Favorable response to composite treatment including infliximab in a case of granulomatous cheilitis with Crohn's disease

Abstract

chest and groin was also sutured. Eight months later, skin ulcers appeared on the surgical scar of the forehead, nasolabial groove and chest, but not on the groin. Physical examination revealed a large, deep ulceration with elevated edematous borders (Fig. 1c,d). The ulcer was reddish and granular on the surface and surrounded by erythema. Bacterial cultures from superficial pus showed Pseudomonas aeruginosa, for which oral antibiotics resulted in no effect. Laboratory examination showed an increased number of white blood cell counts (10 000/lL with 89% neutrophils) and elevated C-reactive protein (10.9 mg/dL). A skin specimen biopsied from the edematous erythema on the chest showed dense, diffuse infiltration of neutrophils in the dermis and subcutaneous tissue (Fig. 1e). The patient soon died of congestive heart failure.Pyoderma gangrenosum usually occurs between the ages of 20 and 50 years. 1 Our case was an elderly patient with developing PG unassociated with systemic disorders. There are several reports of PG occurring at percutaneous surgical sites, namely, breast surgery, pacemaker implantation, splenectomy, hysterectomy, endoscopic tube insertion, cholecystectomy and cesarean delivery, 1 which suggest that pathergy reaction is implicated as a triggering role in susceptible patients. Pathergy can be seen in approximately 20% of cases, 1 and several speculations, namely, an aberrant immune response to minor trauma, defective cell-mediated immunity, aberrant integrin oscillations on neutrophils and abnormal neutrophil tracking, have been reported. 1-3 In our case, PG was induced at the skin and cartilage recipient sites (face), as well as sites of removed cartilage (chest). In contrast, the skin of the groin sutured after taking full-thickness skin did not result in PG. It is unlikely that PG was induced by the rejection of autologous skin grafts, because PG was also induced on the chest, where a skin graft was not performed. Therefore, it is suggested that the induction of PG is related to the depth of the skin damage. Cases of PG after autologous breast reconstruction have been reported, 4 which support the speculation that invasive operation can induce PG. Our case developed PG 8 months after the operation. Peristomal PG occurs more than 20 years after enterostomy/ colostomy. 5 Thus, PG may occur even after long periods following surgical operation.

Recurrent granulomatous cheilitis associated with Crohn’s disease successfully treated with ustekinumab: case report and literature review

“… 9 There were 12 additional published clinical cases (eight with CD) reporting successful treatment of cheilitis granulomatosa with infliximab, especially in childhood. 10 , 13 – 22 Kakimoto et al reported a patient with cheilitis without CD in which infliximab induced a good initial response but triggered an acute infusion reaction; remission was achieved after switching to adalimumab. 24 Gaya et al reported a case of isolated cheilitis successfully treated with infliximab.…”

“…In Table 1 we summarize the characteristics of patients with granulomatous cheilitis, with or without coexistent CD, treated with biologics. 4,9,10,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] There was a clear predominance of cheilitis in children and young adults (mean age 26 years, SD 15), and 55% of the patients were female. Dupuy et al have reported a greater male predominance and a young age of onset in CD patients who present with oral manifestations.…”