[Scleroedema adultorum Buschke in a diabetic subject: intravenous immunoglobulin therapy].
BACKGROUND
Scleroedema adultorum Buschke (SB) is a rare disease involving scleroedema of the neck and shoulders. It can extend to the rest of the trunk and the limbs but characteristically spares the extremities. Three types of SB are distinguished: the first is acute and develops after an infectious disease, the second is of insidious evolution and is associated with monoclonal gammopathy, and the third is associated with type 2 diabetes.


METHODS
We report the case of a type 2 diabetic patient presenting with progressive, oedematous timbering of the trunk associated with impaired mobility, dysphagia and restrictive respiratory syndrome. SB was diagnosed on the basis of clinical presentation and histology. Treatment was mandatory because of the adverse impact of the disease. A therapy that would not worsen the patient's comorbidities had to be chosen. Intravenous immunoglobulins were thus initiated with excellent response as of the first cycle regarding trunk mobility and dysphagia. Cutaneous rigidity improved steadily until the end of treatment (eight cycles).


CONCLUSIONS
Therapeutic abstention is the rule in SB if it has no severe functional repercussions. Nevertheless, there is no clearly indicated treatment once therapy becomes necessary. Control of underlying diabetes usually does not improve the scleroedema and the metabolic syndrome contraindicates most of the treatments reported in the literature. In this article, we suggest a new treatment of SB in the diabetic patient.
Barde
C
C
Service de dermatologie et vénéréologie, hôpital universitaire de Genève, 24, rue Micheli-Du-Crest, 1211 Genève 14, Suisse. caroline.barde@hcuge.ch
Masouyé
I
I
Saurat
J-H
JH
Le Gal
F-A
FA
D003680:Deglutition Disorders / Q000209:etiology; D003924:Diabetes Mellitus, Type 2 / Q000150:complications*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012592:Scleredema Adultorum / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous