When A Non-Healing Wound Has A Dermatologic Origin

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Author(s): 
William Fishco, DPM, FACFAS

   Anthrax is caused by a bacterium called Bacillus anthracis. Humans can become infected with the spore-forming bacteria by close contact with farm animals that are infected. The bacterium typically enters the body through an open skin lesion. Active infection not only includes skin breakdown but there is typically nausea, vomiting, malaise, fever and sore throat.

What You Should Know About Pyoderma Gangrenosum

Pyoderma gangrenosum is a non-infectious neutrophilic dermatosis. Typically, the wounds start with vesicles, bullae and/or pustules. Later in the disease process, the skin breaks down to full-thickness ulcerations of skin with undermined violaceous borders.

   Ulcers associated with pyoderma gangrenosum are painful. Legs are the most common anatomic region associated with pyoderma gangrenosum but all skin including mucous membranes can be affected. In many cases, pyoderma gangrenosum may be associated with an underlying medical condition. These conditions include inflammatory bowel disease, rheumatologic disorders, hepatitis C, leukemia, lymphoma and drug-induced cases.

   One can make the diagnosis via biopsy and exclusion. There are no laboratory parameters to aid in the diagnosis. Histopathology is typically non-specific. Suppurative folliculitis and dense neutrophil infiltrates are typically visible. Treatment of pyoderma gangrenosum lesions is usually more medically oriented versus surgical. Debridement of a pyoderma gangrenosum wound can trigger pathergy and the wound can worsen. Medical treatment usually includes oral and topical corticosteroids, immunosuppressive agents, sulfa drugs and/or cytotoxic drugs.

   For review of the laboratory data from our patient, she had a white blood cell count of 7.2 with a normal differential. Her hemoglobin was 9.8 g/dL. All serology markers were negative. Her radiographs showed mild periosteal reaction of the leg, the MRI was negative for osteomyelitis and Doppler studies were negative for venous reflux or obstruction. The cultures of the wound and blood were negative. The biopsy of the wound revealed no vasculitis and various stages of ulceration and granulation tissue.

   Despite not having classic neutrophil infiltrates in the biopsy, I made the diagnosis of pyoderma gangrenosum by exclusion. The patient is currently undergoing medical treatment and has shown improvement over the past six months. Initially, she took methylprednisolone (Solu-Medrol, Pfizer) 1 g daily for three days and the wound showed immediate improvement. She is currently on a regimen of minocycline and dapsone. She is applying clobetasol (Temovate, GlaxoSmithKline) ointment and Adaptic (Systagenix) with a light dressing daily. Every other day, she is performing a bleach bath of the leg, which is composed of 1 tablespoon of bleach per gallon of water.

In Conclusion

Pyoderma gangrenosum can be a limb- and life-threatening dermatosis. The diagnosis occurs by biopsy and exclusion of other dermatologic conditions. If one suspects pyoderma gangrenosum, be cautious with any surgical debridement, which can make the ulcer worse. This difficult case’s diagnosis and management entailed a multidisciplinary approach including podiatry, internal medicine, vascular specialists and dermatology. Ultimately, the definitive diagnosis occurred with negative serological markers and biopsy that ruled out carcinoma and infection.

   Dr. Fishco is board-certified in foot surgery and reconstructive rearfoot and ankle surgery by the American Board of Podiatric Surgery. He is in private practice in Phoenix. Dr. Fishco is also a faculty member of the Podiatry Institute.

Reference

1. Savige J, Davies D, Falk RJ, et al. Antineutrophil cytoplasmic antibodies and associated diseases: A review of the clinical and laboratory features. Kidney International. 2000; 57(3):846–862.

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Eli Regevsays: February 3, 2014 at 1:07 pm

Thank you presenting this interesting case for us. Pyoderma gangrenosum is a cutaneous manifestation of internal diseases as you mentioned and especially IBD.
In the following case, neither the skin biopsy or the clinical background support this diagnosis. What was the reaction to biopsies? Was there was any deterioration in wound condition due to this wound biopsies? Was there any pathergy sign?

