Ann Dermatol.  2010 Feb;22(1):77-80. 10.5021/ad.2010.22.1.77.

Diffuse Cutaneous Mastocytosis with Generalized Bullae

• Lee EH
• Kim MR
• Kang TW
• Kim SC

• KMID: 2172045
• DOI: http://doi.org/10.5021/ad.2010.22.1.77

Abstract

We report on a 9-month-old female infant with multiple tense bullae and erosions covering the entire body, including the face, scalp, and trunk. The histopathological examination revealed sub-epidermal bullae with a dense dermal cellular infiltrate. The infiltrate was identified as a collection of mast cells using toluidine blue and Giemsa stains. The direct immunofluorscence was negative. A diagnosis of cutaneous diffuse mastocytosis with generalized bullae was made based on these clinical and histopathological findings. In cases with diffuse cutaneous mastocytosis with generalized bullae, systemic involvement is more frequent and more severe compared to other types of cutaneous mastocytosis. Some lethal outcomes have been reported. This is the first reported case of diffuse cutaneous mastocytosis in the Korean literature.

Keyword

Diffuse cutaneous mastocytosis; Generalized bullae

MeSH Terms

Azure Stains
Blister
Female
Humans
Infant
Mast Cells
Mastocytosis
Mastocytosis, Cutaneous
Scalp
Tolonium Chloride
Transcutaneous Electric Nerve Stimulation
Azure Stains
Tolonium Chloride

Figure

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  Fig. 1 (A, B) Multiple tense bullae and erosions developed with peau d'orange-like skin on the face, scalp, and trunk. (C) Darier's sign was positive on the thigh.

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  Fig. 2 (A) Sub-epidermal bullae with a dense cellular infiltration in the upper dermis (H&E, ×200) and (B) dense mast cell infiltration with some eosinophils in the upper dermis (H&E, ×400).

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  Fig. 3 Mast cells were stained with toluidine blue (A: ×200) and Giemsa stains (B: ×200).

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