Keratosis peripapillary Otto–Moncorps

Abstract

OttoMoncorps keratosis is a rare dermatosis, which is excessive keratinization of the nipple and areola of the mammary gland with an unknown etiology and pathogenesis. According to the classification proposed by LevyFrankel in 1938, the disease can be represented by three types: type 1 ― a variant of the epidermal nevus, type 2 ― associated with other diseases and type 3 ― idiopathic. The diagnosis is based on the data of the clinical picture, represented by diffuse keratinization of the nipple and areola of the mammary gland, dark brown in color, resembling basalt cubes. To date, there are no effective pathogenetic methods of treatment, methods of regular topical therapy are recommended (topical steroids and retinoids, keratolytics in high concentrations, emollients and moisturizers), as well as surgical methods of treatment (excision of hyperkeratotic overlays with a carbon dioxide laser, radiosurgical methods). The article presents a unique case of hyperkeratosis of the periarticular circle and breast nipple type 3 according to LevyFrankel in a 39-year-old patient. The diagnosis was made on the basis of anamnesis data (onset of the disease at puberty), a typical clinical picture (bilateral lesion; dermatosis in the form of hyperkeratotic layers resembling basalt cubes), as well as the characteristic course of the disease (relapse after discontinuation of therapy with local remedies). The interest of the presented clinical case lies not only in the rarity of this dermatosis and the duration of the disease history (for 24 years), but also in the presence of an extra-mammary lesion and a possible association with infertility of the patient.