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Scleromyxedema, Lichen Myxedematosus or Papular Mucinosis

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
What is Scleromyxedema?
Causes of Scleromyxedema
Treatments for Scleromyxedema
Scleromyxedema Support
Scleromyxedma Personal Stories
References

What is Scleromyxedema?

Scleromyxedema is a scleroderma-like condition. However, it causes thickening and hardening that makes the skin look too ample, rather than too tight, as in scleroderma. It is also referred to as Scleromyxedema of Groton, lichen myxedematosus, and papular mucinosis. (2)

Causes of Scleromyxedema

Generalized papular and sclerodermoid eruption: scleromyxedema. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Serdar ZA. (PubMed) Indian J Dermatol Venereol Leprol. 2010 Sep-Oct;76(5):592.
Nephrogenic systemic fibrosis in advanced chronic kidney disease: a single hospital's experience in Taiwan. Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD) clinically resembles scleromyxedema which develops in the setting of advanced chronic kidney diseases. Chen W. (PubMed) Eur J Dermatol. 2009 Jan-Feb;19(1):44-9.

Treatments for Scleromyxedema

Intravenous immunoglobulin (IVIg) therapy for scleromyxedema: a case report and review of literature. Scleromyxedema is a chronic, idiopathic disorder characterized by cutaneous dermal mucin deposition in association with increased dermal collagen and absence of thyroid disease. Sroa N. J Drugs Dermatol. 2010 Mar;9(3):263-5. (Also see: IVIg)
Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin. Increasing evidence supports intravenous immunoglobulin as an effective and relatively safe treatment for both cutaneous and extracutaneous manifestations of scleromyxedema, including the dermatoneuro syndrome. JAAD, Volume 60, Issue 6, June 2009.

Scleromyxedema Support

Scleromyxedema Online Support Groups. We currently have two support groups with over 100 members. Bonnie, Scleromyxedema.com.

Scleromyxedema Personal Stories

Phyllis K. Phillips: Scleromyxedema I have been living with scleromyxedema for ten years, and as you may know, there is very little information about it...

References

(2) Scleromyxedema information from the book Systemic Sclerosis, First Edition by Dr. Phil Clements and Dr. Daniel Furst, pages 100-102.
 
Go to Diseases Similar to Scleroderma: Vitiligo
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