Lipoid proteinosis: clinical, histopatthological and ultrastructural study

ABSTRACT

This study demonstrated the reduplication of basal laminae of basement membrane zone and thickened papillary dermis with a marked deposition of hyaline-like material interspersed between thin separated collagen fibrils. Immediately surrounding the blood vessel walls, hyaline deposits were seen together with the reduplication of basal laminae in an onion-like arrangement. The fibroblasts, epithelial cells of eccrine sweat glands and dermal histiocytes showed vacuolated cytoplasm with several types of lysosomes, i.e. empty with curved tubular profiles or farber-like inclusions. Some dermal nerve endings showed dystrophic axons with multiple dense laminated bodies in axoplasm