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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Wiechers, Tim Rabenhorst, Anja Schick, Tina Preussner, Liane M. Förster, Anja Valent, Peter Horny, Hans-Peter Sotlar, Karl Hartmann, Karin |
| Description | Country affiliation: Germany Author Affiliation: Wiechers T ( Department of Dermatology, University of Cologne, Cologne, Germany.); Rabenhorst A ( Department of Dermatology, University of Cologne, Cologne, Germany.); Schick T ( Department of Dermatology, University of Cologne, Cologne, Germany); Preussner LM ( Department of Dermatology, University of Cologne, Cologne, Germany.); Förster A ( Department of Dermatology, University of Cologne, Cologne, Germany.); Valent P ( Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria.); Horny HP ( Institute of Pathology, Ludwig-Maximilians-Universität, Munich, Germany.); Sotlar K ( Institute of Pathology, Ludwig-Maximilians-Universität, Munich, Germany.); Hartmann K ( Department of Dermatology, University of Cologne, Cologne, Germany) |
| Abstract | BACKGROUND: Mastocytosis, characterized by pathologic accumulation of mast cells, can manifest itself in adulthood or childhood. Pediatric patients usually have cutaneous mastocytosis (CM) with mast cell infiltrates limited to the skin and spontaneous improvement of skin lesions after several years. However, there are some patients with persistent disease resembling adulthood-onset mastocytosis. OBJECTIVE: The current classification of CM differentiates between 3 subforms. In clinical practice we noticed that different variants of these subforms might exist, particularly in patients with childhood-onset mastocytosis. Therefore, in the present study, we aimed to investigate whether specific cutaneous lesions in patients with childhood-onset mastocytosis are associated with other disease parameters. METHODS: We analyzed 144 patients with a disease onset of less than age 17 years using a systematic dermatologic approach. RESULTS: One hundred twenty-two patients presented with maculopapular cutaneous mastocytosis (MPCM), 12 patients presented with diffuse CM, and 10 patients presented with solitary mastocytoma of the skin. Patients with MPCM showed particularly heterogeneous cutaneous lesions and were therefore grouped into 3 variants presenting either with small lesions (MPCM-small, skin lesions <1 cm in diameter; n = 19), large lesions (MPCM-large, skin lesions ≥ 1 cm in diameter; n = 89), or atypical lesions (MPCM-other, n = 14). Patients with MPCM-large lesions, compared with those with MPCM-small lesions, were characterized by significantly lower tryptase levels, shorter disease duration, and earlier disease onset. In addition, more patients with MPCM-large lesions exhibited spontaneous regression of cutaneous lesions. CONCLUSION: Our data show that patients with MPCM-large lesions compared with those with MPCM-small lesions have a more favorable disease course and suggest exploring the size of cutaneous lesions as a prognostic parameter in childhood-onset MPCM. |
| File Format | HTM / HTML |
| ISSN | 00916749 |
| e-ISSN | 10976825 |
| Journal | Journal of Allergy and Clinical Immunology |
| Issue Number | 6 |
| Volume Number | 136 |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2015-12-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Discipline Immunology Urticaria Pigmentosa Diagnosis Adolescent Child, Preschool Infant Infant, Newborn Prognosis Proto-oncogene Proteins C-kit Genetics Tryptases Blood Pathology Research Support, Non-u.s. Gov't |
| Content Type | Text |
| Resource Type | Article |
| Subject | Immunology and Allergy Immunology |
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