With the first issue of 2000, our journal’s title was changed to include the word skin and reflect the increasing recognition that our readers care for people with both skin and wound care needs.1 Some diseases, including scleroderma, have a significant impact on the skin. The disease process includes skin fibrosis, vascular abnormalities, and autoantibody production.2 Scleroderma often presents with a “woody” skin that loses its flexibility; can you imagine your skin being hard like a tree trunk, not moving, even with pushing and pulling?
Limited scleroderma (a slower developing, more benign form of the disease) is usually distal to the neck, elbows, and knees with diffuse involvement having proximal lesions as well.2 The Scleroderma Foundation lists other problems encountered by persons with limited scleroderma.3 Known as the CREST syndrome, they are calcinosis or calcium deposits in the skin, Raynaud phenomenon (discoloration of digits with temperature change), esophageal dysfunction, sclerodactyly (woody hard digits), and telangiectasia (small linear arteriolar vessels on the skin surface). Remember, there are many types of scleroderma, and not all people who have scleroderma will have all of these symptoms—each person may have different combinations. Scleroderma often first presents with skin involvement or Raynaud phenomenon, but internal organs can also become involved.
This month’s CE/CME highlights a literature review of scleroderma with earlier diagnosis and newer treatment modalities to address its symptoms. This is especially important given that there is no cure and patients are living longer with the disease. However, we should focus not only on the skin changes (“holes” or wounds, digital ulcers, and tight skin lesions) that a person may have, but also the “whole” person. Scleroderma is largely a rheumatologic disease that affects predominantly young females (30s–50s). We encourage you to think about how this disease impacts quality of life and the lifestyle changes that may be needed. This is especially important during a time of life when young persons are active, focused on their appearance, and the emphasis is on “doing” with their social peers.
The restrictions on upper extremity movement that accompany scleroderma such as limited mobility and impaired joint motion are life altering. How does a younger person adjust and live his/her life with frequent, severe pain in the distal fingers and toes, or the risk for secondary infection, disfigurement, and even finger amputation from ischemia? Imagine not being able to open your mouth wide enough to eat solid foods comfortably, and then suffering abdominal pain from intestinal motility dysfunction. How does a person with scleroderma deal with painful calcium deposits on his/her fingertips? Fingers that are bent and will not straighten and flexed knees that inhibit normal gait have a profound social impact. Unfortunately, most current treatment does not improve skin flexibility, leaving only the option of frequently applying emollient moisturizers to hydrate the skin surface and prevent skin breakdown.
Providers can still help relieve distal digit pain that may be associated with skin ulcers on the tips of the fingers and toes. Severe cases of distal digit ischemia may require intravenous prostaglandin inhibitors and hospitalization. Patients with less severe Raynaud phenomenon often respond to several options including an angiotensin inhibitor blocker (losartan), calcium-channel blocker (nifedipine), or phosphodiesterase type 5 antagonists (sildenafil, tadalafil). Warm gloves or socks along with nitroglycerine patches (12 hours on and 12 hours off but never with phosphodiesterase type 5 antagonists orally) can also improve activities of daily living for persons living with scleroderma.
Nurse theorist Martha E. Rogers4 wrote about her humanistic science for the whole human being: “Man is a unified whole possessing his own integrity and manifesting characteristics that are more than and different from the sum of his parts.”
Your editors-in-chief have adapted those words and do believe that the person with a “hole” (ie, a wound, ulcer, injury, or skin changes) is more than the sum of his/her parts. As skin and wound healers, we must always be aware of, assess, and address person/patient-centered concerns as part of the individual plan of care.
Elizabeth A. Ayello, PhD, RN, CWON, ETN, MAPWCA, FAAN
R. Gary Sibbald, BSc, MD, DSc (Hons), MEd, FRCPC (Med Derm), FAAD, MAPWCA, JM
1. Doan-Johnson S. Welcome to the start of something new. Adv Skin Wound Care 2000;13(1):6.
2. Goodfield MJD, Coulson IH. Scleroderma. In Lebwohl M, Heymann W, Berth-Jones J, Coulson IH, eds. Treatment of Skin Disease. 5th ed. Philadephia, PA: Elsevier; 2017.
4. Rogers ME. An Introduction to the Theoretical Basis of Nursing. Philadelphia, PA: FA Davis Company; 1971.