PF13. Sindromul Netherton (ihtioza lineara circumflexa)
Florica Chiotan, Victoria Balasoiu, Elena Naumescu, Luminita S. Munteanu,
J. D. Diaconu
Bucuresti

Se prezinta cazul unui copil de 31/2 ani, de sex feminin, cu placi eritemato-scuamoase cu contururi policlinice si neregulate, mai active spre periferie, eritem descuamativ pe fata, gat si pielea capului, intens pruriginoase, cheilita angulara si cu modificari ale firului de par - tricorexie invaginata si ruperea firelor de par la 1,5 cm de la emergenta; dezvoltare psihosomatica normala, conforma cu varsta.

Sora copilului, in varsta de 2 ani, prezinta dermatita atopica.

Investigatiile de laborator au evidentiat: aminoacidurie slab pozitiva pentru aminoacizi aromatici, prezenta stafilococului auriu prin insamantari de la nivelul leziunilor.

Tratamentul urmat: oxacilina, prednison, peritol si local rombalsam 20% si unguent cu corticoizi.

PF13. Netherton's Syndrome (Ichthyosis Linearis Circumflexa)
Florica Chiotan, Victoria Balasoiu, Elena Naumescu, Luminita S. Munteanu,
J. D. Diaconu
Bucharest

We present a 31/2-old year female having erythematosquamous plaques with polycyclinic, irregular and active borders, desquamative erythema on the face, neck and scalp, severe itching and angular cheilitis associated with hair defects: trichorrhexis invaginata and hair breakage at 1.5 cm above follicular orifices; physical and neurological examinations are normal.

The patient has a 2-year old sister who suffers from atopic dermatitis.

Laboratory investigations shows low level aminoaciduria (aromatic amino acids) and skin cultures positive for Staphylococus aureus.

The systemic treatment consists of antibiotics (oxacilina), corticosteroids (prednnison) and antihistamines (peritol); local treatment consists of topical steroid preparation and rombalsam 20%.