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May 2013

A Maroon Plaque in an Infant

Author Affiliations

Author Affiliations: University of Texas Southwestern Medical Center, Dallas (Mss Wang and Ma and Drs Savory, Cope-Yokoyama, and Agim); Departments of Pathology (Dr Cope-Yokoyama) and Dermatology (Dr Agim), Children's Medical Center, Dallas.

JAMA Dermatol. 2013;149(5):629-630. doi:10.1001/jamadermatol.2013.251

Large congenital masses appropriately raise clinical concern, especially when they do not present with the classic appearance, thus requiring consideration of broad differential diagnostic categories prior to conclusive diagnosis. We discuss analysis and treatment of a vascular lesion most closely resembling a fibrous hamartoma of infancy (FHI).

A 13-day-old healthy female infant presented for evaluation of a “lump” on her right buttock. She was born at 37 weeks' gestation via scheduled cesarean section. Pregnancy was complicated by gestational diabetes, and birth by initial oxygen saturation of 60% that had to be normalized with supplementation. The plaque appeared asymptomatic at birth and had not changed much by the time of presentation. Physical examination revealed a 3 × 2-cm maroon, firm, well-demarcated, nonblanching plaque with a palpable subcutaneous component and overlying hypertrichosis on the right buttock (Figure 1). Manipulation produced piloerection (positive pseudo-Darier sign). Histopathologic analysis revealed two predominant components, a prominent proliferation of spindle-shaped cells admixed with numerous round blood vessels lined by plump endothelial cells throughout the dermis and subcutis (Figure 2).