Published Online: July 4, 2015
Mastocytosis is characterized by pathologic accumulation of mast cells in tissues. The organ most frequently affected is the skin, followed by bone marrow and gastrointestinal tract. Mastocytosis can manifest itself in adulthood or childhood. Pediatric patients usually show heterogeneous skin lesions with spontaneous improvement after several years. Few patients, however, have persistent disease, resembling adulthood-onset mastocytosis.
In a study recently published in The Journal of Allergy and Clinical Immunology (JACI), Wiechers and colleagues analyzed skin lesions in 144 patients with childhood-onset mastocytosis, hypothesizing that the type of cutaneous lesions correlates with other disease parameters. They used a systematic dermatologic approach to define the morphology and distribution of skin lesions.
The authors found that children with large maculopapular cutaneous lesions, compared to small lesions, had significantly lower tryptase levels, shorter disease duration, and earlier disease onset. Patients with large lesions also exhibited more frequently spontaneous regression of their skin lesions.
The authors’ findings suggest that large maculopapular cutaneous lesions are associated with a more favorable outcome of the disease. The study implies using the size of skin lesions as prognostic factor in pediatric mastocytosis.
The Journal of Allergy and Clinical Immunology (JACI) is an official scientific journal of the AAAAI, and is the most-cited journal in the field of allergy and clinical immunology.