I had an interesting case come into the clinic and I wanted to share it with all of you. This is the first time in my clinical experience with a patient who has full-thickness ulcerations due to necrobiosis lipoidica. Necrobiosis lipoidica is a disorder of collagen degeneration with a granulomatous response, thickening of blood vessel walls, and fat deposition. The main complication of the disease is ulceration, usually occurring after trauma to a particular area. Although infections are rare, full-thickness ulcerations appear to be rarer from my literature search. This particular patient had full-thickness ulcerations with an active infection on presentation.

Case Presentation

This particular patient was a 66 year old female with a history of anxiety, depression, type 1 diabetes, celiac disease, IgA deficiency, CVA (L sided), as well as porphyria, which meant she was unable to take any meds that are broken down through the liver. This made medical management of the infection quite difficult. Infectious disease was consulted to manage the infection portion of the care. She came into the center with punched-out bilateral circumferential pre-tibial full-thickness leg ulcerations caused from this disease. She also had classic telangiectatic vessels, which could be seen on the surface due to her thinning epidermis.

Her largest ulcer measured an area of 16.56 sq cm on initial evaluation. This ulcer was full-thickness and covered in foul-smelling yellow slough. Now, after infection/antibiotic management as well as topical treatment, she has progressed to a 1.56 sq cm shallow partial-thickness ulcer which has begun epithelialization. Although various treatments were tried, she made the most progress with PROMOGRAN PRISMA® Matrix, which logically makes sense as she is deficient in the production of collagen.

Use of Collagen for the Treatment of Necrobiosis Lipoidica

Treatment for necrobiosis lipoidica can feel hopeless at times for the patient, as the exact etiology remains unknown. In addition, any trauma can cause new ulcerations to occur. Spencei and Nahass described a case where they used topical bovine collagen. This is why we chose a collagen-based topical dressing for this patient. Collagen is believed to improve granulation tissue by supporting fibroblast activity and by promoting wound debridement by increasing the number of macrophages and neutrophils at the wound site.

Squamous cell carcinomas can also be linked to those that have chronic lesions due to necrobiosis lipoidica, however her biopsy was negative for this. This condition has also been linked to impaired neutrophil migration, which leads to an increased number of macrophages. This could possibly explain the raised granuloma formation that presents. Many researchers feel that this has a genetic link. However, no one in the patient's family has this ulceration disorder, but they do share the IgA deficiency, celiac disease, as well as the porphyria.

Image by Warfieldian (Own work) [CC BY-SA 3.0 (http://creativecommons.org/licenses/by-sa/3.0)], via Wikimedia Commons

References:
Clement M, Guy R, Pembroke AC. Squamous cell carcinoma arising in long-standing necrobiosis lipoidica. Arch Dermatol. Jan 1985;121(1):24-5
Lim C, Tschuchnigg M, Lim J. Squamous cell carcinoma arising in an area of long-standing necrobiosis lipoidica. J Cutan Pathol. Aug 2006;33(8):581-3.
Mistry N, Chih-Ho Hong H, Crawford RI. Pretibial angioplasia: a novel entity encompassing the clinical features of necrobiosis lipoidica and the histopathology of venous insufficiency. J Cutan Med Surg. Jan-Feb 2011;15(1):15-20
Spenceri EA, Nahass GT. Topically applied bovine collagen in the treatment of ulcerative necrobiosis lipoidica diabeticorum. Arch Dermatol. Jul 1997;133(7):817-8.

About the Author
Lindsay (Prussman) Andronaco is board certified in wound care by the Wound Ostomy Continence Nursing Certification Board. She also is a Diplomate for the American Professional Wound Care Association. Andronaco is the 2011 recipient of the Dorland Health People's Award in the category of 'Wound Ostomy Continence nurse' and has been recognized in Case In Point Magazine as being one of the "Top People in Healthcare" for her "passionate leadership and an overall holistic approach to medicine."