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Urticaria Pigmentosa in Monozygotic Twins

Teresa Boyano, MD; Tomas Carrascosa, MD; Jesus Val, MD; Nieves Porta, MD; Jose L. Agud, MD; Maria J. Garcia, MD
Arch Dermatol. 1990;126(10):1375-1376. doi:10.1001/archderm.1990.01670340127033.
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To the Editor.—  The hallmark of mastocytosis is an increased number of tissue mast cells. In the pediatric age group, the most frequent forms are urticaria pigmentosa and diffuse cutaneous mastocytosis. In the systemic form, mast cell infiltration of bone marrow, liver, spleen, small intestine, and skin is common.We describe two monozygotic twins presenting simultaneously with urticaria pigmentosa. To the best of our knowledge, this is the tenth pair with the disease described in the literature.1,2

Report of Case.—  We examined our patients at the age of 8 months, with a follow-up to the age of 4 years.No evidence of consanguinity or a family history of mastocytosis were disclosed.At 3 months of age, reddish-brown, slightly elevated papules were noted. The papules were 1 to 5 cm in diameter, with an irregular shape and generalized distribution, although the face, palms, and soles were initially spared. Some lesions


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