|LETTER TO EDITOR
|Year : 2022 | Volume
| Issue : 2 | Page : 98-100
Novel use of tofacitinib in the recalcitrant adult generalized pustular psoriasis
K Shreya1, Dinesh P Asati1, Arpita N Rout2, Amulya M Lakshman1
1 Department of Dermatology and Venereology, AIIMS, Bhopal, Madhya Pradesh, India
2 AIIMS, Bhuvaneshwar, Odisha, India
|Date of Submission||18-Sep-2022|
|Date of Acceptance||29-Nov-2022|
|Date of Web Publication||5-Jan-2023|
Department of Dermatology and Venereology, AIIMS, Habibganj, Saketnagar, Bhopal, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Shreya K, Asati DP, Rout AN, Lakshman AM. Novel use of tofacitinib in the recalcitrant adult generalized pustular psoriasis. Indian J Drugs Dermatol 2022;8:98-100
|How to cite this URL:|
Shreya K, Asati DP, Rout AN, Lakshman AM. Novel use of tofacitinib in the recalcitrant adult generalized pustular psoriasis. Indian J Drugs Dermatol [serial online] 2022 [cited 2023 Mar 24];8:98-100. Available from: https://www.ijdd.in/text.asp?2022/8/2/98/367119
A 48-year-old male patient presented to emergency with generalized pustules over erythematous background for 2 months with a chronic history of erythematous scaly plaques over the chest and lower extremities for 8 months [[Figure 1]A, B]. He was previously treated for eczema with oral and injectable steroids by local practitioners; when he was tapered steroids, he suddenly had eruptions of multiple pustules initially starting over the chest and later progressing over the face and extremities. The patient was started on acitretin 25 mg once daily by a nearby dermatologist for 1 month; due to an inadequate response, he was later shifted to cyclosporine 100 mg with no response even after 2–3 weeks.
Then he sought consultation in our hospital where a biopsy shows Munro’s and Kogoj micro abscess with psoriasiform hyperplasia with underlying dilated capillaries with lymphocytic infiltration, and he was started on a once-weekly dose of injectable methotrexate 15 mg and a daily dose of hydrocortisone 50 mg with a decrease in the pustulation. Later, steroid was tapered accordingly over the next 2–3 weeks. Although the frequency of pustulation was reduced, episodes continue to persist, so an injection Etanercept 50 mg twice weekly was introduced. Later, the patient developed recurrent fever and cough which was inconsistent with the pustular episodes. We thought initially that the fever and pustulation were a paradoxical phenomenon for injection etanercept so the medication was withheld. A routine fever profile with a coronavirus disease–2019 (COVID-19) checkup showed positivity for COVID-19 (omicron) and hemolytic anemia. His immunosuppressants were stopped for 1–2 weeks until the resolution of fever and cough. Anemia showed improvement after the stoppage of methotrexate and daily iron and folic acid supplements. He was treated with antipyretics and antitussives for COVID-19. After recovery from COVID-19, the patient was started on tofacitinib where the frequency of pustulation was reduced from four episodes per week to once weekly. Later after 2 weeks, complete resolution of pustular eruptions leaving few scaly erythematous plaques [[Figure 2]A, B] with clinical improvement in general condition was also recorded. The patient was discharged on a full dose of tofacitinib and followed for 2 months with no exacerbation of episode (summarized in [Figure 3]).
Pustular psoriasis is a form of psoriasis that manifests as pustulation on an erythematous background. Pustular psoriasis is a long-term disease with remitting and relapsing course. Triggering factors include smoking, infection, sudden stoppage of medications such as systemic steroids, cyclosporine, methotrexate, and pregnancy. The therapeutic paradox is seen in tumor necrosis factor (TNF)-alfa inhibitors triggering immune-mediated skin diseases. CARD 14, AP1S3, and IL 36 RN are implicated in the pathogenesis.
The disease clinically presents as chronic sterile pustules associated with fever, erythema, pain, burning sensation, and scaling. Types of pustular psoriasis are generalized and localized. The severity of symptoms varies with disease flare. It is associated with life-threatening complications such as sepsis, cholangitis, cholestasis, interstitial pneumonitis, acute renal failure, and cardiorespiratory failure. Histopathology is associated with Kogoj’s subcorneal spongiform pustules, Munro’s micro abscess, psoriasiform hyperplasia, papillary dermal capillary dilation, perivascular neutrophils, and lymphomononuclear infiltrate. The use of tofacitinib in psoriasis and psoriatic arthritis was reported. To date, no case of the use of tofacitinib in pustular psoriasis has been reported. Treatment includes phototherapy, systemic immunosuppressants such as methotrexate, cyclosporine, and biologics such as etanercept, adalimumab, and secukinumab. A comparison of the various therapeutic options is summarized in [Table 1].,,
|Table 1: Therapeutic options with the level of evidence for adult generalized pustular psoriasis|
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Tofacitinib is the first Janus kinase inhibitor approved for psoriatic arthritis. It is potentially a JAK 1, 3 inhibitor and hence reduces IL2, 4, 6, 7, 9, 15, 21, thereby modulating immune and inflammatory pathways. It is rapidly absorbed and reaches peak plasma concentration after 0.5–1 h. Steady-state concentration is achieved in 24–48 h with clearance via 70% hepatic metabolism and 30% renal clearance.
In our case, most of the treatments were tried for controlling acute flares of pustular psoriasis, with inadequate response to the enlisted drugs. A smaller molecule, that is, tofacitinib, at a dosage of 5 mg twice daily was added. After the introduction, significant improvement in the frequency of pustulation was noted after a month with complete recovery in erythema noted after 3 months without any side effects.
In a nutshell, therapeutic options for pustular psoriasis depend on the severity of presentation and underlying risk factors. Still, limited data on the treatment of pustular psoriasis, and further exploration is needed. Tofacitinib is beneficial in psoriatic arthritis, and psoriasis. Its beneficial role in pustular psoriasis needs further attention.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Hoegler KM, John AM, Handler MZ, Schwartz RA Generalized pustular psoriasis: A review and update on treatment. J Eur Acad Dermatol Venereol 2018;32:1645-51.
Sugiura K The genetic background of generalized pustular psoriasis: IL36RN mutations and CARD14 gain-of-function variants. J Dermatol Sci 2014;74:187-92.
Paik J, Deeks ED Tofacitinib: A review in psoriatic arthritis. Drugs 2019;79:655-63.
Burns T, Breathnach SM, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. Hoboken, NJ: John Wiley & Sons; 2008.
Robinson A, Van Voorhees AS, Hsu S, Korman NJ, Lebwohl MG, Bebo BF Jr, et al
. Treatment of pustular psoriasis: From the medical board of the national psoriasis foundation. J Am Acad Dermatol 2012;67:279-88.
Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B Pustular psoriasis: Pathophysiology and current treatment perspectives. Psoriasis (Auckl) 2016;6:131-44.
[Figure 1], [Figure 2], [Figure 3]