Treatment FAQ

who specializes in the treatment of pyoderma gangrenosum

by Alana Kilback Published 3 years ago Updated 2 years ago

Medication

Procedures

Self-care

What kind of doctor treats pyoderma gangrenosum?

Your doctor may refer you to a specialist in skin conditions (dermatologist).

Is pyoderma gangrenosum an autoimmune disease?

The exact cause of pyoderma gangrenosum is unknown (idiopathic) although it is suspected to be an autoimmune disease.

Does pyoderma gangrenosum ever go away?

Pyoderma gangrenosum can be difficult to treat. Treatment can leave some scarring in the affected area. Some people heal very slowly, over months or years. Others may find the condition clears up within a few weeks.

How serious is pyoderma gangrenosum?

Pyoderma gangrenosum is a rare but serious ulcerating skin disease, the treatment of which is mostly empirical. Pyoderma can present to a variety of health professionals and several variants exist that may not be recognised immediately. This can delay the diagnosis and have serious clinical consequences.

Can pyoderma gangrenosum lead to amputation?

In this article, we present a case of pyoderma gangrenosum (PG), misdiagnosed initially as a necrotizing infection that significantly worsened due to repeated surgical debridement and aggressive wound care therapy, almost resulting in limb amputation despite antibiotic therapy.

Is pyoderma life threatening?

PG is still a potentially life-threatening with a mortality rate of up to 30% in some series [70]. Deep purulent ulcerations due to pyoderma gangraenosum.

What bacteria causes pyoderma in humans?

Pyodermas are caused by bacterial colonization or invasion of the skin by coagulase-positive staphylococci, usually Staphylococcus intermedius. In chronic, recurrent, or deep pyodermas, secondary bacterial invaders may also be present, especially Pseudomonas spp., Proteus spp., and Escherichia coli.

How do you know pyoderma is healing?

There is often a halo of erythema surrounding the wound. When the wound starts to heal, you may see string-like projections of new skin starting to grow along the border of the ulcer bed and the skin. Once healed, the site may have a cribriform scar.

Should you debride pyoderma gangrenosum?

Surgery and pyoderma. For extensive wounds with or without necrosis, surgical therapy may be necessary for debridement and/or obtaining coverage.

How do you get rid of pyoderma?

TREATMENT OPTIONSVery superficial or localized cases of canine pyoderma may be treated with topical antibacterial medications alone (Table 1).Generalized or deep cases are usually best treated with a combination of oral antibiotics and topical antibacterial therapies (Table 2).More items...

How painful is pyoderma gangrenosum?

Pyoderma gangrenosum usually starts with a small, red bump on your skin, which may resemble a spider bite. Within days, this bump can develop into a large, painful open sore. The ulcer usually appears on your legs, but may develop anywhere on your body.

Is pyoderma gangrenosum vasculitis?

We found that, in the vasculitis category, Wegener's granulomatosis was the most frequent cause of pyoderma gangrenosum–like ulceration. Lesions resembling pyoderma gangrenosum and involving the head and neck — called “malignant pyoderma” by Perry et al.

What is pyoderma gangrenosum?

Pyoderma gangrenosum care at Mayo Clinic. Pyoderma gangrenosum is a rare condition that can have widespread effects on your body. Proper diagnosis and treatment are key to recovery. Your Mayo Clinic doctors will work with you to develop a comprehensive treatment plan that meets your needs and takes into consideration possible side effects ...

When was pyoderma gangrenosum first identified?

Pyoderma gangrenosum was first identified by Mayo doctors more than 80 years ago . More recently, Mayo clinicians researched the extent to which pyoderma gangrenosum is misdiagnosed and the types of treatment that work best for the condition. By Mayo Clinic Staff.

Can pyoderma gangrenosum be treated?

You may need treatment for other conditions related to pyoderma gangrenosum, such as chronic pain or depression. Your Mayo Clinic dermatologists can work with other Mayo Clinic specialists and your primary care doctor to ensure you get the care you need.

