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Pyoderma gangrenosum
Classification and external resources

Pyoderma gangrenosum on the leg of a patient with Crohn's disease.
ICD-10 L88.
ICD-9 686.01
MeSH D017511

Pyoderma gangrenosum is a disease that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs. When they occur, they can lead to chronic wounds. Ulcers usually initially look like small bug bites or papules, and they progress to larger ulcers. Though the wounds rarely lead to death, they can cause pain and scarring.

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]

Contents

Types

Pyoderma gangrenosum

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

  • the 'typical' ulcerative form, which occurs in the legs
  • an 'atypical' form that is more superficial and occurs in the hands and other parts of the body

Other variations are[2]:

  • Peristomal pyoderma gangrenosum is 15% of all cases of pyoderma
  • Bullous pyoderma gangrenosum
  • Pustular pyoderma gangrenosum[3]
  • Vegetative pyoderma gangrenosum[4]

Causes

Though the etiology is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all pyoderma gangrenosum patients also suffer from illnesses that affect their systemic function.[1] For instance, ulcerative colitis, Crohn's disease, rheumatoid arthritis, and Multiple Myeloma (MM) sufferers have the condition. It can also be part of a syndrome, for instance in PAPA syndrome. Major and minor trauma are also believed to play a role. [5]

Associations

The common conditions associated with pyoderma gangrenosum are:

  • Inflammatory bowel disease:
    • Ulcerative colitis
    • Crohn's disease
  • Arthritides:
    • Rheumatoid arthritis
    • Seronegative symmetrical polyarthritis
  • Haematological disease:
    • Myelocytic leukaemia
    • Hairy cell leukaemia
    • Myelofibrosis
    • Myeloid metaplasia
    • Monoclonal gammopathy (IgA)[6]

Treatment

First-line therapy for disseminated or localized instances of pyoderma gangrenosum is systemic treatment by corticosteroids and cyclosporine. If ineffective, alternative therapeutic procedures include systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; thalidomide; infliximab; or plasmapheresis.[7]

References

  1. ^ a b c Jackson JM and Callen JP. 2006. Emedicine: Pyoderma Gangrenosum. Retrieved on January 23, 2007.
  2. ^ Brooklyn T, Dunnill G, Probert C (2006). "Diagnosis and treatment of pyoderma gangrenosum". BMJ 333 (7560): 181–4. doi:10.1136/bmj.333.7560.181. PMID 16858047.  
  3. ^ Shankar S, Sterling JC, Rytina E (2003). "Pustular pyoderma gangrenosum". Clin. Exp. Dermatol. 28 (6): 600–3. doi:10.1046/j.1365-2230.2003.01418.x. PMID 14616824.  
  4. ^ Langan SM, Powell FC (2005). "Vegetative pyoderma gangrenosum: a report of two new cases and a review of the literature". Int. J. Dermatol. 44 (8): 623–9. doi:10.1111/j.1365-4632.2005.02591.x. PMID 16101860.  
  5. ^ Seat belt pyoderma gangrenosum: minor pressure as a causative factor. Rashid RM. J Eur Acad Dermatol Venereol. 2008 Nov;22(10):1273-4.
  6. ^ Maintenance on GPnotebook site
  7. ^ Reichrath J, Bens G, Bonowitz A, Tilgen W (2005). "Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients". J. Am. Acad. Dermatol. 53 (2): 273–83. doi:10.1016/j.jaad.2004.10.006. PMID 16021123.  

8. Geller, S; Longton, J. (2005) Ulceration of Pyoderma Gangrenosum Treated With Negative Pressure Wound Therapy. JAPMA 95(2): 171-174.

External links

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