Medizinische Universität Graz - Research portal
Selected Publication:
Savic, S.
DERMATOLOGICAL SYMPTOMS IN SYSTEMIC MASTOCYTOSIS
Disease spectrum in patients with elevated tryptase
[ Diplomarbeit ] Medical University of Graz; 2012. pp. 55
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- Authors Med Uni Graz:
- Advisor:
- Aberer Elisabeth
- Aberer Werner
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- Abstract:
- Background Mastocytosis is a rare disease that has systemic and skin manifestations. It is often hard to diagnose, especially in patients with a lack of skin symptoms. One of the characteristics of mastocytosis is an elevated tryptase. Aims 1. To investigate patients with elevated tryptase for their underlying diseases. 2. To see whether increased tryptase can be used as a diagnostic marker for underlying mastocytosis or whether it is only a sign for a mast cell activation. 3. To see whether or not there is a need for full medical examination for an underlying mastocytosis in patients with a mast cell activation syndrome and elevated tryptase. 4. To define how specific is an elevated tryptase for mastocytosis. Methods In this retrospective study conducted at the Department of Dermatology, Division of Environmental Dermatology and Venerology, data of 96 patients with elevated tryptase (>15µg/l) were collected and systematically evaluated. The patients were classified in diagnostic groups, and statistical analysis was performed. Results Only 15.6% of our patients had mastocytosis. The rest of the patients was divided as follows: 36.4% had anaphylaxis, 26% urticaria and angioedema, 4.2% increased local reactions after insect stings, 3.1% drug reactions, and 14.6% different diagnoses. Seven patients had systemic mastocytosis (SM) with skin involvement, five SM without skin involvement and three had urticaria pigmentosa (UP). Patients with SM with skin involvement had highest mean tryptase levels (65.9µg/l). followed by SM - patients without skin involvement (53.5µg/l), UP patients (30.9µg/l), patients who had drug reactions (27.4µg/l), urticaria and angioedema patients (25.1µg/l), patients with anaphylaxis (21.7µg/l), patients with increased local reactions (21.3µg/l) and finally patients who had different diagnoses (19.9µg/l). Conclusion Elevated tryptase is not only a sign for mastocytosis but also seen in urticaria and angioedema, drug reaction and anaphylactic reactions during an acute event. Repeatedly elevated tryptase levels should be determined as a specific marker for mastocytosis. In these patients appropriate investigations should be done. Patients with high tryptase and severe anaphylaxis should be inspected for skin lesions of mastocytosis and their baseline serum tryptase should be determined. In the case of elevated baseline tryptase, these patients should get a total diagnostic body work up for SM.