Departments and specialties

Mayo Clinic has one of the largest and most experienced practices in the United States, with campuses in Arizona, Florida and Minnesota. Staff skilled in dozens of specialties work together to ensure quality care and successful recovery.

Research

See a list of publications by Mayo Clinic doctors on pyoderma gangrenosum on PubMed, a service of the National Library of Medicine.

What is the best treatment for pyoderma gangrenosum?

Immunosuppressive therapies (drugs that suppress the immune system) are sometimes used to treat people with pyoderma gangrenosum. Cyclosporine is effective for many patients. Azathioprine and cyclophosphamide are also immunosuppressive drugs that have been used to treat PG.

What are the diseases associated with pyoderma gangrenosum?

Additional disorders associated with pyoderma gangrenosum include rheumatoid arthritis, acute and chronic myelogenous leukemia, myeloid metaplasia, and paraproteinemias. In some people,the development of pyoderma gangrenosum follows surgery or trauma. This condition is known as pathergy.

What is the name of the lump on the arm that is painful?

Classic pyoderma gangrenosum is often characterized by the presence of pus and can begin with pustules. Pustular pyoderma gangrenosum is characterized by painful bumps (pustules) most often found on the arms and legs. These lesions eventually develop into ulcerations. This form is often associated with inflammatory bowel disease.

How often does pyoderma gangrenosum occur?

Pyoderma gangrenosum affects women slightly more often than men. It occurs most often between the ages of 20 to 50 years. Infants or adolescents account for fewer than 4 percent of cases. One estimate places the incidence of PG at 1 in every 100,000 people in the United States.

What is a classic PG?

Classic PG also occurs near surgical openings (stoma sites) in the body. This condition is referred to as peristomal pyoderma gangrenosum. Atypical or bullous pyoderma gangrenosum is characterized by superficial blisters (bullae). This form of the disease most often affects the hands and is often associated with an underlying disorder especially ...

What are the symptoms of a gangrenosum?

Signs & Symptoms. Pyoderma gangrenosum often begins as small, quick-spreading reddish or purple colored bumps or blisters. These small growths eventually develop into swollen, open sores (ulcerations) with a well-defined blue or violet-colored border. The size and depth of ulcerations vary.

How to treat PG?

Treatment of PG consists of open wet dressings on the ulcers and topical application of anti-inflammatory creams and ointments such as corticosteroids. The skin must be protected from any other injury that could result in development of additional ulcers. In some cases, the grafting of new skin to the wound may be recommended once the inflammation is controlled.

What is a pyoderma gangrenosum?

Several variants exist, but the most common one is classic pyoderma gangrenosum. This presents as a deep ulcer with a well defined border, which is usually violet or blue. The ulcer edge is often undermined (worn and damaged) and the surrounding skin is erythematous and indurated (fig 1). The ulcer often starts as a small papule or collection of papules, which break down to form small ulcers with a “cat's paw” appearance. These coalesce and the central area then undergoes necrosis to form a single ulcer.

What is the best treatment for pyoderma?

Other immunosuppressive agents may be used—firstly, to reduce the dependence on corticosteroids and, secondly, because pyoderma is often resistant to treatment. When corticosteroids fail, the most widely used alternative is ciclosporin. Several case reports and small case series have demonstrated a good clinical response to low dose ciclosporin (level B evidence). Most patients show clinical improvement within three weeks with a dose of 3-5 mg/kg/day. Ciclosporin has several serious side effects, including nephrotoxicity, hypertension, and increased risk of cancer. Such side effects have not been reported for the low doses used to treat pyoderma.16,20

What is pustular pyoderma?

Pustular pyoderma is a rare superficial variant of the disease. Pyoderma often begins as a pustule or group of pustules that later coalesce and ulcerate. This process stops at the pustular stage in pustular pyoderma, and the patient has a painful pustular lesion that may persist for months (fig 3). Pustular pyoderma seems to be confined to patients with inflammatory bowel disease and tends to occur on the trunk and extensor surfaces of the limbs.6-8

How long does it take for a pyoderma to develop?

