{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:33:15Z","timestamp":1787841195654,"version":"build-2784847793"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Mastocytosis is characterized by clonal proliferation of mast cells in various organs and can have isolated cutaneous or systemic involvement. Childhood-onset mastocytosis (COM) is usually cutaneous and regresses spontaneously, while adult-onset mastocytosis (AOM) is often persistent with systemic involvement. There is limited data on COM from India.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>To elucidate the clinicopathological profile of COM.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>\n                      We conducted a retrospective chart review of all the patients with histologically proven COM (\u226416 years), presenting over 11 years (January 2009 to December 2019) to the Dermatology Department. We compiled the demographic data, clinical characteristics (morphology, extent, distribution), laboratory investigations, histopathology findings, imaging (ultrasound abdomen),\n                      <jats:italic toggle=\"yes\">c-KIT<\/jats:italic>\n                      mutation results, where available, and other associated abnormalities, and grouped them according to the WHO classification for mastocytosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Among the 66 patients with COM (M: F\u20131.6:1), 89.4% had onset before 2 years of age. The subtypes were: maculopapular cutaneous mastocytosis (MPCM: 44, 66.7%); mastocytoma of the skin (MOS: 19, 28.8%); diffuse cutaneous mastocytosis (DCM: 2, 3%) and indolent systemic mastocytosis (ISM: 1, 1.5%). Blistering was observed in 29 (43.9%) and Darier sign was elicited in 47 (71.2%) patients. Serum tryptase was elevated in 9\/21 (42.9%) patients, but none had systemic mastocytosis. Three patients had\n                      <jats:italic toggle=\"yes\">c-KIT<\/jats:italic>\n                      mutations (two in exon 8 and one in exon 17). Most patients were managed symptomatically and the patient with ISM improved with imatinib.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>MPCM is the most common variant of COM and most patients had a disease onset before 2 years. Overall, COM had a good prognosis with rare systemic involvement, mitigating the need for extensive evaluation routinely in children.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/idoj.idoj_924_20","type":"journal-article","created":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T09:22:05Z","timestamp":1637054525000},"page":"706-713","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":7,"title":["Clinicopathological Profile of Childhood Onset Cutaneous Mastocytosis from a Tertiary Care Center in South India"],"prefix":"10.4103","volume":"12","author":[{"given":"Dharshini","family":"Sathishkumar","sequence":"first","affiliation":[{"name":"Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abyramy","family":"Balasundaram","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surya Mary","family":"Mathew","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia","family":"Mathew","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meera","family":"Thomas","sequence":"additional","affiliation":[{"name":"Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Poonkuzhali","family":"Balasubramanian","sequence":"additional","affiliation":[{"name":"Department of Haematology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renu","family":"George","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,9]]},"reference":[{"key":"R1-20260330","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1111\/bjd.13567","article-title":"Pediatric mastocytosis: A systematic review of 1747 cases","volume":"172","author":"M\u00e9ni","year":"2015","journal-title":"Br J Dermatol"},{"key":"R2-20260330","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.jaci.2015.08.034","article-title":"Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma and Immunology; and the European Academy of Allergology and Clinical 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