Journal
INDIAN JOURNAL OF DERMATOLOGY
Volume 68, Issue 1, Pages -Publisher
WOLTERS KLUWER MEDKNOW PUBLICATIONS
DOI: 10.4103/ijd.ijd_264_22
Keywords
Childhood; cutaneous mastocytosis; hematopoietic neoplasm
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Cutaneous mastocytosis is a neoplasm characterized by the proliferation and accumulation of mast cells in the skin. It can affect other organ systems as well. Symptoms range from various skin manifestations to systemic reactions. Treatment includes avoiding triggers, medication, and in some cases, more aggressive therapies like stem cell transplantation. This article aims to review the English literature on childhood cutaneous mastocytosis.
Cutaneous mastocytosis is a neoplasm characterized by the proliferation and accumulation of mast cells in the skin. There can be involvement of other organ systems as well. Cutaneous manifestations can vary from mastocytoma to maculopapular lesions to diffuse cutaneous form. There can be symptoms associated with mast cell mediators release like itching, flushing, hypotension, diarrhoea, abdominal pain, and anaphylaxis. Hence, the mainstay of treatment is avoidance of triggers causing these mediators to release, anti-histamines, topical/intra-lesional/systemic steroids, mast cell-targeted therapy, epinephrine, and omalizumab depending upon the severity of symptoms/signs. Childhood cases usually have a good prognosis except in a few cases, especially those with systemic involvement. Such situations might warrant cytoreductive therapy, polychemotherapy, or autologous stem cell transplantation. Here, we intend to review the English literature on childhood cutaneous mastocytosis.
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