atrophoderma vermiculata

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.