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Pyoderma Gangrenosum


Presentation

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
  • 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
  • K42.1 , 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[icd10data.com]
  • We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness.[ncbi.nlm.nih.gov]
  • From pyoderma scientific Latin gangrenosum, neuter singular of gangrenosus gangrenous. Pronunciation pyoderma gangrenosum /ˌpʌɪə(ʊ)ˈdəːmə ˌɡaŋɡrɪˈnəʊzəm[en.oxforddictionaries.com]
  • Factitial Dermatitis (32) Hidradenitis Suppurativa (10) Leishmaniasis (13) Livedoid Vasculitis (13) Necrobiosis Lipoidica (17) Panarteritis Nodosa Kussmaul-Maier (19) Postthrombotic Leg Ulcer (3) Primary Tuberculous Complex (1) Progressive Post-operative Gangrene[dermis.net]
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]
  • 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]
  • The patient may also have fever, malaise, or myalgia. Pyoderma lesions are erythematous due to inflammation, but may also have a blue hue from epidermal necrosis. Treatment Immunosuppressive and anti-infective agents are commonly prescribed.[woundeducators.com]
  • Joint pains (arthralgias) and body malaise (body weakness) may be accompanying symptoms of this condition.[woundcarecenters.org]
Rigor
  • However, the effectiveness of these newer agents remains to be rigorously evaluated. Multi-drug regimens have not been well described in the literature but are an excellent alternative for patients with refractory disease.[ncbi.nlm.nih.gov]
Intermittent Fever
  • A 70-year-old man presented with a 2-month history of intermittent fever and multifocal painful papules, plaques, and ulcerations on his extremities. The histological and culture results of the lesions were consistent with those of PG.[ncbi.nlm.nih.gov]
Saddle Nose
  • Further, there is a high likelihood of nasal complications, including saddle nose deformity, septal perforation, and skin defects.[ncbi.nlm.nih.gov]
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]
Regurgitation
  • Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis.[ncbi.nlm.nih.gov]
Ear Deformity
  • We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness.[ncbi.nlm.nih.gov]
Cauliflower Ear
  • We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness.[ncbi.nlm.nih.gov]
Corneal Opacity
  • Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis.[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]
  • […] 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]
  • Joint pains (arthralgias) and body malaise (body weakness) may be accompanying symptoms of this condition.[woundcarecenters.org]
Ulcer
  • 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[symptoma.com]
  • History of papule, pustule, or vesicle that rapidly ulcerated Clinical examination (or photographic evidence) of peripheral erythema, undermining border, and tenderness at site of ulceration Multiple ulcerations (at least 1 occurring on an anterior lower[en.wikipedia.org]
  • We also report that topical collagenase worsened PG ulcers, consistent with pathergy. Becaplermin can be used to help treat ulcers resulting from calciphylaxis and PG.[ncbi.nlm.nih.gov]
  • Self-induced ulceration (Munchausen's syndrome and factitial ulceration) was the most common diagnosis in this category.[doi.org]
Skin Ulcer
  • 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]
  • The present case highlights the importance of tumor screening in patients with atypical antibiotic-refractory skin ulcers.[ncbi.nlm.nih.gov]
Papule
  • 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]
  • 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.[en.wikipedia.org]
  • Delphi exercise yielded 1 major criterion-biopsy of ulcer edge demonstrating neutrophilic infiltrate-and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule[ncbi.nlm.nih.gov]
Eruptions
  • A 45-year-old hypothyroid woman presented with a 6-year history of recurrent widespread eruptions of rashes and ulcers on her skin. She was diagnosed to have pyoderma gangrenosum and leukocytoclastic vasculitis.[ncbi.nlm.nih.gov]
  • One feature is a phenomenon known as pathergy, in which trauma to the skin can cause new eruptions of ulcers in patients with the disease. Types Two variants of the disease exist--the classic/typical and the atypical variants.[woundcarecenters.org]
Facial Skin Lesion
  • We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment.[ncbi.nlm.nih.gov]
Neglect
  • Medical staff should not neglect peristoaml dermatitis because of its common occurrence. Once the situation develops beyond the doctors' expectation, more efforts should be made to treat it, even expand debridement if possible.[ncbi.nlm.nih.gov]

Workup

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.

Treatment

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:

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].

Prognosis

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)

Etiology

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.

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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

Prevention

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.

Summary

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].

Patient Information

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.

References

Article

  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 

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Last updated: 2019-07-11 21:08