Living with Scleromyxedema

The Impact It has had on My Life

Scleromyxedema is a rare progressive disorder that mimics scleroderma and is characterized by widespread thickened skin and multi-system disease secondary to runcinous deposition in the skin and internal organs.

I was diagnosed 15 years ago by biopsy of the papules that I had on my hand, Scleromyxedema was confirmed. I was told of its rarity and of an unknown future. I was prescribed various treatments and felt like a guinea pig. Treatments ranged from Melphalen, high dose steroids, plasma phoresis and both Alpha and Beta interferon. Topical treatment was also prescribed by way of a tube of cream which I discovered was given to men with genital warts.

Psychologically over fifteen years the disease has been difficult to cope with. When my body started “growing” very visible papules, these were hidden by my clothes. When the papules started covering my face and neck emotionally it was a very different matter.

As well as this, smiling, opening my mouth, moving my face, moving my tongue and swallowing solid food became impossible. Painful muscles and severe fatigue were, and to a certain extent still are, a problem. - Psychologically, just being rare is daunting enough!

Raynauds is also a problem but three-monthly infusions of Iloprost have helped to relieve this aspect.

In 2005 I carried out some research on the internet and came across the John Hopkins Scleroderma Center in Baltimore (USA), which has patients with Scleromyxedema and discovered how the use of Immunoglobulins (IVIG) by regular infusions has changed their lives.

In May 2006 my Consultants agreed to let me have a trial of IVIG. After just one treatment my face started to be more supple and after the second month's treatment I started eating solid food for the first time in five years! By the time I had received the third monthly infusion the papules had cleared from my face and arms and I understand with regular infusions the disease will be kept under control.

I now feel like a new person and would like to conclude by saying during the fifteen years of coping with a rare disease my Consultants and medical staff have always treated me with kindness and done all they could to alleviate my illness. IVIG has changed my life and I cannot thank my Consultants enough for allowing me to have the opportunity of a more normal life.

Thank you. Ann Harris.