Extracutaneous involvement of pyoderma gangrenosum

Abstract

Pyoderma Gangrenosum (PG) is an inflammatory neutrophilic dermatosis (ND) associated with underlying chronic inflammation and/or malignancy. Diagnosis remains to be challenging as a gold standard diagnostic test is lacking. Initial manifestations may include papules, vesicles, or pustules that subsequently develop into ulceration with features of undermining and violaceous borders. Timely recognition of pyoderma gangrenosum is impeded by clinical findings shared with other etiologies, such as granulomatosis with polyangiitis, polyarteritis nodosa, and antiphospholipid syndrome. As with any other ND, extracutaneous involvement may also occur preceding, during, or following the appearance of skin lesions. Sterile neutrophilic infiltrates have been found to affect internal organs supporting the concept of PG being a systemic disease, with lung being the most common extracutaneous manifestation followed by ocular and visceral compromise. Therefore, in this review, we describe the current knowledge of extracutaneous involvement of PG and its respective clinical manifestations to aid dermatologists in diagnosis, management, and determining prognosis.

Disseminated histoplasmosis presenting as pyoderma gangrenosum‐like ulcers in an apparently immunocompetent patient: multiple red herrings presenting a diagnostic challenge

“…Our case presented several red herrings: pyoderma gangrenosum‐like ulcers, and systemic findings (colon stricture, osteolytic‐sclerotic lesions) mimicking its known associations (inflammatory bowel disease, sterile neutrophilic osseous infiltrates) 1,2 . Pyoderma gangrenosum‐like ulcers are uncommon in disseminated histoplasmosis.…”

Pyoderma gangrenosum: a review with special emphasis on Latin America literature

“…), autoimmune inflammatory disease (seronegative polyarthritis, rheumatoid arthritis), and solid tumors (prostate and colon adenocarcinoma) 4, 8. Finally, sterile neutrophilic infiltrates have been found to affect internal organs supporting the concept of PG being a systemic disease 22 Figure 1Classic or ulcerative pyoderma gangrenosum.

Figure 2Classic or ulcerative pyoderma gangrenosum.

Figure 3Vegetative or verrucous pyoderma gangrenosum in a patient with Behcet's disease.

Figure 4Vegetative or verrucous pyoderma gangrenosum in a patient with Behcet's disease.

Figure 5Pyoderma gangrenosum after abdominoplasty.

Figure 6Multiple pyoderma gangrenosum ulcers at the sites of sclerotherapy injections.

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Pyoderma gangrenosum: From historical perspectives to emerging investigations

“… 9,21 By the 1940s, it was accepted that PG most often occurred in association with UC, but was also thought to occur with trauma, infectious arthritis, upper respiratory infection, pleuritis, pericarditis, cholecystitis, genitourinary infections, abscessed tooth, or (rarely) independently 20,21 . The first example of PG with extracutaneous involvement was reported in 1985 as steroid‐responsive pneumonitis, 22 and pulmonary involvement has been the most common extracutaneous manifestation to date 23 …”

“…It could be also be a skin manifestation of autoinflammatory syndromes, and rare familial forms have been reported 26‐28 . Interestingly, nonulcerative forms have too been described, and extracutaneous manifestations may also occur preceding, during, or following the appearance of skin lesions 23,24 . Differential diagnosis is broad, divided into six categories by Weenig et al: vasculopathy, vasculitis, cancer, infection, exogenous tissue injury, and other inflammatory disorders 3 .…”

“…20,21 The first example of PG with extracutaneous involvement was reported in 1985 as steroid-responsive pneumonitis, 22 and pulmonary involvement has been the most common extracutaneous manifestation to date. 23 Today, PG is considered an inflammatory disease which may present in association with autoimmune disorders and cancers. 24,25 It could be also be a skin manifestation of autoinflammatory syndromes, and rare familial forms have been reported.…”