Atrophoderma vermiculatum L90.81

Sporadically occurring, chronic (harmless) disease pattern under several names. Clinically, in children and adolescents, the smallest wormlike, sharply retracted follicular scars (gaping follicles) with unknown etiology are found on the cheeks, usually symmetrically located on both sides. On close inspection, scattered follicular keratoses can often still be detected (see figure). The majority of authors interpret the clinical picture as a scarred final state of "atrophying" keratosis pilaris (faciei) (see also the explanations on the erythema perstans faciei).

Scarred final condition of atrophying keratosis pilaris in the facial area. Sporadic and familial occurrence possible. Gene locus not (yet) known. The clinical picture has been described in connection with a variant of MALTA syndrome (mutation in the MYH9 gene).

Also occurs in Bazex-Dupré-Christol syndrome (back of the hand and foot).

Partial symptom of Loeys-Dietz syndrome (van Dijk FS et al. 2015).

Conspicuous in adolescence or early adulthood; no known gender preference.

Also occurs in Bazex-Dupré-Christol syndrome (back of the hand and foot). Partial symptom of Loeys-Dietz syndrome(van Dijk FS et al. 2015).

Cheeks bds. mostly symmetrical distribution.

1. Scars after small lupus erythematosus chronicus dicoides
2. acne necrotica
3. Varicella scars
4. Comedon nevus

1. Chisholm C et al. (2011) Unusual and recently described cutaneous atrophic disorders. Int J Dermatol 50:1506-151
2. Das A, Podder I (2014) Atrophoderma vermiculatum. Indian Pediatr51:679
3. Darier J (1920) Atrophodermie vermiculée des joues avec kératoses folliculaires. Bull Soc Franc Derm Syph 27: 345
4. Jansen T et al. (2003) Atrophodermia vermiculata: case report and review of the literature. J Eur Acad Dermatol Venereol 17:70-72
5. Schaller J et al. (2010) Sweat duct proliferation associated with aggregates of elastic tissue and atrophodermia vermiculata: a simulator of microcystic adnexal carcinoma. Report of two cases. J Cutan Pathol 37:1002-1009
6. van Dijk FS et al. (2015) Atrophoderma Vermiculatum: A Cutaneous Feature of Loeys-Dietz Syndrome. JAMA Dermatol 151:675-677.