A case of type 1 pachyonychia congenita with response to acitretin

Abstract

An 8-year-old male born of nonconsanguineous marriage presented with multiple raised lesions all over the body, over palms and soles, and thickening of all nails of fingers and toes for 5 years. On examination, there were hyperkeratotic plaques over palms and soles with subungual hyperkeratosis of all finger and toe nails. Oral cavity revealed white hyperkeratotic plaque over buccal mucosa. There were hyperkeratotic papules distributed over dorsum of hands, feet, elbows, knees, trunk, and thighs. Biopsy taken from hyperkeratotic lesion over palms revealed findings suggestive of palmoplantar keratoderma. Considering the clinical features and histopathological examination, a diagnosis of Type 1 pachyonychia congenita was made. After hematological investigations, the patient was started on oral acitretin at a dose of 25 mg thrice a week. There was a marked improvement in the form of reduced hyperkeratosis over palms and soles and subsidence of hyperkeratotic papules and oral lesions, 2 months after treatment with acitretin.