Atypical Wounds: Scleroderma, Marjolin's Ulcer and Kaposi's Sarcoma (Part 2)
By Martin Vera, LVN, CWS
Part 2 in a series discussing the etiology, assessment and management of atypical wounds. Read Part 1 here.
As our journey through the exciting and uncharted, choppy waters of atypical wounds comes to a close, I discuss a few more wounds. The purpose and goal of this blog are to create awareness of other wounds that exist and instill the curiosity in my fellow clinicians to get our research done.
This blog describes a few atypical wounds, including scleroderma, Marjolin's ulcer, and Kaposi's sarcoma (KS). Even with the previous discussion of atypical wounds in this two-part series, many other atypical wounds exist, and I encourage and challenge you to educate yourselves and others, continue doing the research necessary to continue the battle to prevent and heal these wounds, and increase awareness to achieve early detection and have better chances for positive outcomes.
This condition relates to the occlusion of digital vessels associated with excess collagen deposition in those areas causing tissue fibrosis. The condition extends from an autoimmune disorder that is of unknown etiology with progressive inflammation. Affected patients may experience systemic or organ-specific abnormalities, with 30% to 40% of patients developing skin ulcerations during the course or duration of the disease. Scleroderma manifests with skin tightening, itching, and discoloration and with Raynaud's phenomenon (discoloration of fingers or toes secondary to changes in temperature associated with abnormal spasms to blood vessels from diminishing blood or oxygen supply). Some patients experience loss of muscle control in the esophagus that causes cough, difficulty swallowing, and even heartburn, as well as joint pain and muscle weakness.
Common wound sites include the fingertips, knuckles, and elbows, and wounds usually occur over a bony prominence or pressure point. Calcinosis or calcium deposits develop normally in the fingers and cause fibrotic changes, itchy and dry skin, and ulcerations in some instances; the fingers are angular in appearance. Patients have deformed fingers, and telangiectasias (small red spots that appear on the fingers, inside the mouth, and on the face) are common. Laboratory findings include Scl-70 antibody, a specific sclerosis marker and, as discussed in part 1 of this series, CREST syndrome, consisting of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Research into scleroderma treatment is ongoing, but current therapy may include the following: instruction on smoking cessation; debridement, as appropriate; use of bioengineered skin; advanced wound care products, as appropriate; decellularized tissue substitutes; non-steroidal anti-inflammatory drugs; low doses of corticosteroids; narcotics for pain control and to prevent spasms; phototherapy with ultraviolet A or ultraviolet A1 or photochemotherapy (PUVA) ; and physical therapy to assist with skin softening.
Marjolin's ulcer is a very aggressive and rare skin cancer that has been known to develop in scar tissue from burns, chronic wounds caused by trauma, pressure injuries, venous leg ulcers, lupus, snakebites, hidradenitis suppurativa, and osteomyelitis, as well as in other areas that have been affected by chronic inflammation. Marjolin’s ulcer has a very low incidence rate of only 1% to 2% in all burn scars. This type of wound is most commonly seen in the form of squamous cell carcinoma, although it has been identified in other types of cancer, such as basal cell carcinoma, melanoma, liposarcoma, osteosarcoma, fibrosarcoma, and angiosarcoma, just to name a few. It has a latent period of 20 to 30 years, give or take. As research continues to evolve, multiple factors are understood to play a role in the development of Marjolin's ulcer. The literature suggests that the decreased vascularity, combined with weakened epithelium in chronic wounds, favors susceptibility to carcinogens. Also at risk are areas of frequent trauma and inflammation and areas of skin that have been exposed to long-term irritation. There is also, of course, multifactorial combination of both genetic and environmental factors.
As discussed, these wounds may manifest anywhere on the body. If you suspect that a wound is developing Marjolin's ulcer, a biopsy is indicated. The recommended sampling areas are the edges and center of the wound. Treatment continues to evolve with ongoing research. In many cases, wide excisions are recommended to prevent local recurrence, and large areas also may be excised down to bone, depending on the extent of tissue or organs involved. Magnetic resonance imaging is used to assess the amount and extent of destruction and inflammation of tissues. In rare cases, amputation and even excision of reproductive organs are necessary because of the presence of metastases. Treatment is driven by the extent of tissue destruction. Chemotherapy and radiation therapy are appropriate treatment modalities, followed by localized wound care with the use of advanced wound care products. Clinicians must remember and instruct their patients that wound healing is delayed after chemotherapy or radiation therapy, and maintaining an infection-free wound is critical. The prognosis is poor. It is of the utmost importance to be able to provide early wound coverage and to assess and detect unstable scar tissue and chronic non-healing and non-responsive wounds.
