Case of the Month

This 87-year-old-woman has had a non-healing ulcer on her leg (with rolled boarders) for 4 ½ years.

What's Your Diagnosis?

Diagnosis: Pyoderma Gangrenosum

This 87-year-old-woman has had a non-healing ulcer on her leg (with rolled boarders) for 4 ½ years. The ulcer was scheduled to be surgically debrided. At the care facility, one of the medical assistants told her that her grandmother had had a stubborn rash for a few years and a dermatologist (Dr. Crutchfield) had helped her clear it up. She suggested that the patient make an appointment with me to see if there was anything I could do before engaging in a surgical treatment.

Upon examination, I suspected pyoderma gangrenosum. I confirmed this diagnosis with a skin biopsy.

Treatment

We started weekly Unna-boot dressings/changes, gentle Hibiclens washes with saline rinses, and Cyclosporine at a dose of approximately 5mg/kg daily. She weighed 120 lbs. and she was given 300mg of Cyclosporine daily with the appropriate lab monitoring. She tolerated the medication and treatments very well. The last 4 weeks there were some stubborn crusts (see 2nd photo) that we also applied Lauripure ointment (https://lauripure.com/) with CBD oil to decrease crust retention. After 6 months of treatment, the pyoderma gangrenosum had resolved! Additional information

DEFINITION

An inflammatory process that often begins as a suppurative folliculitis manifested clinically as pustules. It rapidly extends to become ulcers marked at their periphery by boggy tissue. The skin disease is associated commonly with inflammatory bowel disease, especially ulcerative colitis, and sometimes with rheumatoid arthritis, paraproteinemia, and myeloma. ADJUNCTIVE DIAGNOSTIC TESTS Search should be undertaken for evidence of systemic diseases such as inflammatory bowel disease, arthritis, paraproteinemia, and leukemia.

COURSE

The first lesion to appear in pyoderma gangrenosum is a folliculocentric pustule that in a short time ulcerates and becomes surrounded by a boggy undermined inflamed border. That border and the ulcer behind it expand centrifugally, sometimes reaching dimensions of many centimeters. It takes many months, and sometimes years, for the ulcer of pyoderma gangrenosum to heal, the residuum being an atrophic scar that exhibits hypopigmentation and hyperpigmentation.

INTEGRATION: UNIFYING CONCEPT

Pyoderma gangrenosum, like Sweet’s syndrome, erythema multiforme, erythema nodosum, and allergic vasculitis, is a distinctive morphologic pattern that results from different causes. In the case of pyoderma gangrenosum, the causes may be inflammatory bowel disease, ulcerative colitis in particular, rheumatoid arthritis, paraproteinemia, and chronic active hepatitis. The bullous expression of pyoderma gangrenosum often is a manifestation of a leukemia, and the pyostomatitis vegetans manifestation of pyoderma gangrenosum usually is a reflection of an inflammatory disease of the bowel. The pustule that heralds the explosive changes that lead to the devastating ulceration of pyoderma gangrenosum is seen histopathologically to be a suppurative folliculitis. When the collection of neutrophils within the infundibulum of a follicle becomes so great that the follicle ruptures, spewing countless neutrophils into the dermis, the stage is set for the phagedenic changes that soon ensue. The contents of extraordinary suppuration in the dermis and subcutaneous fat are discharged to the surface of the severely ulcerated skin. At the periphery of the ulcers, the epidermis is separated from the dermis and appears to “overhang” the edge of the denuded skin. All of the findings just described become progressively more marked over time until, for reasons not understood, the inflammatory process ceases, and the lesion heals with scar. The mechanism whereby pyoderma gangrenosum develops is not known, but the condition seems to be related closely to Sweet’s syndrome, the boggy plaques of which also are made up of dense, diffuse infiltrates of neutrophils. Sweet’s syndrome, like pyoderma gangrenosum, is secondary to a variety of processes as disparate as ulcerative colitis, rheumatoid arthritis, and acute myeloid leukemia.

THERAPY

If an underlying process can be identified, such as ulcerative colitis or rheumatoid arthritis, treatment should be directed toward correcting it. For the ulcers themselves, in addition to local care, systemic treatment with corticosteroids especially, but also of either sulfones or sulfapyridine, is also effective. Chemotherapeutic agents, such as azathioprine and cyclophosphamide, are beneficial in some patients. Newer immunomodulating agents like cyclosporine, mycophenolate mofetil, and tacrolimus (FK506) seem to be of therapeutic value.

As mentioned earlier, she was managed well with weekly Unna-Boot dressings and Cyclosporine.

Reference:

A CLINICAL ATLAS OF 101 COMMON SKIN DISEASES With Histopathologic Correlation A. BERNARD ACKERMAN, HELMET KERL, JORGE SANCHEZ, YING GUO, ANGELIKA HOFER, PAUL KELLY, TETSU KIMURA, GIOVANNI BORRONI, CHARLES CRUTCHFIELD, VOLKER STEINKRAUS, WOLFGANG WEYERS