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Title: Cutaneous mastocytosis with atypical mast cells in a 7-year-old girl
Authors: Marrero Alemán, Gabriel
El Habr, Constantin
Islas Norris, Diana
Montenegro Dámaso, Tarsila
Borrego, Leopoldo 
Sangueza, Omar P.
UNESCO Clasification: 32 Ciencias médicas
Keywords: Cutaneous mastocytosis
Atypical mast cells
Urticaria pigmentosa
Systemic mastocytosis
Issue Date: 2017
Journal: American Journal of Dermatopathology 
Abstract: Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. To our knowledge, there are only 4 reported cases of cutaneous mastocytosis with atypical mast cells. We hereby report a case of urticaria pigmentosa in a 7-year-old female patient. The patient presented with asymptomatic skin lesions of several years duration over the neck and left scapular area. Histopathological examination revealed the presence of middermal perivascular infiltrates mainly composed of mast cells, few lymphocytes, and eosinophils. Most mast cells showed pleomorphic nuclei with bilobed and multilobed morphology that revealed a positive expression for CD117, tryptase, CD68, and Giemsa stains. Based on these findings the diagnosis of urticaria pigmentosa with atypical mast cells was made. Additional tests to rule out systemic involvement were performed. All values, including a tryptase level, were within normal limits. No changes were noted after 1-year follow-up.
ISSN: 0193-1091
DOI: 10.1097/DAD.0000000000000768
Source: American Journal of Dermatopathology[ISSN 0193-1091],v. 39, p. 310-312
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