Treatment of pityriasis rubra pilaris with ustekinumab

Wohlrab, Johannes; Kreft, Burkhard

doi:10.1111/j.1365-2133.2010.09855.x

Cited by 58 publications

(52 citation statements)

References 4 publications

(5 reference statements)

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“…The patient experienced a significant improvement after the first injection at week 4. In view of the response in the case described here and the previous successful reports 12,13 ustekinumab could be included as an alternative agent for treating adult-onset PRP. However, further studies are necessary to more precisely evaluate its safety and efficacy.…”

Section: Resultsmentioning

confidence: 99%

Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris

Stefani

Galluzzo²,

Talamonti³

et al. 2013

J Dermatol Case Rep

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Background: Pityriasis rubra pilaris is a rare, chronic erythematous squamous disorder of unknown etiology. The disease is characterized initially by small follicular papules that coalesce into yellowish pink scaly plaques, palmoplantar keratoderma, diffuse furfuraceous scale of the scalp, and frequent progression to exfoliative erythroderma. Generally it is difficult to discern pityriasis rubra pilaris from other skin conditions but key-clinical features help in the diagnosis such as "islands" of spared skin within generalized erythroderma, follicular keratotic plugs, and an orange hue of the involved skin. Treatment options include topical vitamin D analogues, keratolytics, systemic acitretin, methotrexate, cyclosporine, azathioprine, fumaric acid esters, phototherapy, and anti-TNFα agents. Cases, of pityriasis rubra pilaris, successfully treated with a short-course ustekinumab therapy, have been reported. Main observations:We report a 31-year-old man with pityriasis rubra pilaris, refractory to conventional treatments, successfully treated with ustekinumab monotherapy for over 64 weeks. After failing conventional systemic agents (cyclosporine, aciretin and methotrexate), ustekinumab 45 mg has been prescribed, with the same dosing regimen as in psoriasis. The patient improved dramatically within 4 weeks of the first injection, with markedly less erythema and pruritus. Long-term control of the disease of the disease was achieved (64 weeks of treatment). Conclusion:We report this case in order to show the striking and rapid efficacy of ustekinumab in reducing the signs and symptoms of the disease. Complete remission was achieved after the third injection, but also a long-term control of the disease. The therapy was well-tolerated in our patient and no adverse events occurred.

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Section: Resultsmentioning

confidence: 99%

Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris

Stefani

Galluzzo²,

Talamonti³

et al. 2013

J Dermatol Case Rep

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“…In vitro, alitretinoin showed higher affinity to RAR than to RXR and inhibited production of nitric oxide and proinflammatory cytokines such as tumour necrosis factor (TNF)-α, interleukin (IL)-1β and IL-12p40 (11,12). Accordingly, efficacy of TNF-α inhibitors and the blocking IL-12/IL-23 anti-p40 monoclonal antibody ustekinumab was also shown in PRP case reports (13)(14)(15). Hence, beneficial therapeutic effects of alitretinoin in PRP might possibly be due to suppression of TNF-α and IL-12/IL-23 cytokines.…”

Section: Discussionmentioning

confidence: 81%

Alitretinoin (9-cis Retinoic Acid) is Effective Against Pityriasis Rubra Pilaris: A Retrospective Clinical Study

Amann¹,

Susic²,

Glüder³

et al. 2015

Acta Derm Venerol

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Pityriasis rubra pilaris (PRP) is an uncommon cutaneous disease with disorder of keratinisation. Up to now, systemic retinoids like acitretin or isotretinoin seem to be the most effective therapeutic agents. However, no large trials on this rare disease have been published and no standardised treatment has been established so far. Recently, single case reports demonstrate beneficial effects of alitretinoin (9-cis retinoic acid) in patients with PRP. We performed a retrospective observational analysis of type I adult-onset patients with PRP (n = 5) treated with systemic alitretinoin in our department. Alitretinoin was highly effective in the treatment of PRP in 4 of 5 cases. PASI score was reduced significantly in the alitretinoin responders. We assume that alitretinoin could serve as an additional effective systemic treatment option for type I adult-onset PRP.

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“…The role of ustekinumab in other diseases, including psoriatic arthritis, Crohn's disease and multiple sclerosis, has been investigated [15]. It has also been utilized off license in a number of other conditions with variable success, including pityriasis rubra pilaris, pyoderma gangrenosum and palmo plantar pustulosis [16][17][18]. This latter disease, previously considered a variant of psoriasis, would appear to be an entity distinct from psoriasis [19].…”

Section: Laws and Warrenmentioning

confidence: 96%

Ustekinumab for the treatment of psoriasis

Laws

Warren

2011

Expert Review of Clinical Immunology

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Management of psoriasis over the last decade has changed significantly with the introduction of biological therapies. Ustekinumab is a first-in-class biological agent, inhibiting the action of IL-12 and IL-23, and has provided further evidence for the role of Th1 and Th17 lymphocytes in the pathogenesis of psoriasis. Efficacy has been clearly demonstrated in three Phase III clinical trials. Week 12 Psoriasis Area and Severity Index (PASI) 75 was observed in 66.4-75.7% of patients with PASI 90 achieved in 36.7-50.9%. This marked clinical response is also reflected in a significant improvement in quality of life. The most recent Phase III clinical trial has demonstrated the superior efficacy of ustekinumab (regardless of dosing regimen) compared with high-dose etanercept at week 12. Long-term efficacy has been demonstrated over 148 weeks with 64-76% of patients maintaining PASI 75. The role of ustekinumab in the treatment of psoriatic arthritis has shown some benefit in Phase II clinical trials. Phase III clinical trials are pending and will provide further guidance on management of concurrent disease. The currently available safety data are on the whole reassuring, although ongoing vigilance remains central to the detection of rare or late sequelae.

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Treatment of pityriasis rubra pilaris with ustekinumab

Cited by 58 publications

References 4 publications

Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris

Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris

Alitretinoin (9-cis Retinoic Acid) is Effective Against Pityriasis Rubra Pilaris: A Retrospective Clinical Study

Ustekinumab for the treatment of psoriasis

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