The Journal of Dermatology | 2021
Successful treatment of pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome with granulocyte and monocyte adsorption apheresis
Abstract
Dear Editor, A 34yearold Japanese woman was referred to our clinic with complaints of a 15year history of recurrent and refractory multiple pustules and cystic lesions. She had been treated with systemic and topical antibiotics and oral prednisolone and underwent frequent surgical incisions with only limited effects. On her first visit, multiple acnelike papules and pustules, recessed small scars, and superficial abscesses were observed on her chest (Figure 1a). An abscess was found in the left breast adjacent to the nipple. Acnelike lesions were also present on the upper abdomen. Numerous scars and multiple abscesses were observed on the patient’s back (Figure 1b). In addition, a palmsized circular scar due to repeated ulceration, 7 cm in diameter, was observed on her right lower leg (Figure 1c). Postincision scars were observed on the trunk and right posterior thigh. We considered that the scars on the right lower leg and abscess on her left breast resulted from pyoderma gangrenosum (PG) and suppurative hidradenitis (SH), respectively. Laboratory findings showed an increased white blood cell count (11 250/μL) with neutrophilia (8380/μL) and slightly elevated immunoglobulin (Ig)A at 431 (normal, 93– 393) mg/dL. Creactive protein, IgG, and IgM levels were within normal limits. No obvious joint symptoms were observed. Based on these findings, a diagnosis of PG, acne, and SH (PASH) syndrome was made. A mutation analysis was not performed. She underwent granulocyte and monocyte adsorption apheresis (GMA) twice a week for a total of five sessions. Her lesions responded to some extent; therefore, five additional sessions were scheduled at 7day intervals. After the final GMA therapy, her active pustules and abscess had improved, and her skin lesions had been well controlled for more than 4 years without diseasespecific treatment except for occasional systemic antibiotics for her acnelike eruptions associated with her menstrual cycle. Pyoderma gangrenosum, acne, and SH syndrome is a recently proposed disease entity clinically characterized by PG, acne, and SH,1 belonging to the spectrum of hereditary autoinflammatory syndrome with PG. Although biologic agents targeting interleukin1 and tumor necrosis factorα have been recommended for the treatment of PASH syndrome,2 their efficacy has not been well established because of its rarity. Granulocyte and monocyte adsorption apheresis is an extracorporeal apheresis unit designed to selectively remove pathogenic granulocytes and macrophages that produce proinflammatory cytokines.3 The efficacy of GMA on PG has been reported,4 and treatment of PASH syndrome with GMA has been described in a single case report.5 In this report, PASH syndrome improved with GMA and concomitant systemic steroids followed by continuous adalimumab to maintain remission. The PG lesion in our patient was inactive, while her multiple acne and SH lesions, which should share a common pathogenesis with PG, were still intense. Therefore, we determined that the disease activity of her PASH syndrome was still active. We treated her with GMA, and the symptoms improved with GMA alone. The remarkable outcome in the present case demonstrates the importance of myeloid lineage cells in the pathogenesis of PASH syndrome