Treatment of scleromyxoedema with hydroxychloroquine.
BACKGROUND
Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. Patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis.


METHODS
Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG lambda paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p.o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter.


RESULTS
Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61+, 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months.


CONCLUSIONS
Hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.
Terheyden
Patrick
P
Department of Dermatology, University of Würzburg, Würzburg, Germany. terheyden-p.derma@mail.uni-wuerzburg.de
Becker
Jürgen C
JC
Lurz
Christa
C
Kahaly
George J
GJ
Bröcker
Eva B
EB
D000328:Adult; D003879:Dermatologic Agents / Q000627:therapeutic use; D004890:Erythema / Q000209:etiology / Q000473:pathology* / Q000517:prevention & control*; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009230:Myxedema / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D010818:Practice Patterns, Physicians'; D045743:Scleroderma, Diffuse / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome
D003879:Dermatologic Agents; D006886:Hydroxychloroquine