Recent BP230-knockout experiments with subsequent blistering and recently identified plectin/HD1 mutations in epidermolysis bullosa simplex patients suggest that defective expression of BP230 and plectin/HD1 may predispose to blister formation in human skin. We have studied the expression of the epithelial adhesion complex as well as the basement membrane and anchoring fibril antigens in uninvolved dermatitis herpetiformis skin to find out if alterations can be detected in these structures predisposing to the blister formation typical of the disease. Ten uninvolved dermatitis herpetiformis skin specimens, which all showed clear granular deposits of IgA under the basement membrane in direct immunofluorescence and five normal skin specimens, were studied by indirect immunofluorescence technique. Six uninvolved dermatitis herpetiformis skin specimens showed distinctly decreased immunoreaction for BP230 and four uninvolved dermatitis herpetiformis skin specimens showed distinctly decreased immunoreaction for plectin/HD1. All five skin controls showed strong immunoreactions for BP230 and plectin/HD1. Other hemidesmosomal proteins including BP180 and integrin alpha6beta4, as well as basement membrane proteins laminin-5, laminin-1, nidogen and type IV collagen, and the anchoring fibril protein type VII collagen showed a normal strong expression. Our results suggest that alterations in BP230 and plectin/HD1 may contribute or predispose to blister formation in dermatitis herpetiformis skin.
Pituitary-adrenal function was investigated in 23 patients with rheumatoid arthritis and in 19 patients with bronchial asthma following prolonged glucocorticoid therapy. The duration of continuous treatment varied from 2 months to 15 years and the total dose of steroids from 400 mg to 54 g of prednisolone or its equivalent. After treatment had been discontinued, pituitary ACTH-reserve was estimated by means of an oral metyrapone test and adrenal response to ACTH by means of an intravenous or intramuscular ACTH-test. The pituitary ACTH-response was evaluated by determining the increment in the excretion of 11-deoxy-17-hydroxycorticosteroids during the administration of the metyrapone blocking agent and adrenal response to ACTH by the excretion of 11-oxygenated corticosteroids. The reduction of the metyrapone and ACTH responses were found to be related to the duration of prednisolone treatment as well as to the daily and total administered dose, although a marked individual variation in several instances could be observed in the degree of suppression of endogenous ACTH or corticosteroid production. Deficient metyrapone and ACTH responses were more frequently encountered in bronchial asthma than in patients with rheumatoid arthritis who had received comparable doses of glucocorticoids during similar periods. No negative metyrapone tests, however, in either group of patients were obtained for 12 months or unless the total dose of steroids administered was at least 3.5 g of prednisolone or its equivalent. Most patients who showed reduced adrenocortical response to ACTH had received large total doses of corticosteroids and the lowest total dose on which a subnormal ACTH-response was observed was 6 g. The results demonstrate that both pituitary ACTH-reserve and adrenocortical response to ACTH can be reduced by long-term adrenal steroid therapy, although the loss of pituitary ACTH-response occurs earlier during the course of treatment.
A 25-year-old Finnish male had had brownish-black nodules along the shafts of his scrotal hair for a period of 1 1/2 years. From the clinical appearance, piedra was suspected. Fungal culture demonstrated Trichosporum cutanum (T. Beigelii) and a diagnosis of white piedra was made. The patient has never been outside the boundaries of Finland.
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