Pyodermatitis–pyostomatitis vegetans after subtotal colectomy for ulcerative colitis

Abstract

A 51-year-old Japanese woman underwent subtotal colectomy for ulcerative colitis 5 years before her first visit to our hospital, and her symptoms stabilized. She presented with a 2-month history of proliferative, pustular lesions on her face, scalp, and gingival and anal mucosae. Histological examination showed features similar to those of pemphigus vegetans. However, the results of immunofluorescence studies, immunoblot analysis and enzyme-linked immunosorbent assay for pemphigus were negative, and colonoscopy revealed a relapse of ulcerative colitis in the remaining rectal mucosa, so a diagnosis of pyodermatitis-pyostomatitis vegetans was made. This is the first report of mucocutaneous lesions that developed with a relapse of ulcerative colitis in the remaining rectum after subtotal colectomy. Moreover, a close correlation was found between the condition of bowel inflammation and that of mucocutaneous lesions.

Skin Manifestations of Inflammatory Bowel Disease

“…In general, bowel disease antedate oral involvement by several years (Leibovitch et al, 2005). The rash of PPV has been reported to correlate with underlying bowel disease activity (Ayangco et al, 2002; Kitayama et al, 2010). The pathogenesis of PSV, PDV, or PPV is not clear, but has been hypothesized to be due to aberrant immune responses in IBD to cross-reacting antigens in the skin and bowel resulting in mucocutaneous manifestations (Femiano et al, 2009).…”

“…A case report demonstrated successful PSV remission with topical fluocinonide gel, but the patient relapsed and ultimately required total colectomy to achieve and maintain remission (Calobrisi et al, 1995; Femiano et al, 2009). In fact, subtotal colectomy in a patient with UC resulted in the development of PPV and relapse of bowel disease 5 years after surgery (Kitayama et al, 2010). …”

A Case of Pyodermatitis-Pyostomatitis Vegetans

“…PD-PSV is closely associated with IBD, with the latter occurring in 70% of patients with PD-PSV. 12 Skin lesions, however, may precede gastrointestinal symptoms in approximately 15% of patients, indicating the need for regular and careful gastroenterological monitoring of these patients. 5 PD-PSV may therefore constitute a hypersensitivity reaction associated with IBD.…”

Pyoderma Vegetans Associated with Ulcerative Colitis: A Case with Good Response to Steroids

“…To differentiate PV from other diseases, evaluations of wound, yeast and mycobacterium tuberculosis culture and direct immunofluorescence study must be performed in order to rule out pemphigus vegetans. The association of PV with UC is well known but rarely reported in literature [2, 3, 6, 8, 9, 10]. Since PV is considered a marker of inflammatory bowel disease, a presumptive diagnosis of PV should induce a complete gastrointestinal investigation.…”

“…The treatment of PV focuses on control of the underlying disease. In previously reported cases, the skin lesions regressed after management of bowel disease with sulfasalazine, mesalazine or colectomy [3, 8]. Despite the bacterial etiology of the lesions in PV, systemic antibiotics alone are no effective treatment [7, 11].…”