[Efficacy of intravenous immunoglobulin in the treatment of scleromyxoedema].
BACKGROUND
Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG).


METHODS
A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease.


CONCLUSIONS
The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.
Sillard
L
L
Service de dermatologie, hôpital Archet 2, CHU de Nice, route de St-Antoine-de-Ginestière, 06200 Nice, France.
Passeron
T
T
Cardot-Leccia
N
N
Perrin
C
C
Lacour
J-P
JP
Ortonne
J-P
JP
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D024821:Metabolic Syndrome / Q000150:complications; D008875:Middle Aged; D009077:Mucins / Q000032:analysis; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis; D010266:Paraproteins / Q000032:analysis; D012074:Remission Induction; D053718:Scleromyxedema / Q000188:drug therapy* / Q000209:etiology; D012867:Skin / Q000737:chemistry / Q000473:pathology; D013792:Thalidomide / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007146:Immunoglobulin lambda-Chains; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D009077:Mucins; D010266:Paraproteins; D013792:Thalidomide