Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis

Putra, Prasta Bayu and Radiono, Sunardi and Danarti, Retno (2021) Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis. Dermatology Online Journal, 27 (2). ISSN 10872108

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Abstract

Epidermolytic ichthyosis (EI, OMIM 113800) is a rare autosomal dominant keratinization disorder that is caused by keratin 1 or keratin 10 gene mutation. It can be classified clinically based on the presence of palmoplantar hyperkeratosis involvement and extent of skin involvement. The diagnosis is made by clinical and histopathological examinations that can be confirmed by genetic testing. We present a 2-year-old girl who presented with erythematous and thick scaling skin. Her condition began at birth as multiple flaccid blisters that would easily break into erosions. There was no history of similar condition nor consanguinity within her family. Skin examination revealed diffuse erythematous skin covered with thick scales and erosion, predominantly on her face, extremities, palms, and soles. The skin histopathology examination showed diffuse parakeratosis with vacuolar and granular degeneration within granular and spinous layers along the epidermis. She was diagnosed with generalized EI with palmoplantar hyperkeratosis based on the clinical and histopathological examinations. Clinical improvement was observed after a one-month treatment with mupirocin cream, sodium bicarbonate bath, and moisturizer after bathing. © 2021 Dermatology Online Journal. All rights reserved.

Item Type: Article
Additional Information: Cited by: 0
Uncontrolled Keywords: Child, Preschool; Female; Humans; Hyperkeratosis, Epidermolytic; Keratoderma, Palmoplantar; bicarbonate; pseudomonic acid; pseudomonic acid; Article; Auspitz sign test; blister; body height; body weight; case report; child; child health care; clinical article; clinical feature; clinical outcome; disease course; drug response; epidermolytic ichthyosis; erythema; female; histopathology; human; ichthyosis; lymphocytic infiltration; medical history; nail dystrophy; palmoplantar keratoderma; physical examination; preschool child; skin disease assessment; skin examination; treatment duration; vital sign; blister; cream; disease duration; drug effect; erythema; generalized epidermolytic ichthyosis; human tissue; parakeratosis; skin biopsy; complication; epidermolytic hyperkeratosis; palmoplantar keratoderma
Subjects: R Medicine > RL Dermatology
Divisions: Faculty of Medicine, Public Health and Nursing > Public Health and Nutrition
Depositing User: Sri JUNANDI
Date Deposited: 25 Sep 2024 00:44
Last Modified: 25 Sep 2024 00:44
URI: https://ir.lib.ugm.ac.id/id/eprint/4705

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