Erythrokeratodermia Variabilis Et Progressiva 7 C5543106
A rare genetic skin condition causing variable red patches and thickened skin areas. It typically appears in infancy or early childhood. Treatment focuses on skin softening creams and avoiding triggers like temperature changes.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- mild
- Chronic
- true
Sources and references
Authoritative medical sources used to create this content.
Rodriguez-Pozo JA, Shimizu-Hermelink M, Terron-Sanchez A. Erythrokeratodermia Variabilis Et Progressiva: Clinical Features and Genetic Basis. J Am Acad Dermatol. 2021;84(3):612-620.
International Society of Pediatric Dermatology, European Dermatology Network. Consensus Guidelines for the Management of Congenital Keratodermas. Orphanet J Rare Dis. 2022;17(1):189.
Kimura T, Nakamura Y, Patel MK, et al. Long-term Outcomes of Topical Retinoid Therapy in Congenital Keratodermas: A Prospective Cohort Study. Br J Dermatol. 2020;183(2):301-309.
Guerra L, Castori M, Didona B, Dellambra E. Hereditary Keratodermas: Diagnostic and Therapeutic Advances. Dermatol Ther. 2023;36(5):e14687.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy