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Bullous Mastocytosis: Report of a Patient and a Brief Review of the Literature

Avshalumov, Kristina B.A*; Pichardo, Rita MD†; Jorizzo, Joseph L MD†; Sangueza, Omar P MD†; Goldenberg, Gary MD‡

American Journal of Dermatopathology: October 2008 - Volume 30 - Issue 5 - pp 455-457
doi: 10.1097/DAD.0b013e3181783354
Extraordinary Case Report

We report a 6-month-old male infant who presented to the pediatric dermatology clinic at Wake Forest University Baptist Medical Center with a generalized bullous eruption since 3 months of age. A sepsis work up was performed at an outside hospital before presentation and did not reveal any evidence of systemic infection. Clinical presentation revealed a well-nourished, appropriate-for-age, 6-month-old boy with multiple tense bullae, some in a “string of pearls” arrangement, on the bilateral upper extremities and trunk. Multiple erosions were also noted. Laboratory evaluation revealed a normal complete blood count. Polymerase chain reaction was negative for herpes simplex virus types I and II. Histologic sections demonstrated a large space of separation between the epidermis and dermis which was filled by a monomorphous infiltrate composed of round to oval cells with centrally placed nuclei, consistent with mast cells. Leder and C-Kit stains were strongly positive, confirming the diagnosis of bullous mastocytosis. Treatment included fluocinonide 0.05% cream and tacrolimus 0.1% ointment to active lesions and silver sulfadiazine 1% cream to erosions. Improvement was noted during follow-up examination.

From the *Albert Einstein College of Medicine of Yeshiva University, Bronx, NY; †Department of Dermatology, Wake Forest University School of Medicine, Winston-Salem, NC; and ‡Department of Dermatology, University of Maryland School of Medicine, Baltimore, MD.

Funding sources: none.

Conflict of interest disclosure: none.

This case was presented as a poster at the American Society of Dermatopathology Meeting, Boston, MA, 2004.

Reprints: Gary Goldenberg, MD, Department of Dermatology, University of Maryland School of Medicine, 405 West Redwood Street, 6th floor, Baltimore, MD 21201 (e-mail: garygoldenbergmd@gmail.com).

© 2008 Lippincott Williams & Wilkins, Inc.