Clinical and Experimental Dermatology | 2021
Recalcitrant psoriatic onycho‐pachydermo‐periostitis successfully treated with guselkumab
Abstract
Psoriatic onycho-pachydermo-periostitis (POPP) is an uncommon and very disabling variant of psoriasis, characterized by severe onychodystrophy and thickening of the connective tissue above the distal phalanx with radiological changes and dactylitis, providing the classic drumstick-like deformity of the digits. It is classified as a subset of psoriatic arthritis. Reported treatments such as sulfasalazine, nonsteroidal antiinflammatory drugs and methotrexate have showed variable and inconsistent results. An 80-year-old woman presented in December 2017 with an 18-month history of nail changes, painful swelling of the fingers and toes, and severe functional impairment (Dermatology Life Quality Index 22) and was diagnosed with POPP according to clinical (Fig. 1a,b) and radiological findings (erosions of the distal phalanx with osteophytes, subungual exostosis and prominent periarticular soft tissues of the distal phalanx of the left great toe and several fingers on both hands). She was otherwise healthy and had no personal history of other dermatological conditions, but had a history of Crohn disease (CD) during early adulthood in complete remission during the past 20 years without therapy. She also had a positive family history of psoriasis. The patient had been treated previously with standard doses of ciclosporin (5 months), acitretin (4 months) and methotrexate (9 months) with scant response. She was started on adalimumab (Humira ; AbbVie, Chicago, IL, USA) 40 mg subcutaneously (SC) alternately weekly after the induction phase. At Week 16 only a partial response was seen, with slight improvement of pustules, erythema and pain (Fig. 2e,f). Moreover, from Week 2 of adalimumab therapy the patient reported onset of very itchy lesions on her extremities (Fig. 2a–d), which were clinically and histologically compatible with atopic dermatitis (AD; topical emollients and steroids were introduced without success and the AD necessitated withdrawal of adalimumab at Week 20, with complete improvement of AD in 6 weeks. Ustekinumab (Stelara ; Janssen Biotech, Horsham, PA, USA) 90 mg SC (at Day 0, Week 4 and every 12 weeks thereafter) was then started. Only minimal improvement was seen after 16 weeks of ustekinumab therapy (Fig. 2g,h) and there was an increase in joint pain and C-reactive protein level; therefore, methotrexate (MTX) 10 mg/week SC was added. At the 7-month follow-up the response to ustekinumab plus MTX was not optimal and we decided to switch to guselkumab monotherapy (Tremfya ; Janssen Biotech) 100 mg SC (on Day 0, Week 4 and every 8 weeks thereafter) (Fig. 1c,d). At Week 12 of guselkumab therapy, an impressive improvement was seen in the absence of joint pain and the patient had a DLQI of 2. At the most recent follow-up (Week 28 of guselkumab therapy), almost complete resolution was achieved (Fig. 1e,f). Treatment of POPP is challenging. There have been previous reports of MTX therapy in POPP with some benefits, and during the past 15 years, a few cases have been published of favourable response with tumour necrosis factor (TNF)-a inhibitors. One case of ustekinumab therapy with improvement (after loss of efficacy with infliximab) was reported but withdrawal after 19 months was necessary due to new onset of bullous pemphigoid. In the current paper, we present the first case, to our knowledge, successfully treated with guselkumab with an extraordinarily rapid response. In our patient, partial response of POPP and development of AD forced adalimumab withdrawal. AD-like eruptions have been rarely described with anti-TNF-a therapy. Interferon (IFN)-c is the major cytokine Correspondence: Dr Alexandra Maria Giovanna Brunasso, Department of Dermatology, Galliera Hospital, Mura delle Cappuccine, 14 Genoa Liguria, Genoa 16100, Italy E-mail: [email protected]