Annals
of Dermatol 1998;10(1);39-43
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. (Ann Dermatol 10:(1) 39-43,
1998). |
