| Summary: | Dowling–Degos disease (DDD) is a rare genodermatosis with autosomal dominant inheritance. It is characterized by reticulate pigmentation of flexures, comedo-like follicular papules, and perioral pitted scars. Here, we report a 3-year-old female child presenting with hyperpigmentation of the external genitalia, intergluteal fold, and armpits with follicular keratotic papules over the neck. Histopathological examination showed hyperkeratosis, irregular acanthosis, papillomatosis, and basal layer pigmentation in the epidermis with elongated and branched rete pegs and relatively more melanin concentration in the tips of the rete pegs. The diagnosis of DDD was made. This case is reported due to the unusual early onset and involvement of external genitalia.
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