S8C32P18HyalineAngiopathy

S8C32P18-1: In the category of the vascular hyalinoses, lipoid proteinosis and erythropoietic protoporphyria (EPP) are distinctive, but are remarkably similar. In this example of EPP, the vessels of the dermis are tortuous and, focally, lumens appear to be duplicated. The walls of vessels are thickened and hyalinized. Near the dermal-epidermal interface, some of the deposits are globular.



S8C32P18-2: At higher magnification, the vessels have thickened walls and narrowed lumens. Some of the vessels, in cross-section, show reduplication of basement membranes. Some of the membranous portions are coarsened and hyalinized. In addition, at the peripheries of the vessels, there are coarse, hyaline lamellae.

S8C32P18-3: At higher magnification, the hyaline deposits contain loosely spaced, delicate fibrils. At the point of the red arrow, some of the perivascular hyalin is globular and brightly acidophilic (proteinaceous) ( EPP); this type of change might be characterized as “exudative.”

S8C32P18-4: Focally, some of the hyalin in the wall of small vessels is globular and brightly acidophilic (the qualities are those of a proteinaceous deposit, but the tinctorial qualities are those of fibrinoid). The matrix among the hyaline lamellae has mucoid qualities. Endothelial cells are swollen; some have vacuolated cytoplasm. In the vessel to the right, the swollen endothelial cells have obliterated the lumen of the vessel (EPP).

S8C32P18-5: There are prominent, concentrially laminated and confluent deposits of hyalin in the perivascular spaces. Focally, some of the hyalin is brightly acidophilic (if the acidophilic deposits are compared to glomerular lesions, the bright red deposits might be characterized as exudative lesions) (EPP).

S8C32P18-6: The perivascular changes of lipoid proteinosis are similar to those of EPP. In this field of a lesion of lipoid proteinosis, concentric hyalin lamellae (in basement membrane-like arrangements) are prominent about sweat glands. Sweat gland involvement of this type is more characteristic of a lesion of lipoid proteinosis.

S8C32P18-7: In this area of a lesion of lipoid proteinosis, a portion of the sweat gland apparatus is cut in longitudinal section. The epithelial structure shows early, epithelial atrophy (compression atrophy?). The hyaline deposits are membranous and confluent. Focally, collagen bundles of the reticular dermis are entrapped in the hyalin.

S8C32P18-8: With this fluorescent technique for the demonstation of lipid deposits, the vessels in this lesion of lipoid proteinosis show positive deposits of lipids.

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