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]
The disease was identified in 1908. 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
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
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]
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]
Histology: Exclusion of infection (including histologically indicated stains and tissue cultures)
Pathergy (ulcer occurring at sites of trauma, with the ulcer extending past the area of trauma)
Personal history of inflammatory bowel disease or inflammatory arthritis
History of papule, pustule, or vesicle that rapidly ulcerated
Clinical examination (or photographic evidence) of peripheral erythema, undermining border, and tenderness at the site of ulceration
Multiple ulcerations (at least 1 occurring on an anterior lower leg)
Cribriform or “wrinkled paper” scars at sites of healed ulcers
Decrease in ulcer size within 1 month of initiating immunosuppressive medications
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]
1 2 Partridge AC, Bai JW, Rosen CF, Walsh SR, Gulliver WP, Fleming P (August 2018). "Effectiveness of systemic treatments for pyoderma gangrenosum: a systematic review of observational studies and clinical trials". The British Journal of Dermatology. 179 (2): 290–295. doi:10.1111/bjd.16485. PMID29478243. S2CID3504429.
1 2 Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G (September 2009). "Pyoderma gangrenosum: an updated review". Journal of the European Academy of Dermatology and Venereology. 23 (9): 1008–17. doi:10.1111/j.1468-3083.2009.03199.x. PMID19470075. S2CID29773727.
↑ Langan SM, Powell FC (August 2005). "Vegetative pyoderma gangrenosum: a report of two new cases and a review of the literature". International Journal of Dermatology. 44 (8): 623–9. doi:10.1111/j.1365-4632.2005.02591.x. PMID16101860. S2CID34574262.
↑ Marzano AV, Damiani G, Ceccherini I, Berti E, Gattorno M, Cugno M (2017). "Autoinflammation in pyoderma gangrenosum and its syndromic form (pyoderma gangrenosum, acne and suppurative hidradenitis)". British Journal of Dermatology. 176 (6): 1588–1598. doi:10.1111/bjd.15226. PMID27943240.
↑ Rashid RM (November 2008). "Seat belt pyoderma gangrenosum: minor pressure as a causative factor". Journal of the European Academy of Dermatology and Venereology. 22 (10): 1273–4. doi:10.1111/j.1468-3083.2008.02626.x. PMID18837131. S2CID27476857.
↑ Reichrath J, Bens G, Bonowitz A, Tilgen W (August 2005). "Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients". Journal of the American Academy of Dermatology. 53 (2): 273–83. doi:10.1016/j.jaad.2004.10.006. PMID16021123.
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