Diffuse cutaneous mastocytosis responding to cyproheptadine

Abstract

A 3-month-old male infant was referred to our department with a generalized brown, thickened leathery skin and blisters which had been present since birth; the blisters developed recurrently at sites of minor trauma, healing without scar formation, although Darier's sign was positive. Microscopically, biopsy specimens showed a dense dermal band-like infiltrate with mast cells, whereas a biopsy from a vesicle showed subepidermal bulla formation. Physical examination did not reveal any systemic involvement with mastocytosis and the patient was treated with cyproheptadine, which has considerable anti-serotonin activity and, interestingly, reduced the degree of blistering; this treatment might therefore be tried in other cases.

Regulation of Mast Cell Responses in Health and Disease

“…33 It appears to be a minor component in human mast cells. 34 Whether the amounts present are sufficient to impact mast cell function in humans is unclear, but there are reports of effective therapy with the anti-serotonerigic agent, cyproheptadine, in patients with some forms of urticaria, 35 , bullous lesions 36 and irritable bowel syndrome, the latter being attributed to immunologically-induced release of serotonin from mast cells. 37 …”

Human mast cells are capable of serotonin synthesis and release

“…9 Furthermore, the increased levels of serotonin found in some patients with mastocytosis could be associated with the increased mast cell burden and could help explain a report of a response to cyproheptadine in a pediatric patient with bullous lesions. 10 Eventual full-text publication of research abstracts presented at an allergy/ immunology meeting To the Editor: An important measure of the quality of a scientific meeting of any medical professional organization is the determination of the publication fate of the research abstracts presented at the meeting. 1,2 The standard method of assessing this objectively is to investigate the abstract/ publication ratio; that is, to determine the percentage of the original research abstracts presented that are subsequently published as full-text articles in peer-reviewed journals.…”

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

“…Blistering, bullae, prolonged skin bleeding and even lifethreatening episodes related to MC-mediator release are frequent complications of diffuse cutaneous mastocytosis in children [11][12][13][14][15][16][17][18][19][20] but exceptional in urticaria pigmentosa.…”