RESUMO
Dowling-Degos disease (DDD) is an uncommon autosomal dominant genodermatosis that resides in the spectrum of diseases presenting with reticulate pigmentation. This disease has varied phenotypic expressions, the classical presentation being reticular pigmentation of flexures involving the axilla, submammary folds, inguinal folds, and neck. Follicular DDD is a variant of DDD with a unique presentation of folliculocentric papules, macules, pits, and comedones associated with the characteristic histological findings of follicle-centered, pigmented, branching, antler horn-like rete ridges sparing the interfollicular epidermis. Due to the rarity and paucity of data about this entity, we describe this case of a 28-year-old female who presented with perioral pitted scars and multiple hyperpigmented folliculocentric comedo-like papules over the face, neck, cubital fossa, and upper trunk, unaccompanied by the typical non-follicular, reticulate flexural hyperpigmentation, which clinically posed a diagnostic challenge. The diagnosis was confirmed by histopathology. We intend to increase clinicians' cognizance with respect to the unique clinical and histopathologic presentation of follicular DDD. More genetic studies could bring more understanding of this complex spectrum.
RESUMO
Urticaria pigmentosa is the most common form of mastocytosis that often develops in infancy or early childhood. We report two male children- first, a 7-month-old child with a history of asymptomatic multiple dark colored skin lesions macules with wheals on gentle rubbing (Darier's sign) and second, a 2-year-old child with similar clinical presentation without Darier's sign. Dermoscopy showed dark brown lines in a reticulate pattern which is an exaggeration of the pigment network seen in the normal skin. The reticulate pigment network was darker and thicker in the child with positive Darier's sign. This is the first case report of dermoscopy of urticarial pigmentosa with and without Darier's sign reported in skin of colour.