Two cases of severe hidradenitis suppurativa with failure of anakinra therapy

Dear Editor, Hidradenitis suppurativa (HS) is a chronic inflammatory and debilitating skin disease with a poor response to traditional treatments. Of the new therapeutic options available, successful off‐label use of anakinra has been reported in patients with severe HS, and in a patient with pyoderma gangrenosum, acne and suppurative hidradenitis (PASH) syndrome.1 2 3 4 We describe our experience with two patients with severe HS, both of whom were treated with subcutaneous administration of anakinra 100 mg daily. Physical examination and laboratory analyses were undertaken on both patients every 4 weeks. The main aim was to evaluate their response after 12 weeks of treatment. The patients completed the Dermatology Life Quality Index (DLQI)5 questionnaire and underwent Physician's Global Assessment (PGA), which gives a score ranging from 0 (clear) to 5 (very severe), at both the beginning and the end of the treatment. Owing to the extent and severity of the lesions, the Hidradenitis Suppurativa Score, simplified by Sartorius et al.,6 was considered inadequate to evaluate the treatment response. The first patient was a 32‐year‐old man with PASH syndrome of 15 years, with no response to multiple antibiotics, methotrexate, sulfone, ciclosporin, finasteride, surgery and biological therapies (infliximab, adalimumab, ustekinumab). He was an ex‐smoker with a body mass index (BMI) of 25 and a family history of HS. At the start of treatment, the patient had a DLQI score of 17 and a PGA score of 4. In the past year he had been treated with oral corticosteroids (40 mg daily), trimethoprim/sulfamethoxazole (800/160 mg daily) and amoxicillin/clavulanic acid (2 g three times daily). Considering the limited control of his lesions he was also treated with anakinra. Analysis of the cytokine profile in a blood sample did not show high levels of interleukin‐1β (3 pg mL−1; normal range 0–5 pg mL−1). After 12 weeks there were no changes in DLQI or PGA scores and therefore treatment with anakinra was stopped (Fig. 1a–d). (a) Nape of the neck of the first patient before anakinra treatment [Physician's Global Assessment (PGA) score of 4]. (b) Without change after 12 weeks of treatment with anakinra treatment (PGA score of 4). (c) Abscess on the right side of the trunk in patient 1 before treatment with anakinra. (d) The abscess was unchanged after 12 weeks of treatment with anakinra. The second patient was a 30‐year‐old man with acne, Crohn disease and severe HS of 5 years, localized mainly in the groin area, with resistance to systemic drugs (multiple antibiotics, isotretinoin, infliximab) and partial results with surgery. The patient had a DLQI score of 15 and PGA score of 4. He was a nonsmoker with a BMI of 19.9 and no family history of HS. The patient was being treated with isotretinoin (30 mg daily), clindamycin (300 mg twice daily) and levofloxacin (500 mg twice daily) when he started receiving anakinra. After 2 months both facial and inguinal abscesses worsened (Fig. 2a,b), with a resulting PGA score of 5 and a DLQI score of 17. Treatment with anakinra was stopped. Significant improvement was seen after a 10‐day course of oral corticosteroids. Groin of the second patient. (a) Before treatment with anakinra treatment [Physician's Global Assessment (PGA) score of 4]. (b) New abscesses after 8 weeks of treatment with anakinra (PGA score of 5). Excellent results with anakinra have been achieved in many cases of HS,2 3 4 including in an open‐label pilot study with six patients.1 Only one recent article has reported its failure (in a patient with a stage III Hurley disease).7 In conclusion, we have presented two cases of severe HS without response to, and, indeed, worsening with, anakinra treatment. In our opinion, further clinical trials are necessary to evaluate the efficacy profile of anakinra in patients with HS. Funding sources: none. Conflicts of interest: none declared.