Graham Little Piccardi Lasseur Syndrome: A Rare Case Report with Concomitant Hypertrophic Lichen Planus
A 35-year-old female patient presented with the chief complaint of multiple pruritic follicular keratotic and spinous papules initially over wrists and forearms, gradually involving whole body, sparing face, palms and soles since past 10 years, followed by appearance of hyperkeratotic violaceous itchy plaques over malar area of face with madrosis and scarring alopecia of scalp since 4 years. Multiple dilated follicular orifices plugged with keratotic debris were present on scalp with marked perifollicular violaceous hyperpigmention and complete loss of hair []. Axillary and pubic hair were sparse with history of recurrent episodic shedding since last 3 years. Violaceous pigmentation over buccal and gingival mucosae was seen along with melanonychia in fingernails. No history of photosensitivity or similar complaints in family.\nRoutine investigations including viral markers for hepatitis B and C and serum antinuclear antibody levels were normal. Histopathological examination of hyperkeratotic alopecic plaque on scalp showed dilated follicular infundibula, with basket-weave hyperkeratosis in upper portion and compact hyperkeratotic plug in lower portion. Epithelium lining the follicular infundibulum showed marked hypergranulosis along with presence of vacuolar degeneration and apoptotic keratinocytes. Moderately dense perifollicular lymphocytic inflammation, lichenoid interface dermatitis and loss of sebaceous glands with follicular scarring were suggestive of lichen planopilaris with cicatricial alopecia. Biopsy from the follicular papules on body also revealed similar histopathological features consistent with lichen planopilaris []. Patient was put on oral retinoids in form of tablet isotane 20 mg once daily with regular follow up since past 2 months.