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Diffuse cutaneous mastocytosis in Child
Other Resources UpToDate PubMed

Diffuse cutaneous mastocytosis in Child

Contributors: Paul Curtiss MD, Gabriela Cobos MD, Susan Burgin MD
Other Resources UpToDate PubMed


Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis that occurs in the pediatric population and is characterized by an abnormal accumulation of mast cells within the dermis. Unlike other forms of cutaneous mastocytosis, DCM generally involves the entire skin and frequently results in more severe disease phenotypes. DCM typically presents within the first 2-6 months of life, but it may be present at birth.

The clinical presentation may be variable. A distinctive presentation in infants is that of generalized bullae formation with underlying normal or erythrodermic skin. Another presentation in infancy is that of infiltrated papules and plaques with a "grained leather" appearance on which smaller vesicles can occur. Here, blistering is less severe and a less consistent feature. Features common to both these presentations include Darier sign, dermographism, flushing, and pruritus. In older children and teenagers, the appearance is variable and may include diffuse infiltration and/or hyperpigmentation, nodular mastocytomas, and pseudoxanthomatous lesions.

While the disease is generally limited to the skin, massive cutaneous mast cell degranulation often results in systemic side effects. Gastrointestinal symptoms are common, including poor feeding, diarrhea, and, rarely, upper gastrointestinal bleeding. DCM patients are at high risk for anaphylaxis, which may be triggered by a variety of stimuli including vomiting, change in temperature, or flares of cutaneous disease.

Blistering disease generally resolves between 3 and 5 years of age. Unlike adult forms of mastocytosis, DCM is generally self-limiting, and patients experience complete resolution between 10 and 13 years of age. A small subset of patients have germ line mutations resulting in a familial form of DCM, which may have a more protracted course. Blistering lesions in these patients may resolve; however, they often continue to have other cutaneous features of DCM through adolescence and into adulthood.


Q82.2 – Mastocytosis

703826004 – Diffuse cutaneous mastocytosis

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Differential Diagnosis & Pitfalls

For blistering variants:
  • Staphylococcal scalded skin syndrome (SSSS)
  • Bullous impetigo
  • Epidermolysis bullosa (including Epidermolysis bullosa simplex, Junctional epidermolysis bullosa, and Dystrophic epidermolysis bullosa subtypes)
  • Atopic dermatitis (eczema)
  • Linear IgA bullous dermatosis of childhood
  • Bullous congenital ichthyosiform erythroderma
  • Early congenital syphilis
  • Kindler syndrome
  • Juvenile xanthogranuloma
  • Arthropod bite or sting
  • Giant congenital nevus
Diffusely infiltrated skin:
  • Lymphoma

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Last Reviewed:11/21/2017
Last Updated:06/14/2018
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Diffuse cutaneous mastocytosis in Child
A medical illustration showing key findings of Diffuse cutaneous mastocytosis (Skin Presentation) : Flushing, Bullae, Darier's sign positive, Erythroderma, Peau d'orange, Thickened skin, Widespread distribution, Pruritus, Vesicles
Clinical image of Diffuse cutaneous mastocytosis - imageId=1325657. Click to open in gallery.
Copyright © 2024 VisualDx®. All rights reserved.