Epidermolytic Ichthyosis Treated with Low-dose Isotretinoin

Affecting only 1 in 200,000–300,000 persons worldwide, epidermolytic ichthyosis (EI) is a rare autosomal dominant genodermatosis presenting with a characteristic phenotype of generalized corrugated scaling. We report a case of EI highlighting significant improvement in clinical outcomes and quality of life. A 27-year-old Filipino male sought to consult for generalized foul-smelling “wood bark” like scaling since early childhood. Histologic findings confirmed EI. The condition was managed with hydration, lubrication, keratolysis, and low-dose isotretinoin (0.5 mg/kg/day). Clinical outcomes, measured using the visual index for ichthyosis severity scoring and dermatology life quality index, revealed significant improvement. EI is a rare life-long disease of cornification which can severely incapacitate the person affected. Given the limited cases, this case report supports the efficacy of oral isotretinoin in its treatment. The recovery in the cosmetic and functional capacity parallels the upliftment of psychosocial burden. Overall, the management led to an improvement in the quality of life.

Authors

Publication Details

Journal
Journal of the Philippine Dermatological Society
Published
2024-11-01
DOI
https://doi.org/10.4103/jpds.jpds_28_24
Primary Topic
melanin and skin pigmentation
Type
article
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article

Epidermolytic Ichthyosis Treated with Low-dose Isotretinoin

Janelle Go, Nica Minerva Quijalvo Olivar-Floro
Journal of the Philippine Dermatological Society
melanin and skin pigmentation
article

Epidermolytic Ichthyosis Treated with Low-dose Isotretinoin

Janelle Go, Nica Minerva Quijalvo Olivar-Floro
article en

Abstract

Affecting only 1 in 200,000–300,000 persons worldwide, epidermolytic ichthyosis (EI) is a rare autosomal dominant genodermatosis presenting with a characteristic phenotype of generalized corrugated scaling. We report a case of EI highlighting significant improvement in clinical outcomes and quality of life. A 27-year-old Filipino male sought to consult for generalized foul-smelling “wood bark” like scaling since early childhood. Histologic findings confirmed EI. The condition was managed with hydration, lubrication, keratolysis, and low-dose isotretinoin (0.5 mg/kg/day). Clinical outcomes, measured using the visual index for ichthyosis severity scoring and dermatology life quality index, revealed significant improvement. EI is a rare life-long disease of cornification which can severely incapacitate the person affected. Given the limited cases, this case report supports the efficacy of oral isotretinoin in its treatment. The recovery in the cosmetic and functional capacity parallels the upliftment of psychosocial burden. Overall, the management led to an improvement in the quality of life.

Journal of the Philippine Dermatological SocietyVol. 33(2)
Openalex Percentile: Top 74%
melanin and skin pigmentation
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Epidermolytic Ichthyosis Treated with Low-dose Isotretinoin — Janelle Go, Nica Minerva Quijalvo Olivar-Floro · Journal of the Philippine Dermatological Society (2024) | TGRS Research Map | TGRS