therapy in conjunction with topical corticosteroids to eradicate infection as well as hasten repair of the skin's barrier function. These cases are presented to call attention to the role of Staphylococcus aureus in the pathogenesis of severe radiation dermatitis and the need to include appropriate antibiotic therapy based on culture in the management of acute radiation dermatitis.

Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis.

BACKGROUND: The potential association of dermal melanocytosis with lysosomal storage disease in infancy is an uncommonly known and poorly understood entity. OBSERVATIONS: We describe 2 infants with extensive dermal melanocytosis in association with GM1 gangliosidosis type 1 and Hurler syndrome, respectively. A literature analysis revealed 37 additional cases. Clinically, dermal melanocytosis associated with lysosomal storage disease is characterized by extensive, blue cutaneous pigmentation with dorsal and ventral distribution, indistinct borders, and persistent and/or "progressive" behavior. GM1 gangliosidosis type 1 and Hurler syndrome are the most common underlying disorders associated with these cutaneous features. CONCLUSIONS: In the appropriate clinical setting, an unusual presentation of dermal melanocytosis in an infant may be a cutaneous sign of an underlying lysosomal storage disease. The pathogenetic mechanisms behind this association remain to be elucidated.