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Chronic cutaneous mastocytosis is a genodermatosis of unknown etiology and that is within the group of rare or infrequent diseases. They are characterized by the growth and accumulation of mast cells in the skin and other organs of unknown cause and manifest as urticaria pigmentosa. The case of a male patient is presented, with erythematopapular lesions in the region of the hands that spread to the entire body and scalp that, when they disappeared, left hyperchromic spots with Darier's sign: positive. The diagnosis of urticaria pigmentosa was confirmed by skin biopsy. It is a rare disease with a low incidence, the cutaneous form has a favorable prognosis, so it is of vital importance to know and diagnose this entity early with a multidisciplinary management to prevent the systemic manifestations that appear. Taking a symptomatic treatment will allow a better quality of life in infants. The case is presented because mastocytosis is considered a rare disease, the prevalence and incidence of these cases is very low.

The mastocytoma is a genodermatosis of unknown etiology, so it is included in the group of rare or uncommon diseases; it usually appears in childhood or at birth. It is characterized by local or systemic accumulation of primed cells, which causes the appearance of persistent, pigmented and very itchy skin lesions with exclusive skin involvement, usually presents as a single lesion in the neck, trunk, arms and legs. Typical features are that of a reddish-brown or yellowish brown nodule, of gummy consistency, the surface looks like orange peel, and Darier's sign. The case of a 4-month-old infant with a diagnosis of solitary mastocytoma with Darier's sign is presented. This report is made due to the low frequency of this disease at the Paquito González Cueto de Cienfuegos University Pediatric Hospital. Currently the patient is monitored by dermatology specialists, with antihistamine treatment and mast cell stabilizer.

The mastocytoma is a genodermatosis of unknown etiology, so it is included in the group of rare or uncommon diseases; it usually appears in childhood or at birth. It is characterized by local or systemic accumulation of primed cells, which causes the appearance of persistent, pigmented and very itchy skin lesions with exclusive skin involvement, usually presents as a single lesion in the neck, trunk, arms and legs. Typical features are that of a reddish-brown or yellowish brown nodule, of gummy consistency, the surface looks like orange peel, and Darier's sign. The case of a 4-month-old infant with a diagnosis of solitary mastocytoma with Darier's sign is presented. This report is made due to the low frequency of this disease at the Paquito González Cueto de Cienfuegos University Pediatric Hospital. Currently the patient is monitored by dermatology specialists, with antihistamine treatment and mast cell stabilizer.