A case of lipoid proteinosis was subjected to clinical, histologic and electron microscopic study. Palpebral biopsies showed a large dermal infiltration composed of closely interwoven granulo-filamentous material in the superficial and deep dermis. Collagen fibers were normal. Fibroblasts seemed to play an active role in the synthesis of this accumulating substance. Around the dermal capillaries there was a prominent concentric multilamination of the basal lamina which was considered a reaction of the endothelial cells to the irritation caused by compression of the accumulating substance.

Type II fucosidosis in an autosomal recessive disease. The paper presents a case of a patient with alpha-L-fucosidase of whom a skin specimen was examined under the electron microscope. Storage material was observed mainly in endothelial cells of blood capillaries and Schwann cells surrounding small peripheral nerves of papillary dermis. Within both cells two different kinds of inclusions were revealed: (1) clear vacuoles and (2) dense bodies with an internal structure prevalently lamellar. All these ultrastructural alterations were observed long before the appearance of clinically defined angiokeratoma at cutaneous level. Hence, they present the same alteration found in the absence of angiokeratoma in type I fucosidosis.