This condition presents suddenly in the form of a small, reddened, painful pustule, most commonly due to any trauma affecting the skin. This pustule may then grow bigger in size and eventually burst, leaving behind an ulcer. The ulcer usually keeps enlarging and there may be more than one such ulcers. They also rapidly deepen and are extremely painful.

The ulcer(s) may keep on enlarging or start healing on their own, depending upon the host's immune system.

Culture negative pulmonary infiltrates are the most common extracutaneous presentation [7].

• Pain

  Pyoderma gangrenosum presents as a rapidly enlarging, very painful ulcer. It is one of a group of autoinflammatory disorders known as neutrophilic dermatoses. The name pyoderma gangrenosum is historical. [dermnetnz.org]

  For now I’ll get you some pain relief and the nurses to redress it.” [medicaleducationleeds.com]

  Case Presentation: A 46 year old male with chronic history of sniffing cocaine presented to the emergency department for recurrent painful lower extremity ulcers. He had previously failed a course of clindamycin therapy. [shmabstracts.com]

  Hyperbaric oxygen therapy (HBOT) has been shown effective in the treatment of PG, reducing pain and tempering the need for medication. [ncbi.nlm.nih.gov]

• Gangrene

  K43.1-, K44.1, K45.1, K46.1 ) gangrene in other peripheral vascular diseases ( I73.- ) gangrene of certain specified sites - see Alphabetical Index gas gangrene ( A48.0 ) pyoderma gangrenosum ( L88 ) Type 2 Excludes gangrene in diabetes mellitus ( E08 [icd10data.com]

  From pyoderma + scientific Latin gangrenosum, neuter singular of gangrenosus gangrenous. Pronunciation pyoderma gangrenosum /ˌpʌɪə(ʊ)ˈdəːmə ˌɡaŋɡrɪˈnəʊzəm/ [en.oxforddictionaries.com]

  [Disease/Finding], pyoderma gangenosum, pyoderma gangrenosa, pyoderma gangrenous, gangrenosum pyoderma, gangrenous pyoderma, Pyoderma gangrenosum, Pyoderma gangrenosum (disorder), gangrenosum; pyoderma, pyoderma; gangrenosum Spanish PIODERMA GANGRENOSO [fpnotebook.com]

  The name pyoderma gangrenosum is a misnomer; it has neither an infectious nor gangrenous etiology. Pyoderma gangrenosum is commonly associated with systemic disease. [aocd.org]

• Malaise

  RESULTS: Although PSPG can demonstrate fever, malaise, systemic signs, and serology that mimic the sepsis of necrotizing fasciitis, we identify some features that can aid diagnosis of pyoderma gangrenosum in the absence of pathergy (which is pathognomonic [ncbi.nlm.nih.gov]

  Systemic symptoms such as fever and malaise are common. The ulcers can coalesce to form larger ulcers, often with cribriform or sieve-like scarring. Symptoms and signs can vary with the subtype. [msdmanuals.com]

  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. [doi.org]

  Systemic symptoms such as fever and malaise are common. The ulcers can coalesce to form larger ulcers, often with cribriform or sieve-like scarring. [merckmanuals.com]

• Falling

  Figure Presentation and successful treatment of a pyoderma gangrenosum–like lesion in a 79-year-old patient with cognitive impairment and frequent falls. (A) Lesion on the right lower extremity at follow-up (November 26, 2017). [jaoa.org]

  ABSTRACT Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. There are several subtypes, with ‘classical PG’ as the most common form in approximately 85% cases. [rcpjournals.org]

  She initially was seen 2 months prior for a wound on the left knee due to a fall as well as cellulitis, which was treated with intravenous vancomycin and ceftriaxone. [mdedge.com]

  This condition falls in the spectrum of the neutrophilic dermatoses, inflammatory disorders that have in common a tendency for pathergy (induction of the inflammatory process after skin trauma) and the presence of noninfectious neutrophilic infiltrates [doi.org]

• Collapse

  Lichen planopilaris is characterized by immune privilege collapse of the hair follicle’s epithelial stem cell niche. J Pathol (2013) 231(2):236–47. doi:10.1002/path.4233 PubMed Abstract | CrossRef Full Text | Google Scholar 22. Wenzel J, Tuting T. [frontiersin.org]

• Nasal Ulcer

  Pyoderma gangrenosum should be included in the differential diagnosis when nasal ulceration, abscesses, and sinusitis do not improve with antibiotics and drainage. [ncbi.nlm.nih.gov]

• Arthralgia

  CONCLUSION: PG patients with IBD had increased occurrence rates of arthralgia or arthritis, anemia, and ANCA-positive status compared to idiopathic PG patients. [ncbi.nlm.nih.gov]

