Goltz syndrome (Focal Dermal Hypoplasia) with exuberant fat herniations, syndactyly, papillomas, skin defects, eye involvement and false positive anti HIV serology

Goltz syndrome (focal dermal hypoplasia) is a rare congenital mesoectodermal dysplasia with multisystemic involvement. it was first described by goltz in 1962 and more than 200 cases reported worldwide (leite et al., 2005). skin lesions are characterized by athrophic, hyperpigmented linear or reticulated macules, multiple mucocutaneous papillomas, fat herniations or fat tumors. besides the skin, other structures frequently involved are the skeletal system, eyes, teeth, hair, nails and central nervous system (kanitakis et al., 2003).the association between goltz syndrome and anti-hiv serology was not found in the literature. we report 32 year-old woman with typical cutaneous leisons of goltz syndrome associated with false positive anti-hiv serology and secondary infertility.