Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita.

A dystrophic bullous eruption that met all the criteria for epidermolysis bullosa acquisita (EBA) developed in a 19-year-old woman five years before the onset of clinical and serologic evidence of systemic lupus erythematosus (SLE). Electron microscopic studies of skin lesions both before and after the development of SLE were consistent with the previously reported electron microscopic findings in patients with EBA. Direct immunofluorescence microscopic studies done on bullae before and after the diagnosis of SLE showed linear depositions of immunoglobulin and complement; indirect immunofluorescence microscopic study findings consistently showed no abnormalities. These findings have been noted in other cases of EBA and may implicate autoimmune, immunologic factors in the pathogenesis of the disease process. To our knowledge, the finding of SLE in association with EBA has not been previously reported.

Lucio's phenomenon.

A 38-year-old woman had diffuse, nonnodular, lepromatous leprosy and Lucio's phenomenon. Most cases of Lucio's phenomenon have been reported to have a leukocytoclastic vasculitis as the underlying pathologic abnormality. In this patient, however, the histologic picture of an early lesion of Lucio's phenomenon showed a milk, mononuclear cell infiltration, endothelial swelling, vascular thrombosis, and ischemic necrosis. Lepra bacilli were abundant around nerves and blood vessels, and many were noted in vascular walls and endothelium. Our findings raise the possibility that some cases of Lucio's phenomenon may be caused by vascular damage due to direct invasion of Mycobacterium leprae and not necessarily by leukocytoclastic vasculitis.

Cutaneous manifestations of tumoral calcinosis.

We have followed up a large family in which seven members have tumoral calcinosis. One girl had the skin lesions of localized calcinosis cutis apart from the typical subcutaneous deposits of calcium. Like most persons with tumoral calcinosis, our patient had normal serum calcium concentrations; however, the serum phosphorus levels were greatly elevated. The familial occurrence and elevated serum phosphorus levels suggest the possibility of some as yet undefined, heritable metabolic defect as the underlying cause. The occurrence of tumoral calcinosis with localized calcinosis cutis is a rare association, and there has been only one other reported case to our knowledge. This report describes our patient and offers a brief discussion of tumoral calcinosis. The therapeutic response to the phosphate depletion regimen and topical steroids was disappointing in our case.