Pathologic features of XXXX thick skin.

@entity1 @entity6 @entity1711 @entity2631 @entity1723 @entity1710 @entity631 @entity785
Skin thickening simulating @entity2631 , or progressive @entity785 , has previously been reported in @entity1 and adults with @entity1723 . We have studied eighty-nine @entity1 with @entity1723 and twenty-five normal control subjects. Clinical evidence of skin thickening ( @entity6 thick skin) was found in 22% of @entity1 with @entity1723 and in 4% of control subjects (p less than 0.05). Full-thickness skin biopsy specimens were taken from the forearm of nine @entity1 with @entity1723 and @entity6 thick skin, four @entity1 with @entity1723 and clinically normal skin, four @entity1 with progressive @entity785 , and four normal control subjects. The sections stained with @entity1710 and @entity1711 showed increased thickness of the dermis of the forearm skin in all @entity6 @entity1 . In @entity6 thick skin the collagen bundles were large, disorganized, and separated by clear spaces. Small amounts of acid mucopolysaccharides were present in the upper reticular dermis of five @entity1 with @entity6 thick skin. Electron microscopy of the dermis showed @entity631 membrane thickening in Groups 1, 2, and 3. All @entity1 with @entity6 thick skin showed active fibroblasts and extensive collagen polymerization in the rough endoplasmic reticulum. Occasional collagen flowers were noted in all @entity1 with @entity6 thick skin. Measurements of 100 collagen fibers in the upper and lower reticular dermis of each biopsy specimen showed predominance of large fibers (greater than 60 nm) in Groups 1 and 2. Unlike @entity2631 , @entity6 thick skin resulted in small fiber sizes (less than 60 nm) only rarely, and bimodality of fiber sizes was not seen.(ABSTRACT TRUNCATED AT 250 WORDS)
