Pyoderma gangrenosum

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Pyoderma gangrenosum
Crohnie Pyoderma gangrenosum.jpg
Pyoderma gangrenosum on the leg of a person with ulcerative colitis.
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg
Usual onset40s or 50s [1]
Treatment Corticosteroids, ciclosporin, infliximab, canakinumab [2]

Pyoderma gangrenosum is a rare, inflammatory skin disease where painful pustules or nodules become ulcers that progressively grow. [3] Pyoderma gangrenosum is not infectious. [3]

Contents

Treatments may include corticosteroids, ciclosporin, infliximab, or canakinumab. [2]

The disease was identified in 1930. It affects approximately 1 person in 100,000 in the population. Though it can affect people of any age, it mostly affects people in their 40s and 50s. [1]

Types

Pyoderma gangrenosum Pyoderma gangrenosum 01.jpg
Pyoderma gangrenosum

There are two main types of pyoderma gangrenosum: [1]

Other variations are: [4]

Presentation

Associations

The following are conditions commonly associated with pyoderma gangrenosum: [7] [8]

A rare [10] syndromic association called pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPA syndrome), a type of autoinflammatory disorder, is associated with mutations in the proline-serine-threonine phosphatase-interacting 1 gene ( PSTPIP1 ). [10] [11]

Causes

Though the cause is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. In support of an immune cause, a variety of immune mediators such as interleukin (IL)-8, IL-1β, IL-6, interferon (IFN)-γ, granulocyte colony-stimulating factor, tumor necrosis factor alpha, matrix metalloproteinase (MMP)-9, MMP10, and elafin have all been reported to be elevated in patients with pyoderma gangrenosum. [12]

Also in support of an immune cause is the finding that at least half of all pyoderma gangrenosum patients suffer from immune-mediated diseases. [1] For instance, ulcerative colitis, rheumatoid arthritis, [4] and monoclonal gammopathies [13] have all been associated with pyoderma gangrenosum. It can also be part of autoinflammatory syndromes such as PAPA syndrome. [10] [11] Marzano et al. (2017) identified a variety of single-nucleotide polymorphisms (SNPs) linked to autoinflammation that were carried, singly or in combination, in subsets of patients with pyoderma gangrenosum, acne and suppurative hidradenitis syndrome (PASH syndrome) or isolated pyoderma gangrenosum of the ulcerative subtype. [14]

One hallmark of pyoderma gangrenosum is pathergy, which is the appearance of new lesions at sites of trauma, including surgical wounds. [15]

Diagnosis

Diagnosis of PG is challenging owing to its variable presentation, clinical overlap with other conditions, association with several systemic diseases, and absence of defining histopathologic or laboratory findings. Misdiagnosis and delayed diagnosis are common. It has been shown that up to 39% of patients who initially received a diagnosis of PG have an alternative diagnosis. [16] In light of this, validated diagnostic criteria have recently been developed for ulcerative pyoderma gangrenosum. [17]

Diagnostic criteria

In addition to a biopsy demonstrating a neutrophilic infiltrate, patients must have at least 4 minor criteria to meet diagnostic criteria. [17] These criteria are based on histology, history, clinical examination and treatment.[ citation needed ]

Treatment

First-line therapy for disseminated or localized instances of pyoderma gangrenosum is systemic treatment with corticosteroids and ciclosporin. Topical application of clobetasol, mupirocin, and gentamicin alternated with tacrolimus can be effective. Pyoderma gangrenosum ulcers demonstrate pathergy, that is, a worsening in response to minor trauma or surgical debridement. Significant care should be taken with dressing changes to prevent potentially rapid wound growth. Many patients respond differently to different types of treatment, for example some benefit from a moist environment, so treatment should be carefully evaluated at each stage.[ citation needed ]

Papules that begin as small "spouts" can be treated with Dakin's solution to prevent infection and wound clusters also benefit from this disinfectant. Wet to dry applications of Dakins can defeat spread of interior infection. Heavy drainage can be offset with Coban dressings. Grafting is not recommended due to tissue necrosis.[ citation needed ]

If ineffective, alternative therapeutic procedures include systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and ciclosporin; tacrolimus; thalidomide; infliximab; or plasmapheresis. [18]

See also

Related Research Articles

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Ulcerative colitis (UC) is one of the two types of inflammatory bowel disease (IBD), with the other type being Crohn's disease. It is a long-term condition that results in inflammation and ulcers of the colon and rectum. The primary symptoms of active disease are abdominal pain and diarrhea mixed with blood (hematochezia). Weight loss, fever, and anemia may also occur. Often, symptoms come on slowly and can range from mild to severe. Symptoms typically occur intermittently with periods of no symptoms between flares. Complications may include abnormal dilation of the colon (megacolon), inflammation of the eye, joints, or liver, and colon cancer.

<span class="mw-page-title-main">Psoriasis</span> Skin disease

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<span class="mw-page-title-main">Mouth ulcer</span> Sore on the mucous membrane of the oral cavity

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<span class="mw-page-title-main">Immunosuppressive drug</span> Drug that inhibits activity of immune system

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<span class="mw-page-title-main">Febrile neutrophilic dermatosis</span> Medical condition

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<span class="mw-page-title-main">Chronic inflammatory demyelinating polyneuropathy</span> Medical condition

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<span class="mw-page-title-main">Lupus erythematosus</span> Human disease

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Pathergy is a skin condition in which a minor trauma such as a bump or bruise leads to the development of skin lesions or ulcers that may be resistant to healing. Pathergy can also lead to ulcerations at the site of surgical incisions. Pathergy is seen with both Behçet's disease and pyoderma gangrenosum. A highly similar phenomenon known as the Koebner phenomenon occurs in autoimmune diseases such as psoriasis and systemic lupus erythematosus, among others.

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Palisaded neutrophilic and granulomatous dermaititis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows.

<span class="mw-page-title-main">PAPA syndrome</span> Genetic disorder in humans

PAPA syndrome is a rare genetic disorder characterised by its effects on skin and joints. The acronym PAPA stands for pyogenic arthritis, pyoderma gangrenosum and acne.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which a person's blood plasma is allowed to cool substantially, causing the (reversible) precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins.

<span class="mw-page-title-main">Livedoid vasculopathy</span> Medical condition

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Superficial granulomatous pyoderma is a cutaneous condition, a variant of pyoderma gangrenosum characterized by a localized superficial vegetative or ulcerative lesion, which usually follows trauma, such as surgery.

Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.

<span class="mw-page-title-main">Behçet's disease</span> Inflammatory disorder

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis. The sores can last from a few days, up to a week or more. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go.

<span class="mw-page-title-main">Enteropathic arthropathy</span> Medical condition

Enteropathic arthropathy commonly referred to as enteropathic arthritis, is a type of arthritis linked to Crohn's disease, ulcerative colitis, and chronic inflammatory bowel diseases.

<span class="mw-page-title-main">Colonic ulcer</span> Medical condition

Colonic ulcer can occur at any age, in children however they are rare. Most common symptoms are abdominal pain and hematochezia.

References

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