Papular Elastorrhexis: A Case and Differential Diagnosis

Abstract

Papular elastorrhexis is a rare cutaneous disorder that occurs predominantly during adolescence. The disorder is characterized by asymptomatic indurated white or flesh papules showing decreased and fragmented elastic fibers in the dermis. We herein report on a 12-year-old girl with multiple asymptomatic white, nonfollicular, firm papules scattered on the extremities and trunk. Histological examination revealed focal areas showing increased layers of collagen fibers and decreased and fragmented elastic fibers in the upper dermis.

Immunopathological and ultrastructural features in a case of papular elastorrhexis

“…Another case showed a reduction in epidermal melanin granules, while the number of S100-positive melanocytes was normal [ 5 ] ; in our case the number of Melan A-positive and S100-positive cells tended to be decreased. On the other hand, in keeping with previous reports [6][7][8][9] , we observed a perivascular lymphohistiocytic infi ltrate in the dermis. In our case, however, ultrastructural analysis showed the diminution and degeneration of fi broblasts and elastic fi bers as well as swollen collagen bundles, suggesting that the elastic tissue disorder might result from disorganized fi broblasts.…”

The utility of elastic Verhoeff‐Van Gieson staining in dermatopathology

“…Recent study by Revelles et al confirmed the absence of elastic fibers in PXE‐like papillary dermal elastolysis by antibodies to serum amyloid P that stains microfibrils of elastic fibers . Increased and thickened collagen fibers have been described in some cases of papular elastorrhexis …”

Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases

“…The most widely used classification system is the one proposed in 1980 by Uitto et al who divided the connective tissue nevi of the skin into 3 groups according to their predominant dermal component and the presence of specific genetic patterns of inheritance and abnormalities of other organs as well. The resulting categories of connective tissue nevus are collagenomas, elastomas and proteoglycan lesions, which consist of excess collagen, elastin and glycosaminoglycans, respectively and each include both acquired and inherited conditions . Recognizing these lesions is important as they may be part of syndromic disorders such as Proteus syndrome, tuberous sclerosis, Buschke‐Ollendorf syndrome, Goltz syndrome, MEN1 and Cowden disease .…”