Acitretin Use in Epidermal Nevus Syndrome

Corresponding author: Wei-Ming Wu, Department of Dermatology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Ta-Pei Road 123, Niao-Sung Hsiang, 83301 Kaohsiung, Taiwan TEL: 886-7-7317123 ext. 2299 FAX: 886-7-7337612 E-mail: [email protected] Funding source: none Confl ict of interest: none declared CASE REPORT A 24-year-old girl presented with linear verrucous skin lesions since birth. Patient also had a history of neonatal seizure and cataract operation at the age of 3 month old. Patient was the product of a full-term uncomplicated pregnancy and delivery; her birth history was uneventful with no traumatic injury or hypoxia. The skin lesion consisted of intensely pruritic erythematous verrucous plaques, arranged in a linear pattern following the lines of Blaschko on the left lower extremities and did not cross the midline (Fig. 1). Throughout the patient’s childhood, the lesions persisted and extended according to the patient’s body size. A skin biopsy from one of the verrucous lesions confirmed the epidermal nevus. No changes of epidermolytic hyperkeratosis were noted. In addition to the skin lesion, patient also had frequent episodes of seizure attacks during childhood. Tracing through the patient’s childhood, she had mental retardation with autism and developmental delay. No other obvious deformities could be found in the orthopaedic, dental, ear, nose and throat or ophthalmologic examination. A chest Xray and brain ultrasound examination did not reveal any specific abnormalities. Patient’s family history was unremarkable. According to the parents, compliance of topical therapy was poor with 5-fl uorouracil (5-FU) and locasalen (fl umethasone-salicylic acid preparations) due to skin irritation. For the past 12 years, patient only used readily available skin care products. Over the years, no change in her skin condition was observed apart from increasing pruritus and hyperkeratotic lesions over the left lower extremity. As various topical treatments have failed to manage clinical symptoms, the patient was provided with acitretin (Neotigason, Roche) with an initial daily dose of 25 mg and patient tolerated well to the treatment. After 5 weeks of treatment, there was a significant flattening of hyperkeratotic plaques without further progression of skin lesions (Fig. 2). Since then, patient’s skin lesion was controlled with acitretin and whenever the acitretin was ceased, patient’s symptoms flared within weeks. Both the patient and mother were pleased with the symptomatic and cosmetic improvement. Currently, patient tolerates well from the oral acitretin with decreased hyperkeratosis and erythema along with decreased pruritic severity.