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Observation
July 2018

Warty Fingers and Toes in a Child With Congenital Lymphedema: Elephantiasis Nostras Verrucosa

Author Affiliations
  • 1Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
  • 2Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
  • 3Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
JAMA Dermatol. 2018;154(7):849-850. doi:10.1001/jamadermatol.2018.0210

Hennekam syndrome is a rare congenital disorder characterized by primary, asymmetrical lymphedema that develops in the prenatal period and commonly affects the face, extremities, genitalia, and mucosal linings.1 We herein report a child with Hennekam syndrome who presented with distinct cutaneous findings.

A preschool child with Hennekam syndrome presented for the evaluation of gradually progressive warty skin lesions on hands and feet. The child was otherwise healthy and had not experienced recurrent infections, lymphangitis, or cellulitis. The CD4 and CD8 cell counts and levels of immunoglobulins IgG, IgA, IgM, IgD, and IgE were normal. Examination revealed asymmetrical, nonpitting edema of the upper limbs, right lower limb, and genitalia. Interestingly, the dorsal aspect of the right hand and foot showed multiple, skin-colored to pinkish, round to filiform, translucent, papulovesicular lesions coalescing to form verrucous excrescences, chiefly over the knuckles and periungual areas (Figure 1).

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