  Arthralgias are common. In this subtype, a single, indolent, mildly painful plaque or superficial ulcer develops, most often on the head or neck. The border is not undermined and the base is not necrotic. [msdmanuals.com]

  […] in the dermis with secondary necrosis of the epidermis the first sign is a pustule with surrounding erythema with time an ulcer develops the walls of the ulcer are blue and well defined there may be systemic symptoms and signs such as: fever myalgia arthralgia [gpnotebook.co.uk]

  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. [doi.org]

  Arthralgias are common. Vegetative (superficial granulomatous pyoderma) subtype: In this subtype, a single, indolent, mildly painful plaque or superficial ulcer develops, most often on the head or neck. [merckmanuals.com]

• Fracture

  Patient 3 had a lower extremity tibial fracture to which a cast was applied. When the cast was removed, what was thought to be an abscess was debrided. [woundsresearch.com]

  Pathological fractures are described in the literature [ 8 ]. Concurrent appearance of CRMO and pyoderma gangrenosum is mentioned sporadically in the literature so far [ 2, 16, 25 ]. [bmcoralhealth.biomedcentral.com]

• Ulcer

  The skin then breaks down resulting in an ulcer. The ulcer can deepen and widen rapidly. Characteristically, the edge of the ulcer is purple and undermined. Pyoderma gangrenosum is usually very painful. [dermnetnz.org]

  The ulcers can coalesce to form larger ulcers, often with cribriform or sieve-like scarring. Symptoms and signs can vary with the subtype. [msdmanuals.com]

  Pyoderma gangrenosum (PG) is an uncommon, recurrent and chronic skin disease characterized by its distinct chronic ulcer. Initial lesions may resemble small bug bites or papules that eventually develop deep ulceration and chronic wounds. [aocd.org]

  Most ulcers are less than 3 cm in size; however, the ulcer can be multiple and an enormous ulcer of 30 cm has been reported [ 8, 39, 47 ]. [gastro.oxfordjournals.org]

• Skin Disease

  Pyoderma Gangrenosum Source: Patient Pyoderma gangrenosum (PG) is a rare and serious skin disease. [evidence.nhs.uk]

  On DermNet NZ Ulcers and erosions Neutrophilic dermatoses Sweet syndrome Neutrophilic dermatosis of hands Synthetic wound dressings Dermatological emergencies online course Skin manifestations of haematological disease Plasmapheresis for skin disease [dermnetnz.org]

  Pyoderma gangrenosum (PG) is a rare inflammatory and ulcerative skin disease of unknown etiology characterized by neutrophilic infiltration of the dermis, mainly affecting the lower extremities. [ncbi.nlm.nih.gov]

  Pyoderma gangrenosum is a rare, destructive inflammatory skin disease of which a painful nodule or pustule breaks down to form a progressively enlarging ulcer. [autoimmune.org]

• Skin Ulcer

  Definition A skin ulcer is an open sore in the skin. Pyoderma gangrenosum is an uncommon form of skin ulcers. It usually occurs on the lower legs, but can occur anywhere on the skin. Side View of Skin Ulcer Copyright © Nucleus Medical Media, Inc. [winchesterhospital.org]

  Discussion Pyoderma gangrenosum is a rare dermatosis characterized by rapidly progressive skin ulceration. [jmedicalcasereports.com]

  Patients who have skin ulcers that are painful, rapidly growing, and hard to heal should see a doctor. The diagnosis of PG is made based on history and skin examination. [jamanetwork.com]

  A skin ulcer is an open sore in the skin. Pyoderma gangrenosum is an uncommon form of skin ulcers. It usually occurs on the lower legs, but can occur anywhere on the skin. Side View of Skin Ulcer Copyright © Nucleus Medical Media, Inc. [uvahealth.com]

  Skin ulcers, especially of the lower extremities, encompass a myriad of causes that a clinician must analyze. Case Re- port. [ncbi.nlm.nih.gov]

• Papule

  Initial lesions may resemble small bug bites or papules that eventually develop deep ulceration and chronic wounds. [aocd.org]

  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. [doi.org]

  Subsequently, these papules, both biopsied and unbiopsied papules, rapidly progressed into ulcers consistent with prior episodes of PG. Immunohistochemical examination of the papules revealed a dense perivascular CD3+ infiltrate. [frontiersin.org]

  Pyoderma gangrenosum (PG) is a rare, neutrophil-predominant dermatosis that usually presents as a papule or pustule and progresses into a painful ulcer. [ncbi.nlm.nih.gov]

