Mast cell tumor history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
History and Symptoms
There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include: •cutaneous manifestations •flushing •hypotension and syncope •abdominal pain •nausea, vomiting and diarrhoea •malabsorption •bone pain and pathological fractures •associated haematological disorders
MPCM (urticaria pigmentosa) is the most common clinical variant in which fixed, reddish brown lesions occurring as maculo-papules, plaques, nodules or blisters are found. These lesions urticate in response to physical irritation (Darier’s sign). Urticaria Pigmentosa (UP) lesions tend to be larger, better delineated, and more hyperpigmented in children, as compared to adults, who tend to have numerous small lesions that coalesce to form mottled areas. The trunk and thigh are more commonly involved with sparing of face, palms and soles. DCM is a rare variant of childhood mastocytosis that appears as diffuse infiltrative yellow-orange xanthogranuloma-like subcutaneous nodules, or as a widespread urticarial eruption with bullae and redness. The clinical course of DCM is more severe than that of mastocytoma and MPCM and can even be life-threatening, due to hypovolemic shock, mast cell leukemia, gastrointestinal hemorrhage, and cachexia[1]
References
- ↑ Ferrante, Giuliana; Scavone, Valeria; Muscia, Maria; Adrignola, Emilia; Corsello, Giovanni; Passalacqua, Giovanni; La Grutta, Stefania (2015). "The care pathway for children with urticaria, angioedema, mastocytosis". World Allergy Organization Journal. 8 (1): 5. doi:10.1186/s40413-014-0052-x. ISSN 1939-4551.