Erythema induratum of Bazin and active renal tuberculosis

typical of the Herlitz type of lEB is absent. The patients lose their nails in the first year of life and have severe caries. Patients with CJEB also have laryngeal involvement with "webbing" and nasal erosions. Our patient, however, did not have steno­ sis of the anterior nares like the three patients described by Haber et a1. The pedigree ofour patient indicates an autosomal recessive mode of inherit­ ance (Fig. n We found a normal expression of basement membrane zone components in the skin of our patient. The monoclonal marker for lethal JEB