Korean J Perinatol.  2014 Jun;25(2):105-109. 10.14734/kjp.2014.25.2.105.

A Case of Diffuse Cutaneous Mastocytosis in a Newborn

• Park MN
• Kim GA
• Chey MJ
• Shim GH

• KMID: 2280962
• DOI: http://doi.org/10.14734/kjp.2014.25.2.105

Abstract

Diffuse cutaneous mastocytosis (DCM) is a rare variant of mast cell disease with widespread erythema and is clinically apparent in early infancy. We report the case of a 1-day-old female neonate who presented with diffuse flush, pruritus, and extensive blistering. DCM was diagnosed by immunohistochemical staining with anti-CD117, which revealed mast cell infiltration. DCM is a severe and heterogeneous cutaneous disease, and is associated with mast cell mediator-related symptoms and risk of anaphylactic shock. We describe this case and provide the first literature review of neonatal onset DCM in Korea.

Keyword

Cutaneous mastocytosis; Diffuse cutaneous mastocytosis; Newborn

MeSH Terms

Anaphylaxis
Blister
Erythema
Female
Humans
Infant, Newborn*
Korea
Mast Cells
Mastocytosis
Mastocytosis, Cutaneous*
Pruritus

Figure

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  Fig. 1. Clinical presentation: Patient at birth with diffuse erythematous rash on back (A). Partly hemorrhagic vesicle and bulla on axilla (B). Hemorrhagic crusts and erosion found on the face (C). Diffuse cutaneous mastocytosis with erythema and leathery thickened skin on abdomen (D).

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  Fig. 2. Histopathology and immunohistochemistry of tissue specimens from skin lesions. Histological appearance of a patient with diffuse cutaneous mastocytosis (A, magnification ×100). Diffuse infiltration of mast cells in the dermis (B, magnification ×400).

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  Fig. 3. Mast cell infiltration was demonstrated by CD117 staining (brown).

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