• Dermatitis

  Code Applicable To Dermatitis gangrenosa Purulent dermatitis Septic dermatitis Suppurative dermatitis Type 1 Excludes pyoderma gangrenosum ( L88 ) pyoderma vegetans ( L08.81 ) L08.0 ) The following code(s) above L88 contain annotation back-references [icd10data.com]

  Abstract Dermatitis artefacta may represent a real challenge for the clinician. As the patient does not admit self-inflicting the lesions, misdiagnosis with other diseases, such as pyoderma gangrenosum, is common. [ncbi.nlm.nih.gov]

• Aggressive Behavior

  Considering the extension of the lesion and the aggressive behavior of the disease, azathioprine was introduced early on, 20 days after the start of the corticosteroid therapy, with the goal of weaning from the corticosteroids in a medium time frame. [scielo.br]

• Irritability

  Parastomal PG – occurs close to abdominal stomas, usually in patients with irritable bowel disease, ileostomies, or colostomies that have been placed for malignancies; may present with bridges of epithelium that traverse the ulcer base; do not respond [accessmedicinenetwork.com]

  These ulcers may begin as small-irritated bumps from an injury. However, the ulcer can grow up to 7.9 in (inches) (20 cm [centimeters]). The ulcers often have purple edges that appear worn away. [winchesterhospital.org]

  Half of patients have a systemic inflammatory condition such as ulcerative colitis, Crohn’s disease, irritable- bowel syndrome, lupus, or rheumatoid arthritis. About a quarter of pyoderma cases begin after some sort of trauma or surgery. [woundeducators.com]

Work up includes a detailed history and physical examination followed by tests.

Laboratory Tests

• Complete blood count
• Blood profile
• Liver function tests
• Urinalysis 
• Kidney function tests
• Tissue culture of affected area
• VDRL Test to exclude STDs
• Antiphospholipid antibody test to exclude Wegener's and vasculitis
• Serum immunofixation electrophoresis to rule out gammopathy

Imaging Studies

Imaging studies are not usually done, however, a chest and abdominal X-ray and/or CT scan, bone marrow biopsy, colonoscopy and other such tests may be performed to check for underlying systemic diseases, if any.

Test Results

The diagnosis of pyoderma gangrenosum does not have any definite diagnostic factors or tests. So the diagnosis is made based on exclusion of all other diseases.

• Neutrophilia

  Here, markers for inflammation such as CRP, leukocytosis, and neutrophilia were elevated in 50-73% of the PG patients. © 2017 S. Karger AG, Basel. [ncbi.nlm.nih.gov]

  […] border Other causes of cutaneous ulceration have been excluded Minor criteria History suggestive of pathergy or clinical finding of cribriform scarring Systemic diseases associated with pyoderma gangrenosum Histopathological findings (sterile dermal neutrophilia [dermnetnz.org]

  A clinical-histological approach is required in order to make the diagnosis and to exclude other ulcerative processes involving dermal neutrophilia [4]. [worldwidewounds.com]

  The laboratory investigation of the peripheral blood revealed normocytic anemia, lymphocytopenia and neutrophilia. C-reactive protein was increased with 21.08 mg/l (normal value < 5 mg/l) and antinuclear antibodies were negative. [karger.com]

• Hyperglobulinemia

  Monoclonal or polyclonal hyperglobulinemia may also be associated with PG. [3] However, the immunological abnormalities associated with PG are not always consistently observed in all patients and it is unclear whether or not they are an epiphenomena. [idoj.in]

Medications

Antibiotics are generally not helpful for treating uncomplicated cases of pyoderma gangrenosum [8]. The drugs that may be given for treatment of pyoderma gangrenosum are:

• Immunomodulators like Infliximab, Thalidomide, Clofazimine, etc.
• Immunoglobulins
• Immunosuppressants like Cyclosporine, Azathioprine, Chlorambucil, etc
• Corticosteriods
• Topical therapies like Cromolyn sodium, Aminosalicylic acid, etc.

The best documented treatments for PG are systemic corticosteroids and Cyclosporin A [9].

Surgical Treatment

Surgery should be avoided, if possible, because of the pathergic phenomenon that may occur with surgical manipulation or grafting, resulting in wound enlargement [10].

Some reports suggest that about half of pyoderma gangrenosum patients have recurrences [5]. However, it should be noted that unless there are complications, the prognosis of this condition is generally very good.

Once the appropriate treatment is begun, the most effective being a combination of immunosuppressant and corticosteroid, the ulcer heals with minimal scarring. Follow up is generally required but not extensive, providing the ulcer isn't very deep and the condition is uncomplicated.

