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).
Bishnoi A, Vinay K, Vishwajeet V, Saikia UN, Panigrahi I, Kumaran MS. Warty Fingers and Toes in a Child With Congenital Lymphedema: Elephantiasis Nostras Verrucosa. JAMA Dermatol. 2018;154(7):849–850. doi:10.1001/jamadermatol.2018.0210
Dermatology in JAMA: Read the Latest
Customize your JAMA Network experience by selecting one or more topics from the list below.