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Skin-nontumor / Clinical Dermatology

Other dermatoses

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.

Description
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● 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

Epidemiology
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● 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)

Clinical images
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Various images

Micro description
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● 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

Micro images
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Diagram

   
Various images

Positive stains
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● Elastic stains (highlights elastic fibers)

Differential diagnosis
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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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