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Mastocytoses are a heterogeneous group of disorders of unknown etiology characterised by the accumulation of mast cells, particularly in the skin. Mastocytosis may be limited to the skin or involve internal organs, especially those playing the lymphoreticular function. We present a case of 9-year-old girl, with a history of maculopapules at the age of two, followed by yellow-brownish, nodular skin lesions. The skin lesions, located on the trunk, neck and face were accompanied by pruritus. The histopathological examination of the node confirmed the diagnosis of mastocytoma. A bone scintigram showed an increased uptake of radiotechnetium around the right coxal region. Application of antihistaminic drugs and phototherapy (UVA) has led to partial remission of skin manifestations of the disease. A long-term follow-up of the child is necessary.

Background: Urticaria pigmentosa (UP) is characterized by dense aggregates of mast cells in the dermis. There is consistent evidence from the literature that mast cells may play a pathogenetic role in the development of neurofibromas and other tumors., Objective: To study the concomitant appearance of UP with neurofibromas and neurofibroma-like neoplasms., Methods: We analyzed 31,752 records of patients examined at the Department of Dermatology in the year 2000, looking for UP and associated neurofibromas and neurofibroma-like neoplasms in persons younger than 18 years., Results: We identified a total of 27 patients suffering from UP, with 16 persons younger than 18 years. One 12-year-old Caucasian boy demonstrated multiple cutaneous mastocytomas consistent with the diagnosis of UP. On his trunk, four café-au-lait spots were found. A cutaneous neurofibroma was confirmed by skin biopsy. Magnetic resonance imaging detected multiple neoplasms located at the nerve roots of the spine, resembling plexiform neurofibromas., Conclusions: There may be a concomitant appearance of UP and neoplasms, with mast cells possibly playing a causative role. The existence of neoplasms, including neurofibromas and neurofibroma-like lesions should be considered when examining UP cases., (Copyright 2003 S. Karger AG, Basel)

A patient with long-standing nodular mastocytosis developed a slate-blue lesion on the scalp which produced symptoms of histamine release on mechanical and thermal stimulation. Light and electron microscopic examination of the lesion showed zones of mast cells and zones of melanized cells. A transition zone showing cells with dual (melanosomal and mast) granulation, as well as granules representing intergrades between classical mast granules and melanosomes, was present. Features of this lesion add to the accumulated evidence that there is a histogenetic relationship between mast cells and melanocytes.

A case of systemic mastocytosis is reported with an observation period of 20 years. During these two decades multiple manifestations of the disease appeared including urticaria pigmentosis, episodic histamine release, gastro-intestinal involvement and hepatosplenomegaly. The most extraordinary, and possibly unique phenomenon, has been the development of a massive proliferation of large mastocytomas mainly, but not exclusively, limited to the lower extremities. For different reasons (mechanical disability, bleeding, cosmesis), these tumors have required repeated admissions for surgical removal. The most successful technique has involved use of the ultrasonic scalpel.