Pyoderma usually develops rapidly and can progress from a pimple to a crater in 24-48 hours

What is bullous pyoderma?

Bullous pyoderma is a superficial variant that affects the upper limbs and face more than the lower limbs. It is associated mostly with haematological conditions. This form of the disease presents as concentric bullous areas that spread rapidly in a concentric pattern. They may break down to form more superficial ulcers than those seen in classic pyoderma, although they still have the blue undermined edge. Prognosis is often poor because of the underlying haematological malignancy.8,9

Can pyoderma gangrenosum be seen on legs?

Classic pyoderma gangrenosum can occur on any skin surface, but is most commonly seen on the legs. Patients are often systemically unwell with symptoms such as fever, malaise, arthralgia, and myalgia. Lesions are usually painful and the pain can be severe. When the lesions heal the scars are often cribriform. Early diagnosis and prompt treatment reduce the risk of scars, and disfigurement may occur if the diagnosis is missed.1Pathergy occurs in 25-50% of cases—lesions develop at the site of minor trauma, so surgery or debridement are contraindicated.2

Is pyoderma gangrenosum a serious skin disease?

Pyoderma gangrenosum is a rare but serious ulcerating skin disease, the treatment of which is mostly empirical. Pyoderma can present to a variety of health professionals and several variants exist that may not be recognised immediately. This can delay the diagnosis and have serious clinical consequences.1The mainstay of treatment is long term immunosuppression, often with high doses of corticosteroids or low doses of ciclosporin. Recently, good outcomes have been reported for treatments based on anti-tumour necrosis factor α, and infliximab proved effective in a randomised controlled trial. This article reviews the presentation of pyoderma gangrenosum and the therapeutic options available.

What is PG treatment?

Recent Advances:PG treatment typically involves reducing inflammation, controlling pain, promoting wound healing, and treating the underlying etiology. Recent advances have been made with regard to targeted therapies for PG with topical, intralesional, and systemic medications.

What is PG in wound care?

Significance:Pyoderma gangrenosum ( PG) is a rare neutrophilic ulcerative dermatosis that poses a high burden of morbidity due to underdiagnosis, resistance to therapy, and limited therapeutic options. Optimization of wound care strategies and multimodal anti-inflammatory approaches are necessary to mitigate multiple converging pathways ...

What is wound management?

Wound management includes gentle cleansing without sharp debridement, limited topical antibacterial use, and maintenance of a moist environment to promote epithelial migration. Critical Issues:Wound dressings and compression therapy, in particular, introduce a wide variety of therapeutic options.

What is the first line of treatment for PG?

First-line treatment is aimed at optimising local wound care , particularly important in cases of PG arising on the leg, where wound healing can be delayed by vascular disease. Supportive therapy with appropriate dressings, compression (if arterial insufficiency has been excluded) and adequate analgesia are all essential to optimise healing. Potent topical corticosteroids and tacrolimus ointment applied to the ulcer surface are useful and intralesional injections of corticosteroid into the erythematous active border may be considered.35

What are the most common systemic conditions associated with PG?

Underlying systemic conditions are found in up to 50% of cases and so it is imperative to try and identify such conditions once a diagnosis of PG has been made.14Most frequently associated conditions include inflammatory bowel disease in up to 30% of cases, rheumatoid arthritis and seronegative arthritides in up to 10% of cases, haematological malignancies or monoclonal gammopathies (in particular immunoglobulin A gammopathy) in 5% of cases and other malignancies in 5%. Other systemic conditions have also more rarely been associated with PG, including those of chronic infection and inflammation.1,16Drugs such as propylthiouracil, tyrosine kinase inhibitors, TNFα inhibitors and granulocyte-colony stimulating factor have been implicated but the underlying disease for which the medication has been prescribed may be the triggering factor.17

What is the importance of skin biopsy?

