Solitary perioral porokeratosis

A 50-year-old brown-skinned female presented to the dermatology outpatient department with an asymptomatic skin colored lesion on the upper and lower lip of 2.5 years’ duration. The lesion started insidiously as a skin-colored papule near the angle of mouth. The papule gradually increased in size extending into the angle of mouth and adjacent labial mucosa and also on the upper and lower lip. There was no history of any discharge from the lesion. On examination, a well-demarcated, irregular plaque with atrophic center and peripheral keratotic margin with a thin furrow [Figure 1] involving the left side of the upper lip, lower lip and angle of mouth, and adjacent labial mucosa was seen. Examination of the oral mucosa revealed angular cheilitis and poor oral hygiene. Histopathology of skin biopsy specimen revealed cornoid lamella with underlying agranulosis [Figure 2]. Based on clinical and histopathological features, a diagnosis of solitary oral plaque type of porokeratosis of Mibelli was made.

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Figure 1:

Well-demarcated, irregular plaque with atrophic center and peripheral keratotic margin with a thin furrow.

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Figure 2:

Column of parakeratotic cells extending through stratum corneum with underlying agranulosis (H and E, ×40).

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Porokeratosis is a rare keratinization disorder characterized by annular plaque with atrophic center and thready keratotic border described by Mibelli and Resphigi in 1893. Histologically, it is characterized by column of parakeratosis known as cornoid lamella. Treatment options include both topical and systemic therapies. Topicals include topical 5-Fluorouracil, topical retinoids, calcineurin inhibitors, and topical vitamin D3 analogs and systemic options include oral retinoids. Surgical options include excision, cryotherapy, electrodessication, and dermabrasion.^[1]