A Face Almost Too Tight to Smile

A Face Almost Too Tight to Smile

By Richard P. Holm, MD
 
For years I cared for a lovely woman with scleroderma. It all started with Raynaud’s phenomenon a condition causing her fingers, after an exposure to cold, to become pale-white then blue or red and painful. Raynaud’s phenomenon occurs in about four percent of the population, is usually not associated with any other condition, but sometimes goes with rheumatoid arthritis, lupus or, in this case, scleroderma. My patient also experienced progressively tight and shiny skin on her hands, then chest, then everywhere. She had tiny red spider vein spots (telangiectasia), heartburn, weight loss, weakness and puffy joints. Over the years her fingers became scarred and quite immobile with always worsening sores on the tips. Through it all, this wonderful lady had a very positive attitude and always seemed to have a smile on her face, although, near the end, the skin of her face became so tight that smiling became a challenge.
 
Yearly, about three in 100,000 middle-aged U.S. adults develop scleroderma (or when full-blown called systemic sclerosis). Normally, after a cut or a sore develops and in order to heal the wound, our bodies trigger both the immune system (to fight off invasion of bacteria, fungi and viruses) and special repair cells (to close off the wound with collagen and scar tissue). Once the damage has been repaired, extra collagen production usually stops, but not with scleroderma. For some unknown reason, scarring continues uncontrolled. At this time, treatment for this very puzzling auto-immune condition is restricted to treatment of symptoms; no cure for scleroderma exists.
 
We think that inheritance, hormones, certain infections and long-term exposure to pesticides, silica dust or polyvinyl chloride all might have something to do with the cause, but we really don’t know. We also don’t know why four times as many women get it as men; why it it can involve just a spot or a sword shape skin area and never progress; why it may or may not occur with Raynaud’s phenomenon, involvement of the esophagus and smaller parts of the skin but doesn’t progress; and why, in some unfortunate cases, it progresses everywhere, like in my patient.
 
Similar to many other autoimmune conditions, scleroderma is one where the immune system, for some undefined reason, runs amok, mistaking normal tissue as a foreign invader. Scientists and physicians are on the hunt to learn first why scleroderma happens and then how to stop the process.
 
If we understood more about scleroderma, we would all find it easier to smile.
 
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