PG remains a clinical and sometimes challenging diagnosis and although histology of skin biopsies can be supportive, the main value of the skin biopsy is to exclude other causes of cutaneous ulceration and to allow specimens to be sent for bacterial, mycobacterial and fungal cultures. The biopsy should include the active border of the ulcer and penetrate deep to subcutaneous tissues. Patients should be warned that such a surgical procedure will invariably cause an enlargement of the ulcer, as well as potentially inducing a pathergic immunological response to trauma.

What is a classic PG?

Classical PG presents most commonly as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/erythematous border.1The lower legs are most frequently affected although PG can present at any body site. The lesion may be precipitated by minor trauma, a phenomenon known as ‘pathergy’.6Indeed PG lesions are all too often misdiagnosed as simple non-healing ulcers and patients undergo debridement which can result in catastrophic deterioration of the condition through this pathergic response. The condition predominantly affects adults but childhood cases are rarely reported.7Although there may be some families with PG and inherited syndromes in which PG is a feature, the majority of patients do not have a family history of the condition.

Is there a diagnostic criteria for PG?

Thus far there are no validated, established diagnostic clinical or pathological criteria to diagnose PG. Su et alhave proposed a diagnostic tool requiring two major and two minor criteria (see Table ​Table1),1), maintaining PG as a diagnosis of exclusion.30More recently, Maverakis et alhave proposed new criteria based on a consensus of international experts, requiring one major and four minor criteria (see Table ​Table22).31This is yet to be widely adopted, but no longer renders PG as a diagnosis of exclusion which may thus provide an improved diagnostic tool.

Does pyrin inhibit inflammasome activation?

A genetic basis for PG has so far been documented in the syndromic presentations with mutations in the PSTP1P1/CD2BP1gene in PAPA and PASH syndromes. Normally pyrin inhibits inflammasome activation but the PSTPIP1mutant inhibits the anti-inflammatory effect of pyrin and this results in the release of pro-inflammatory cytokines.27The results of more sophisticated exome sequencing and next generation sequencing in other patients with PG are awaited.

Is PG treatment based on case series?

Treatment of PG remains largely anecdotal with only two randomised control led trials (RCT), and is therefore based largely on case series and poorly evidenced publications.32,33With no national or international guidelines, management is therefore challenging, and treatment choice is based on the severity and extent of the PG (Fig ​(Fig33).34

How to treat pyoderma gangrenosum?

Pyoderma gangrenosum is generally managed through a stepwise approach using a combination of systemic and topical therapies. Immunosuppression with corticosteroids and ciclosporin is the mainstay of treatment, with the use of other agents largely dependent on local protocols and experience. [1-3]

Why is pyoderma gangrenosum difficult to diagnose?

Diagnosis of the condition is difficult due to a lack of biomarkers or other distinguishing features, and treatment can also be challenging. No single specific therapy for pyoderma gangrenosum is available and few clinical trials have been performed into the condition.

What is the best treatment for ulcers?

Topical Treatments. Topical treatment is essential for the management of emerging ulcers. Highly potent topical corticosteroids have been shown to induce remission in certain cases, while triamcinolone injected into the ulcer edge and topical tacrolimus have both been shown to be effective certain patients. [1] .

Why is systemic therapy important?

Systemic therapy is particularly important in patients with widespread or rapidly progressing disease. Most patients are prescribed oral corticosteroids early in the disease to try to induce remission. Patients receiving long-term steroid therapy may also require a bone-protecting agent such as minocycline.

Can surgery be used for pyoderma gangrenosum?

In extreme cases, surgery may be used, although this can itself be a trigger for pyoderma gangrenosum. [2] . Autologous split-skin grafts have been used with some success, although this necessitates the creation of a new wound at the donor site. Use of bioengineered skin may be an option in the future.

Is pyoderma gangrenosum unpredictable?

Unpredictability. Treatment of pyoderma gangrenosum is often based on trial and error, and long-term outcomes are unpredictable. However, if poorly managed, the condition can be severely debilitating with a devastating effect on quality of life.

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