When A Non-Healing Wound Has A Dermatologic Origin

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Author(s): 
William Fishco, DPM, FACFAS

   Venous stasis ulcerations are the most common wounds that occur on the medial aspect of the lower leg due to the course of the great saphenous vein. Venous stasis ulcerations are typically shallow/superficial, have an irregular border, often look weepy and wet, and are usually not painful. There is typically concomitant edema on the leg. In addition to the ulceration, the skin color is usually discolored purple or brown, superficial varicosities may be visible, there is often hardening (induration) of soft tissues, and accompanying dry scaling skin may be visible (venous stasis dermatitis).

   One confirms the diagnosis with an ultrasound test to determine the degree of venous reflux. Treatment includes wound care and compression. Mechanical compression can occur with dressings, compression hosiery and/or lymphedema pumps. Surgical treatments may include endovenous laser treatment and/or skin grafting of the wound. Ultimately, one needs to address the swelling disorder or the risk of recurring ulcers is great.

   A group of granulomatous diseases that include vasculitis can cause skin ulcerations. These conditions include Wegener’s granulomatosis and Churg-Strauss syndrome.

   Wegener’s granulomatosis is also known as granulomatosis with polyangiitis. Patients with this syndrome typically have kidney and lung disease. The main serological marker is anti-proteinase 3 antineutrophil cytoplasmic antibodies. This marker can aid in the diagnosis of Crohn’s disease and inflammatory bowel disease.

   Churg-Strauss syndrome is also known as eosinophilic granulomatosis with polyangiitis. This is an autoimmune disorder in patients with atopy (allergic hypersensitivity). Symptoms include upper respiratory disorders including asthma and reactive airway disease. There may be history of hay fever, allergic rhinitis and sinusitis. Elevations of perinuclear anti-neutrophil cytoplasmic antibodies may be present in about 50 percent of the cases of Churg-Strauss syndrome.1 In a complete blood count with differential, the eosinophils will typically be 10 percent or higher whereas a normal differential of eosinophils should be between 1 and 4 percent.

   Non-melanoma carcinomas may cause chronic wounds that do not respond to wound care. A common non-melanoma carcinoma affecting the skin is squamous cell carcinoma. Carcinomas are skin cancers that are typically slow growing and affect sun-exposed areas. Sites of skin that have been burned, scarred or ulcerated for a long period of time are susceptible to cancerous changes. Less common non-melanoma skin cancers include Kaposi’s sarcoma, which is common in patients with HIV infection or in otherwise healthy elderly males from Mediterranean ancestry. Also, primary cutaneous lymphoma (mycosis fungoides), which is a low-grade lymphoma, can be associated with ulcerations and tumors. One can confirm these conditions through biopsy and histodiagnosis.

   Ulcerations of skin can occur from arterial insufficiency. Unlike venous stasis ulcers, arterial ulcers are painful and deeper. A classic arterial ulcer has a “punched out” appearance. Contrary to venous stasis wounds, these ulcers are more likely to be on the lateral side of the leg/ankle (or on the foot). The skin is usually cool to touch and other signs of peripheral arterial disease (such as loss of hair growth or pale color of skin surrounding the wound) are present. The skin may be thin, atrophic and shiny. Testing to rule out arterial insufficiency includes ankle-brachial index, Doppler studies and angiography.

   Infectious diseases can also be the cause of the wound. Sporotrichosis is a fungal infection caused by Sporothrix schenckii. The fungus is ubiquitous throughout the world and is present in the soil. The organism is most commonly introduced through the skin by a rose thorn prick. The classic infection includes suppurating nodules along a lymphatic channel. A less common presentation is pulmonary sporotrichosis that disseminates to organs including skin to cause lesions.

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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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