Pyoderma gangrenosum-like ulcerations in granulomatosis with polyangiitis: two cases and literature review

Abstract

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing small vessel vasculitis associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Skin manifestations, mostly represented by palpable purpura, papulonodular lesions and livedo reticularis, are present in up to 50% of the cases. Ulcerations with undermined, raised erythematous-violaceous border resembling pyoderma gangrenosum (PG) have rarely been reported as skin involvement in GPA. The presence of circulating ANCAs with a cytoplasmic labelling pattern, the involvement of internal organs, particularly of the lung, and the absence on histology of a mainly neutrophilic infiltrate in early phases of the cutaneous lesions may be regarded as clues to rule out true PG and confirm the diagnosis of GPA skin ulcerations simulating PG. Herein, we describe two paradigmatic cases of such a unique presentation of GPA and a literature review focusing on clinicopathological features of GPA presenting with PG-like ulcerations in the skin has been provided. Moreover, referring to the scenario observed in these two cases, an easy-to-use working approach for the differential diagnosis between the two conditions has also been proposed.

Granulomatosis with polyangiitis preceded with pyoderma gangrenosum in 41-year-old man

“…The presence of circulating c�ANCA, in� volvement of internal organs �especially the lungs� and lack of neutrophilic infiltration in the biopsy of skin le� sions can help to rule out and confirms the diagnosis of GPA, which simulates PG. 5,7,9,10 �hen the clinical picture of these two entities is similar, there is a problem which is both diagnostic and therapeutic. 6 Difficulties in assessing the histopathological picture such as lack of clear diagnos� tic criteria for PG and recommendation to repeat biopsy in order to determine the proper diagnosis.…”

“…2,5,7 Therefore, epidemiological data regarding the actual co�e�istence of PG and PGA are controversial. 9,11 Genoveese et al 9 re� viewed 28 cases and established guidelines useful for dis� tinguishing these conditions: �1� typical raised, erythema� tous�wavy edges are less visible in GPA ulcers compared to PG; (2) histology of early PG lesion consists mainly of neutrophilic inflammatory infiltrate; (3) c-ANCA positiv� ity is compatible with GPA; (4) systemic involvement is common in GPA and rare in PG. 9,2 On the other hand, PG may, although rarely, occur sec� ondary or coe�ist with GPA, as in the case of our patient.…”

Penile pyoderma gangrenosum: Successful treatment with thalidomide

“…Genital PG is rare and often misdiagnosed as other disorders (Larsen & Thyssen, ). The differential diagnoses of penile PG include bacterial, viral, and fungal infections (Kikuchi et al, ), sexually transmitted diseases, erosive lichen planus, Behçet's disease (Genovese, Tavecchio, Berti, Rongioletti, & Marzano, ), malignant tumors (Gonzalez‐Sabin, Rodriguez‐Diaz, Gonzalvo‐Rodriguez, & Astola‐Hidalgo, ), trauma, and artifact (Satoh & Yamamoto, ).…”

Pyoderma gangrenosum ‒ cutaneous and extracutaneous manifestations

“…Furthermore increased levels of cytokines have been detected by the immunohistochemistry methods in the skin lesions. Over-expression of TNF-α, IL-17, IL-1β, IL-8 and increased synthesis of matrix metalloproteinases (MMPs) have been found in PG, which play a significant role in the development of the inflammatory processes and cause damage to the tissue (2,(5)(6)(7)(8).…”