Elastosis perforans serpiginosa

Synonym(s)

Elastosis perforans serpiginosum; elastosis intrapapillare; elastoma intrapapillare perforans; elastoma intrapapillare perforans verruciformis; elastosis perforans; elastoma verruciform perforans; keratosis follicu-laris et parafollicularis serpiginosa; keratosis follicularis serpiginosa; reactive perforating elastosis

Definition

Skin condition with abnormal dermal elastic tissue fibers and other connective tissue elements expelled via trans-epidermal elimination

Pathogenesis

Granulomatous inflammation displaying an atypical method for removing elastic tissue from the area of involvement

Clinical manifestation

Three subtypes:

Reactive form: associated with other diseases such as Down syndrome, Ehlers-Dan-los syndrome, Marfan syndrome, osteogen-esis imperfecta, scleroderma, acrogeria, pseudoxanthoma elasticum Drug-induced form: associated with peni-cillamine use

Idiopathic form (most common variety): flesh-colored or pale red, umbilicated papules grouped in linear, arciform, circular, or serpiginous patterns; most commonly occurring over the nape of the neck

Enchondromatosis with multiple cavernous hemangiomas

Differential diagnosis

Reactive perforating collagenosis; perforating folliculitis; Kyrle's disease; folliculitis; prurigo nodularis; granuloma annulare; tinea corporis; lupus erythematosus

Therapy

Tretinoin; isotretinoin; cryotherapy; elec-trodessication and curettage

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