Incontinentia Pigmenti Associated with Congenital Heart Diseases

Incontinentia pigmenti(IP) is an X-linked dominantly inherited disorder with female predominance. Skin lesions are characterized by three or four stages; vesicobullous, verrucous, hyperpigmented and hypopigmented lesions. About 80% of patients with incontinentia pigmenti have one or more associated ectodermal or mesodermal anomalies involving teeth, nail, hair, eye, breast, bones and nervous system.

A newborn girl had erythematous based vesicles and bullae on her trunk and extremities with peripheral eosinophilia. Within several days, she showed linear verrucous plaques. A skin biopsy specimen showed eosinophilic spongiosis in the epidermis and numerous eosinophils in the dermis. The diagnosis of IP was made. She was revealed to have some congenital heart anomalies; atrial septal defect (ASD) and patent ductus arteriosus(PDA).

Cases of IP with congenital heart disease have been reported very rarely. Therefore, we report this unique case of IP associated with ASD and PDA.