Premium
Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study
Author(s) -
Fradet M.,
Negretto M.,
Tournier E.,
Laurent C.,
Apoil P.A.,
Evrard S.,
Degboe Y.,
Del Mas V.,
Lamant L.,
Dubreuil P.,
Laroche M.,
Mailhol C.,
Hermine O.,
Paul C.,
Bulai Livideanu C.
Publication year - 2019
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1111/jdv.15638
Subject(s) - cutaneous mastocytosis , systemic mastocytosis , medicine , tryptase , urticaria pigmentosa , mast cell , bone marrow , population , pathology , dermis , dermatology , immunology , environmental health
Background Mastocytosis is characterized by the accumulation/proliferation of abnormal mast cells. The frequency of isolated cutaneous involvement in adults with mastocytosis has not been fully determined. The main objective of our study was to assess the frequency of isolated cutaneous mastocytosis (CM) in adults with mastocytosis skin lesions. The second objective was to compare the clinical, histological, biological and imaging features in patients with isolated CM and patients with systemic mastocytosis ( SM ). Methods We included all patients with histology‐proven mastocytosis skin lesions between January 2009 and December 2017. The mastocytosis diagnosis was made according to the international diagnostic criteria. All data were collected from a dedicated specific case report. Results Among 160 patients with mastocytosis skin lesions, 25 patients had isolated CM (15.6%), 105 had SM and 30 (18.7%) patients had undetermined mastocytosis. Skin KIT mutation ( OR : 51.9, 95% CI: 3.9–678, P = 0.001) and high bone marrow tryptase ( OR : 97.4, 95% CI: 10.3–915, P = 0.001) were strong predictors of SM . The prevalence of osteoporosis was higher in the SM population than in the isolated CM population. Moreover, a decrease in bone mineral density over a short period of follow‐up (1–2 years) was associated with SM . There were no differences between the two groups regarding the frequency of mast cell activation symptoms, the presentation of skin lesions, the number of mast cells in the dermis and the level of serum tryptase. We propose considering the KIT mutation status and bone marrow tryptase levels to aid the diagnosis of isolated CM in adult mastocytosis patients. Conclusion Only a small minority of adults with mastocytosis skin lesions has isolated cutaneous involvement. In 18.7% of mastocytosis cases, even complete workup does not allow for a precise classification of patients.