Reticulated pigmented anomaly of the flexures - M. Dowling-Degos disease in association with acne inversa

Reticulated pigmented anomaly of the flexures (Dowling-Degos disease) is a rare autosomal-dominant genodermatosis with variable penetrance that is cha racterized by reticulated hyperpigmentation of the flexures. Acne inversa h as been previously described in the literature under several synonyms, such as acne triad, acne tetrad and hidradenitis suppurativa. Acne inversa is a n inflammation affecting the pilosebaceous units of the flexures,We report a case of Dowling-Degos disease in association with acne inversa. The assoc iation of these diseases has been described several times and it appears no t to be coincidental. Pathogenetic mechanisms leading to this coincidence a re discussed.