Pyoderma gangrenosum: Difference between revisions
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== Background == |
== Background == |
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=== Subtypes === |
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* Ulcerative: ulceration with rapidly-evolving purulent wound |
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* Pustular: discrete pustules, sometimes self-limited, and most commonly associated with [[inflammatory bowel disease]] |
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* Bullous: superficial bullae with ulcerations |
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* Vegetative: erosions with superficial ulcers |
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=== Associated Disorders === |
=== Associated Disorders === |
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* [[Inflammatory bowel disease]] (40%) |
* [[Inflammatory bowel disease]] (40% of cases with a known association), [[ulcerative colitis]] more than [[Crohn disease]] |
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* [[Inflammatory arthritis]] (20%) |
* [[Inflammatory arthritis]] (20%), particularly seronegative [[rheumatoid arthritis]] or [[spondylitis]] |
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* Solid organ malignancy (5-10%) |
* Solid organ malignancy (5-10%) |
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* Hematologic malignancy (5-10%) |
* Hematologic malignancy (5-10%) |
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* Other hematologic disorder (5%), including [[MGUS]], [[myelodysplastic syndrome]], or [[polycythemia vera]] |
* Other hematologic disorder (5%), including [[MGUS]]/[[myeloma]], [[myelodysplastic syndrome]], or [[polycythemia vera]], [[lymphoma]], [[leukemia]] |
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* Rarely, [[hepatitis C]] |
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* Idiopathic in about 50% of cases |
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== Clinical Manifestations == |
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* Chronic wound, most commonly on lower legs in the pretibial area |
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* Can occur anywhere on the body, particularly at surgical sites |
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* Rarely, can involve upper airway, eye, genital mucosa, lung (sterile neutrophilic pulmonary infiltrates), splenic infiltrates, myositis, and sterile osteolysis |
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== Diagnosis == |
== Diagnosis == |
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=== Diagnostic Criteria[[CiteRef::maverakis2018di]] === |
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=== Diagnostic Criteria<ref>Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. JAMA Dermatol. 2018 Apr 1;154(4):461-466. doi: [https://doi.org/10.1001/jamadermatol.2017.5980 10.1001/jamadermatol.2017.5980]. PMID: [https://pubmed.ncbi.nlm.nih.gov/29450466/ 29450466].</ref> === |
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* Major criterion: |
* Major criterion: |
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* Interpretation: |
* Interpretation: |
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** Positive if at the major criterion and at least four minor criteria are present |
** Positive if at the major criterion and at least four minor criteria are present |
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== Further Reading == |
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* Pyoderma gangrenosum – a review. ''Orphanet J Rare Dis'' 2, 19 (2007). doi: [https://doi.org/10.1186/1750-1172-2-19 10.1186/1750-1172-2-19] |
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Latest revision as of 17:20, 27 September 2024
Background
Subtypes
- Ulcerative: ulceration with rapidly-evolving purulent wound
- Pustular: discrete pustules, sometimes self-limited, and most commonly associated with inflammatory bowel disease
- Bullous: superficial bullae with ulcerations
- Vegetative: erosions with superficial ulcers
Associated Disorders
- Inflammatory bowel disease (40% of cases with a known association), ulcerative colitis more than Crohn disease
- Inflammatory arthritis (20%), particularly seronegative rheumatoid arthritis or spondylitis
- Solid organ malignancy (5-10%)
- Hematologic malignancy (5-10%)
- Other hematologic disorder (5%), including MGUS/myeloma, myelodysplastic syndrome, or polycythemia vera, lymphoma, leukemia
- Rarely, hepatitis C
- Idiopathic in about 50% of cases
Clinical Manifestations
- Chronic wound, most commonly on lower legs in the pretibial area
- Can occur anywhere on the body, particularly at surgical sites
- Rarely, can involve upper airway, eye, genital mucosa, lung (sterile neutrophilic pulmonary infiltrates), splenic infiltrates, myositis, and sterile osteolysis
Diagnosis
Diagnostic Criteria1
- Major criterion:
- Biopsy of ulcer edge demonstrating a neutrophilic infiltrate
- Minor criteria:
- Exclusion of infection
- Pathergy
- Personal history of inflammatory bowel disease or inflammatory arthritis
- History of papule, pustule, or vesicle that rapidly ulcerated
- Peripheral erythema, undermining border, and tenderness at site of ulceration
- Multiple ulcerations (at least one occurring on an anterior lower leg)
- Cribriform or "wrinkled paper" scar(s) at sites of healed ulcers
- Decrease in ulcer size within one month of initiating immunosuppressive medications
- Interpretation:
- Positive if at the major criterion and at least four minor criteria are present
Further Reading
- Pyoderma gangrenosum – a review. Orphanet J Rare Dis 2, 19 (2007). doi: 10.1186/1750-1172-2-19
References
- ^ Emanual Maverakis, Chelsea Ma, Kanade Shinkai, David Fiorentino, Jeffrey P. Callen, Uwe Wollina, Angelo Valerio Marzano, Daniel Wallach, Kyoungmi Kim, Courtney Schadt, Anthony Ormerod, Maxwell A. Fung, Andrea Steel, Forum Patel, Rosie Qin, Fiona Craig, Hywel C. Williams, Frank Powell, Alexander Merleev, Michelle Y. Cheng. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum. JAMA Dermatology. 2018;154(4):461. doi:10.1001/jamadermatol.2017.5980.
