Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination: A Case Report. Issue 6 (November 2016)

• Record Type: Journal Article
• Authors: Hudson, Alexandra; Finlayson, Laura
• Is Part Of: Journal of cutaneous medicine and surgery. Volume 20:Issue 6(2016:Nov./Dec.)
• Page Start: 596
• Page End: 599
• Publication Date: 2016-11
• Subjects: cutaneous mastocytosis -- pediatric; Dermatology -- Periodicals; Skin -- Diseases -- Treatment -- Periodicals; Skin -- Surgery -- Periodicals; Dermatology -- Canada -- Periodicals; Skin -- Diseases -- Treatment -- Canada -- Periodicals; Skin -- Surgery -- Canada -- Periodicals; 616.5005
• DOI: 10.1177/1203475416661312
• Languages: English
• ISSNs: 1203-4754

Abstract

Background: Diffuse cutaneous bullous mastocytosis is the most rare subtype of cutaneous mastocytosis, characterized by generalized skin infiltration with mast cells and blistering. Objective: To increase the awareness of the natural history and potential adverse complications of this rare cutaneous condition. Method and Results: We report a case of a male diagnosed on day 7 of life with follow-up of his progression over 6 years. When he was 2.5 months old, he was admitted to hospital postvaccinations with a flare of his blistering that was complicated by disseminated intravascular coagulation and polyuric acute tubular necrosis. Blistering ceased at 3 years, but at 6 years, extensive urtication continued in response to known triggers and a suboptimal dose of mast cell membrane stabilizers and histamine-1 and -2 receptor antagonists. Conclusion: This case discusses the progression of this rare condition over 6 years and highlights the importance of reaching optimal pharmacologic blockage of histamine-1 and -2 receptors and stabilization of mast cell membranes in patients persistently experiencing ongoing pruritus, urtication, and flushing symptoms.