CUTANEOUS MASTOCYTOSIS IN ADULTS - EVALUATION OF 14 PATIENTS WITH RESPECT TO SYSTEMIC-DISEASE MANIFESTATIONS

Background and Objective: Systemic mastocytosis is a rather rare disor der involving the skin and several other organs. The aim of this study was to analyse the extent of extracutaneous manifestations in 14 adul t patients who presented with prominent cutaneous involvement within t he last 5 years. Results: The cutaneous lesions were clinically diagno sed as telangiectasia macularis eruptiva perstans in 2 patients, urtic aria pigmentosa of varying extent in II and diffuse erythrodermic mast ocytosis in 1 patient. All patients had extracutaneous manifestations with involvement of one additional organ system in 6/14 cases, two in 5/14 and three in 3/14. Ten out of 14 patients suffered from generaliz ed pruritus, and 11/14 reported mild wheal formation, while 3/14 with multi-organ involvement mentioned recurrent flushing episodes. The gas tro-intestinal tract was involved in 8/14 cases with an increase in ga stric and colon mucosal mast cells in 5/8 cases and gastroduodenitis i n 2. Bone marrow involvement was seen in 7/13 patients, hepatosplenome galy in 2, anaemia in 2 and thrombocytopenia in 3. The disease had a d uration of 0.5-32 years, clinical symptoms remaining basically unchang ed. Malignant transformation was not seen; only I patient developed my elodysplastic syndrome within 2 years after the first cutaneous lesion s. Conclusions: Our study shows that extracutaneous involvement should be carefully considered in adult patients with cutaneous mastocytosis . Systemic multi-organ mast cell disease in adults is a long-lasting d isorder with recurrent episodes of varying clinical symptomatology. Ho wever, the disease shows rather slow progression, and malignant transf ormation is rare. Satisfactory management is achieved by symptomatic o ral drug intake.