Superficial Granulomatous Pyoderma Gangrenosum Involving the Face: A Case Series of Five Patients and a Review of the Literature

Abstract

Background Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often difficult to diagnose. There are few cases reported in the literature of PG involving the face. Aim To share our experience with 5 patients in whom the final diagnosis was PG involving the face, and to review the literature. Methods We report a series of 5 patients with a final diagnosis of PG involving the face and reviewed relevant literature. We searched through PubMed and EMBASE using keywords such as “face” and “pyoderma gangrenosum,” “blastomycosis-like pyoderma gangrenosum, vegetative pyoderma gangrenosum and granulomatous pyoderma gangrenosum.” Results We report 5 patients (4 females) with pyoderma gangrenosum involving the face. All 5 had a final diagnosis of superficial granulomatous PG. All cases presented with nonhealing facial ulcer most commonly on cheeks and a common histopathology of mixed inflammatory infiltrates, multinucleated giant cells, and plasma cells with some granulomatous inflammation. Conclusions PG can involve the face, and all 5 of our patients had the superficial granulomatous as the most common form.

Chronic superficial ulcers on the trunk

“…SGP on the face appears to be refractory to topical and systemic corticosteroids. 2 Anogenital region – Incorrect. The anogenital region is rarely described in SGP.…”

“…This describes findings present in classic ulcerative PG, which lacks the layered suppurative granuloma typical of SGP. 1 , 2 , 3 , 4 Layered suppurative granuloma with necrosis and suppuration surrounded by histiocytes and plasma cells, along with overlying epidermal hyperplasia – Correct. While nonspecific, these findings are consistent in SGP.…”

“…While nonspecific, these findings are consistent in SGP. 1 , 2 , 3 , 4 Pseudoepitheliomatous hyperplasia with neutrophilic abscesses and scar-like fibrosis – Incorrect. Blastomycosis-like pyoderma demonstrates these features; isolation of a pathogenic organism such as Staphylococcus aureus is required for diagnosis.…”

“…This describes findings present in classic ulcerative PG, which lacks the layered suppurative granuloma typical of SGP. 1 , 2 , 3 , 4…”

Disseminated superficial granulomatous pyoderma

“…For diagnosis of SGP, clinical and histopathological features are the mainstay after excluding other causes such as infections, autoimmune/autoinflammatory diseases, granulomatous conditions, or cutaneous neoplasms. 1 , 2 , 3 , 4 , 5 Clinically, the predilection site of SGP is the trunk, but may also rarely affect the face. Unlike classic PG, SGP is usually not associated with underlying systemic conditions.…”