Complications

Ulcerations of pyoderma gangrenosum occur after trauma or injury to the skin in about 30% of patients; this process is termed pathergy [6].

Common complications include:

• Deeply penetrating ulcer
• Numerous ulcers
• Ulcer becoming infected
• Ulcer damaging the vasculature leading to haemorrhage 
• Scarring
• Death (extremely rare)

Pyoderma gangrenosum is an idiopathic condition which may or may not occur due to an underlying systemic disease. It is however proven that the disease is brought upon by an abnormal immune response, more precisely, excessive activity of neutrophils, that results in inflammation of an area of the skin, pustule or nodule formation and the end stage is usually an enlarged ulcer.

Other than inflammatory bowel disease (IBD), arthritis, systemic lupus erythematosus (SLE) and gammopathies, diseases like myeloid dyscrasias, Wegener granulomatosis, chronic hepatitis, PAPA syndrome, etc may also lead to this condition.

Incidence

Each year in the United States, pyoderma gangrenosum occurs in about 1 person per 100,000 people [3].

Age

It mostly affects people in their 40s or 50s [3].

Sex

Pyoderma gangrenosum is more common in females.

This disease occurs due to overexpression of Interleukin 8 (IL-8) in the area of the skin lesion. IL-8 is a potent chemotactic factor of neutrophils. As a result, abnormally large numbers of neutrophils aggregate in the affected area causing the lesion to worsen and ultimately, an ulcer is formed. Depending upon how long the abnormal inflammatory process goes on, the ulcer may get enlarged or start healing on its own.

Pyoderma gangrenosum is of two main types:

• Typical Pyoderma gangrenosum which occurs in the skin of the lower limbs.
• Atypical pyoderma gangrenosum which may occur anywhere else on the body, most commonly affected region being the upper limbs (superficial skin of the hands).

Other less common types include:

• Pustular Pyoderma gangrenosum [4]
• Vegetative Pyoderma gangrenosum
• Peristomal Pyoderma gangrenosum
• Bullous Pyoderma gangrenosum

Pyoderma gangrenosum is a disease occurring due to abnormal immune response. So it does not have an exact prevention. However, by treating any systemic disease appropriately may decrease the risk of developing this condition. Also, by treating any skin lesion promptly may prevent its progression to this disease.

Pyoderma gangrenosum (PG) is an uncommon, ulcerative, cutaneous condition with distinctive clinical characteristics first described in 1930 [1]. It is a disease in which a painful pustule may form, increase in size and break down, forming an ulcer.

Pyoderma gangrenosum is an idiopathic condition. Lesions may occur either in the absence of any apparent underlying disorder or in association with other disease, such as ulcerative colitis, Crohn's disease, polyarthritis, gammopathy and other conditions [2].

This condition is due to an abnormal immune process in response to any trauma affecting the skin. It may begin as a small, red painful swelling, nodule or pustule and lead to ulcer formation and scarring. If you develop such a condition and if you already suffer from a systemic disease like any mentioned above, you should contact your physician immediately.

1. Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:1922-26.
2. Freedberg IM, Eisen AZ, Wolff k. Fitzpatrick Dermatology in General medicine, 6th ed. New York: McGraw Hill;2003
3.  Jackson, J Mark, Callen, Jeffrey P (Apr 23,2012) "Pyoderma Gangrenosum" In Elston. Dirk M. Emedicine
4. Shankar S, Sterling JC, Rytina E. Pustular pyoderma gangrenosum. Clinical and Experimental dermatology 2003 28(6); 600-3 
5. Von Den Driesch P: pyoderma gangrenosum: a report of 44 cases with follow up. Br J Dermatol. 1997,Dec;137(6);1000-5 
6. August PJ, Wells GC. Pyoderma gangrenosum treated with azathioprine and prednisolone. 
7. Brown TS, Marshall GS, Callen JP. Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum:a rarely recognised extracutaneous manifestation of a neutrophilic dermatosis. J Am Acad Dematol. Jul 2000 43(1 pt 1):108-2
8. Lyon CC, et al. Parastomal pyoderma gangrenosum: clinical features and management. J Am Acad Dermatol. 2000;42:992-1002
9. Armstrong PM, et al. Pyoderma gangrenosum. A diagnosis not to be missed. J Bone Joint Surg Br. 1999;81:893-94.
10. Baranska-Rybak W, Kakol M, Naesstrom M, Komorowska O, Sokolowska-Wojdylo M, Roszkiewicz J. A retrospective study of 12 cases of pyoderma gangrenosum: why we should avoid surgical intervention and what therapy to apply. Am surg. Dec 2011, 277(12):1644-9