[POEMS syndrome with primary adrenocortical insufficiency].
BACKGROUND
POEMS syndrome is a multisystem disorder. It usually presents as severe polyneuropathy and monoclonal gammapathy associated with endocrinopathies, organomegaly, skin hyperpigmentation.


METHODS
A patient with POEMS syndrome developed primary adrenocortical deficiency revelated by asthenia and hyperpigmentation. Hydrocortisone replacement therapy produced a rapidly beneficial effect on asthenia and hyperpigmentation.


CONCLUSIONS
Although asthenia and skin pigmentation are common in POEMS syndrome, adrenocortical deficiency is seldom reported. This endocrinopathy might be underestimated in POEMS patients. We suggest systematic screening with a rapid ACTH test in all POEMS patients in order to detect underlying adrenal deficiency.
Mauvais-Jarvis
F
F
Clinique des Maladies endocriniennes et métaboliques, Hôpital Cochin, Paris.
Bertherat
J
J
Ravaud
H
H
Thomopoulos
P
P
Luton
J P
JP
D000304:Adrenal Cortex Function Tests; D000309:Adrenal Insufficiency / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D000324:Adrenocorticotropic Hormone / Q000032:analysis; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000150:complications* / Q000188:drug therapy
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone
Syndrome de POEMS avec insuffisance surrénalienne primaire.