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March 2010

Generalized Lentiginosis, Short Stature, and Multiple Cutaneous Nodules—Quiz Case

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Arch Dermatol. 2010;146(3):337-342. doi:10.1001/archdermatol.2010.7-a

A 14-year-old Indian girl presented in late childhood with generalized lentiginosis. Pigmented cutaneous lesions had been noticed at birth, with steady progression in number and distribution during childhood. There was a paternal history of short stature and multiple pigmented skin lesions. She had also developed a number of slowly progressive, generally asymptomatic, cutaneous nodules. There was no visual or hearing impairment and no developmental delay.

Examination demonstrated multiple lentigines on her head and neck, trunk, and limbs (Figure 1). Lentigines were also seen on the mucosal lips, tongue, and sclera. She was of very short stature with a height at age 11 years below the 0.4th percentile. Hypertelorism and low-set, posteriorly rotated ears were noted. Multiple skin-colored, asymptomatic to mildly tender dermal nodules were present predominantly on her arms (Figure 2). A biopsy of a cutaneous nodule was performed (Figure 3).