KS is a type of cancer that lines lymph or blood vessels. It usually manifests as tumors on the skin, inside the mouth, or on mucosal surfaces; it can also appear in other areas of the body, such as the lungs, lymph nodes, and digestive tract. These lesions are malignant tumors of the epithelial and lymphocytic cells related to herpetic viruses and to human immunodeficiency virus disease. The severity of these lesions, with pain, discomfort, and even life threatening or serious problems, depends on their location. The abnormal cells of KS have specific characteristics: they cause blotches or visible tumors to appear on the skin, and they can be red, brownish, and purplish. Lesions that may appear on the groin or leg areas may cause painful swelling of the legs and feet. Lesions that may appear on the gastrointestinal tract, liver, or lungs may manifest with more serious presenting symptoms, such as bleeding and difficulty breathing, if tumors have developed on the lungs. There is a specific table to assist with staging of KS, and more information may be obtained through the American Cancer Society.
Diagnosing KS initially depends on the MD's suspicion. A medical history review and a physical exam are warranted, including an inspection of visible lesions on the skin and assessment of the inside of the mouth for any lesions. If lesions suspected of being KS are found on the skin or mouth, a biopsy will solidify the diagnosis. Punch biopsy is common on skin lesions, but an MD may opt for an excisional biopsy. If KS is suspected in other areas of the body, then an MD may order chest radiographs, gastrointestinal (upper and lower)endoscopy, or bronchoscopy. As our research remains ongoing, and more and more of this type of KS-associated atypical wound is being evaluated and treated, our arsenal of treatment has improved over the last decade. Depending on the stage of disease, location, and involvement, MDs have several ways of approaching these wounds and may use local therapy for small and localized areas, as well as chemotherapy, radiation therapy, and even immunotherapy. For individuals with compromised immune systems, the most important treatment consists of maintaining immune health and keeping infections under control. Some treatments may cause wounds, such as radiation therapy, and the use of advanced wound care products is warranted. The SWAT team is very important, and it comprises an infectious disease MD, a dermatologist, and an oncologist, in addition to the primary care provider, social worker, dietitian, nurses, and all the regular staff on board.
In conclusion, in this pair of blogs, I was able to describe just a few atypical wounds. There are many more out there, and awareness is crucial. We health care providers who dedicate the majority of or all our time to the field of wound management must continue to educate ourselves and others about these uncommon wound types that exist outside of the more common wounds we see in our usual practice. Our work setting is also a factor in the type of rare or atypical wounds that may be more prevalent. Research is strong and ongoing on all the wounds named, and more treatments are on the horizon and possibly already up and running. My goal was simply to raise an eyebrow and be able hit the curiosity button just enough to draw your interest and possibly generate more research from my fellow colleagues. As always, stick to your guidelines, review your P&P, consult the SWAT team, and mentor, mentor, mentor.
Keep healing, my friends!
American Cancer Society. Kaposi's Sarcoma. Available at: https://www.cancer.org/cancer/kaposi-sarcoma. Accessed May 31, 2018.
Kerr-Valentic MA, Samimi K, Rohlen BH, Agarwal JP, Rockwell WB: Marjolin's ulcer: modern analysis of an ancient problem. Plast Reconstr Surg. 2009;123(1):184–91.
Shah JB, Sheffield PJ, Fife CE, editors. Wound Care Certification Study Guide, 2nd ed. North Palm Beach, FL: Best Publishing; 2016:Chapter 21.
Tobin C, Sanger JR. Marjolin's ulcer: a case series and literary review. Wounds. 2014;26(8):248–54.
About the Author
Martin Vera is a certified wound specialist with over 20 years of nursing experience, with a passion for wound management and patient-centered care.
The views and opinions expressed in this blog are solely those of the author, and do not represent the views of WoundSource, Kestrel Health Information, Inc., its affiliates, or subsidiary companies.