Case Report DOI: 10.18231/2581-4729.2018.0035 IP Indian Journal of Clinical and Experimental Dermatology, April-June, 2018;4(2):156-157 156 Acantholytic dyskeratotic epidermal nevus which mimic as a darier’s disease Rita Vora 1,* , Rochit Singhal 2 , Trusha Patel 3 , Khushboo Modasia 4 1 Professor, 2 I st Year Resident, 3 2 nd Year Resident, 4 3 rd Year Resident, Dept. of Dermatology and VD, Pramukhswami Medical College, Gujarat, India *Corresponding Author: Email: ritavv@charutarhealth.org Abstract Darier’s disease is characterized by multiple hyperkeratotic lesions and crusted papules or plaques involving seborrheic areas along with palmoplantar pits, and nail changes. Epidermal naevus may show acantholytic histology similar to darier's disease. Presenting a case of acantholytic dyskeratotic epidermal nevus mimicking darier’s disease. Negative family history, no significant signs of darier’s disease with limited distribution of lesions favour a nevoid origin. Keywords: Darier’s disease, Epidermal naevus, Retinoids. Introduction Epidermal nevi are localized malformed epidermis consisting of verrucoid scaly papules or plaques presented along the lines of Blaschko's. Rarely, epidermal naevi show acantholytic histology resembling darier's disease, a dominantly inherited skin condition characterized by widespread warty papules. The prevalence of the disease is estimated to be 1 in 50000 people. Phenotypic expression of darier’s disease (DD) is variable. In 1906 the first localised pattern of DD was reported. 1 Other variants to be recognised since then are unilateral, linear, segmental or zosteriform DD. 2 Here we present a case of acantholytic dyskeratotic epidermal nevus mimicking darier’s disease. Case Report A 31-year-old male presented with a 7-year history of skin lesions over body which were progressive in nature. Patient also had associated complaint of itching and burning over the affected sites. Patient did not give history of worsening of lesions in summer or after sun exposure. No other family member had similar illness. Patient was being treated for the same by a private doctor in the form of oral methotraxate and laser therapy before he presented to our department. Cutaneous examination showed multiple hyperpigmented, greasy, warty papules and plaques with adherent crusts and scales over both legs and feet with multiple discrete erythematous keratotic papules over palms, bilateral dorsum of hands, abdomen, back and soles (Fig. 1 a,b,c). There were no other mucosal lesions, nail changes or systemic complaints. Biopsy from the scaly papules showed hyperkeratosis, parakeratosis, moderate acanthosis, orthokeratosis with papillomatosis of epidermis and dyskeratosis (in the form of corps ronds and grains) in the epidermis (Fig. 3). Routine laboratory tests were within normal limits. We gave patient oral steroids and isotretinoin with vitamin A supplements and topical retinoids which showed marked improvement within a year (Fig. 2 a,b,c). Fig.1: pre-treatment photograph (a) Multiple hyperpigmented, greasy, warty papules and plaques with adherent crusts and scales over both legs (b) abdomen (c) back