What do you think? Is the disease causing this skin ulceration? Any way, the steroids she received are non significant and may be not enough if pyoderma gangrenosum is the correct diagnosis.

(The side effects of minocycline and Dapsone are small generally so they are acceptable even there is no definitive diagnosis._

I think topical treatment with "bleach" is unnecessary (I use it very rarely in my practice) and can be harmful, further retarding the healing of this wound. Modern dressings and especially silver dressing s(e.g.Acticoat-3, Smith and Nephew) can make a big difference in my hands.

I wonder what is the basic disease causing this ulceration?
if no deterioration occurred after skin biopsies and if there is no pathergy sign, I would consider skin grafting this wound or using a skin substitute

I will be happy to obtain further information about this patient.

Best regards,

Eli Regev, MD
Plastic Surgery
Wound management

Tel-Aviv, Israel

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William D. Fishcosays: March 2, 2014 at 12:39 pm

Thank you for your comments. I will try to respond/answer all of your questions and comments.

1. Skin biopsy/pathergy/clinical background. Histological biopsy will usually be non-specific and difficult to differentiate from other inflammatory diseases. However, its benefit is to rule out suspicion of malignancy. Biopsy is performed only if clinical suspicion of malignancy exists, due to the risk for pathergy. May also culture for fungal or mycobacterial infection prior to initiation of immunosuppressive treatment. Our patient did not have pathergy from the biopsy. However, pathergy occurs in 20-40% of those with the disease, leaving the other 60-80% without any pathergy. Systemic therapy: corticosteroids are the gold standard tx for PG. Methotrexate, cyclosporine and antibiotics can be complementary to therapy. IV immunoglobulin for intractable PG. However, this can be cost prohibitive and only used if all else fails. Infliximab can also be used.

2. Topical bleach treatments. The bleach baths were prescribed by the dermatology service and we have since discussed this with them and had them discontinued. We switched to Aquacel Ag (another silver dressing). The patient recently had serial debridements and application of Integra grafts.

3. What is the underlying disease? Certainly, we have so far ruled out any underlying malignancies. The diagnosis is essentially made by exclusion. Considering the clinical improvement with a reasonable algorithm for PG, we are confident with the diagnosis of PG without a clear underlying etiology.

Some final thoughts regarding PG. There are two variants of PG. There is the classic ulcerative form, which usually occurs on lower limbs (and trunk, vulva, penis, head, neck and breast). There is also the atypical form, which occurs on the upper limbs and consists of three subtypes. These subtypes are as follows:
a) Pustular. This is most often associated with patients who have coexisting inflammatory bowel disease. Usually, this PG resolves in line w/ resolution of the active mucosal disease of the gut. However, only 50% of the pustular PG have an underlying systemic disease.
b) Bullous. This is associated with myeloid malignancies (i.e. leukemia or myelofibrosis). This presents as painful, superficial blistering with eroded dermatosis. The ulcer can be deep with violaceous erythema in the surrounding area. Treatment of the underlying malignancy can result in simultaneous resolution of the PG.
c) Vegetative. This is confined to the skin with no associated systemic disease. It presents as raised, verrucous, well-defined plaques that are studded with small pustules. Ulcerations occur on the trunk without a violaceous border, pustular base or undermining.

Thank you again for your comments and suggestions. As you know, PG can be a challenging to diagnose and treat.

References

Callen JP, Jackson JM. Pyoderma Gangrenosum: An Update. Rheumatic Disease Clinics of North America. 33(2007) 787-802.

Ratnagobal S, Sinha S. Pyoderma Gangrenosum: Guideline For Wound Practioners. Journal of Wound Care 22(2), February 2013.

Brooklyn T, Dunnil G, Probert C. Diagnosis and Treatment of Pyoderma Gangrenosum. Clinical Review. BMJ 333, July 22, 2006.

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