Skin-nontumor / Clinical Dermatology
Elastosis perforans serpiginosum
Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 25 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.
● Rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements and cellular debris are expelled from papillary dermis through epidermis (eMedicine)
● Back of neck in teenage boys
● Either idiopathic, drug-induced (D-penicillamine), or associated with other inherited disorders (Downs syndrome, Éhlers Danlos syndrome, osteogenesis imperfecta, Marfan’s syndrome, Rothmund–Thomson syndrome)
● Clumps of coarse elastic fibers penetrate epidermis and cause focal epidermal hyperplasia
● Marked increase in elastic tissue in both reticular and papillary dermis
● The vertically oriented fibers of the papillary dermis are thicker than normal
● The center of the lesion shows characteristic transepithelial, parafollicular or transfollicular fibers that are straight, wavy or screw-like in configuration
● The perforating canal contains degenerated epithelial cells, inflammatory debris and numerous elastic fibers
● The epithelium on either side of the perforating canal is acanthotic, often with pseudoepitheliomatous hyperplasia
● Foreign body giant cell reaction is common in superficial dermis; occasionally elastophagocytosis is present
● Elastic stains (highlights elastic fibers)
● Reactive perforating collagenosis: transepidermal elimination of collagen
● Perforating folliculitis: transepidermal elimination of degenerative connective tissue
● Kyrle’s disease: transepidermal degeneration of parakeratin and inflammatory debris
End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Elastosis perforans serpiginosum
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