Journal of the American Academy of Dermatology
-
A 61-year-old man with prurigo nodularis subsequently developed bullous pemphigoid. Direct immunofluorescence studies of a prurigo nodularis-like lesion and peribullous skin showed the deposition of IgG, IgA, and C3 in a linear pattern at the basement membrane zone. ⋯ Immunoelectron microscopy revealed IgG, IgA, and C3 distributed in the lamina lucida and on the undersurface of the basal keratinocytes in both peribullous and prurigo nodularis lesions. These findings confirmed the diagnosis of pemphigoid nodularis.
-
J. Am. Acad. Dermatol. · Nov 1989
Case ReportsCongenital fascial dystrophy: stiff skin syndrome--a human counterpart of the tight-skin mouse.
Four patients are described with stony-hard induration of the skin and deeper tissues, most pronounced on the buttocks, thighs, and legs, and with limitation of joint mobility and contractures of the lower limbs. Two patients were siblings and one was the product of a consanguineous marriage. The disorder appears to be genetically determined, but the mode of inheritance has not been established. ⋯ Except for functional impairment of the lungs caused by an underdeveloped thorax that resulted from pressure of the thickened thoracic fascia, there was no involvement of the viscera or muscles and no immunologic abnormalities. The most important finding was markedly thickened fascia. This hereditary connective tissue disorder has all the characteristics of the tight-skin mouse.