[Hypothalamic-pituitary Langerhans cell histiocytosis in adults].
BACKGROUND
Langerhans cell histiocytosis is a rare entity. Involvement of the pituitary region is frequently delayed and rarely studied.


METHODS
We report some clinical and paraclinical particularities of the disease in four women. Diabetes insipidus was the initial symptom in all cases, accompanied by panhypopituitarism at initial presentation in one woman. Hypopituitarism was diagnosed from 6 months to 4 years after the diabetes insipidus in the other cases, and systemic lesions appeared 3 to 6 years thereafter. Magnetic resonance imaging of the hypothalamic-pituitary axis found tumors in two patients and infundibular thickening in the other two. One patient also had a partially empty sella.


CONCLUSIONS
In adults, pituitary involvement seems to occur predominantly in women, and panhypopituitarism is possible at initial presentation. The clinical polymorphism of Langerhans cell histiocytosis makes this disease difficult and often slow to diagnose.
Essais
Olfa
O
Service d'Endocrinologie, Hôpital Militaire Principal d'Instruction de Tunis, Tunisie. olfaessais@yahoo.fr
Jomaa
Rached
R
Bougerra
Radhia
R
Marrakchi
Zinet
Z
Ben Salem
Leila
L
Ouertani
Haroun
H
Chamakhi
Saïd
S
Neji
Sonia
S
Ben Slama
Claude
C
Zidi
Borni
B
D000328:Adult; D003919:Diabetes Insipidus / Q000209:etiology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D007027:Hypothalamic Diseases / Q000175:diagnosis*; D010900:Pituitary Diseases / Q000175:diagnosis*