atrophoderma vermiculata

(redirected from Atrophoderma Vermiculatum)

atrophoderma vermiculata

A rare autosomal recessive symmetric dermatopathy (genodermatosis) (OMIM:209700) characterised by skin atrophy with sharply demarcated “pits”, variably accompanied by cardiac defects, mental retardation and neurofibromas.

Clinical findings
Usually presents in childhood as an inflammatory follicular atrophy with “worm-eaten” or “honey-combed” reticular atrophy of the skin localised to the cheeks, pre-auricular regions and temples, less commonly on the extensor surfaces of the arms and legs, resulting in follicular atrophic scars which may be accompanied by generalised facial erythema, sparse open and closed comedones, and milia.
 
Prognosis
Usually slowly progressive; spontaneous regression has been reported.
 
Management
Dermabrasion, cryotherapy, UV light radiation, topicals, CO2 and 585 nm pulsed dye lasers.
References in periodicals archive ?
Papules and cysts around the elbow, pitted scars over the healed lesions, atrophoderma vermiculatum and hypo pigmented streaks over the scalp can be associated with APL (7).
Atrophoderma vermiculatum is a rare follicular disorder primarily affecting children with reticular or honeycomb atrophy of the cheeks and forehead.