{"id":384988,"date":"2024-10-26T00:01:00","date_gmt":"2024-10-25T22:01:00","guid":{"rendered":"https:\/\/medizinonline.com\/?p=384988"},"modified":"2024-09-02T10:21:39","modified_gmt":"2024-09-02T08:21:39","slug":"successful-combination-treatment-for-pityriasis-rubra-pilaris","status":"publish","type":"post","link":"https:\/\/medizinonline.com\/en\/successful-combination-treatment-for-pityriasis-rubra-pilaris\/","title":{"rendered":"Successful combination treatment for pityriasis rubra pilaris"},"content":{"rendered":"\n

Pityriasis rubra pilaris is a rare papulosquamous disease characterized by great variability.\nThe clinical spectrum ranges from localized infestation of the extremities to erythrodermic forms.\nTreatment often proves difficult.\nCurrently, oral retinoids are considered first-line systemic therapy.\nBiologics are also increasingly being used for refractory courses – in this case as an add-on to acitretin. <\/strong><\/p>\n\n\n\n

DeBiasio et al.\ndescribe the case of an otherwise healthy 58-year-old woman who developed an extensive erythematous scaly rash that began on the face and scalp and rapidly spread to the trunk and limbs [1].\nThe patient had no history of eczema or psoriasis.\nThe histological findings of a biopsy revealed that it was a classic PRP.\nThe treatment proved to be relatively lengthy, but ultimately the combined use of acitretin and ustekinumab proved to be effective. <\/p>\n\n

Initially, half of the body surface was affected <\/h3>\n\n

The patient’s rash (Fitzpatrick skin type II) was strikingly symmetrical, consisting of bright red scaly plaques on the face, trunk and limbs with follicular papules in the dorsal area of the hand and around the edges of the spreading plaques [1].\nThe BSA (body surface area) <\/em>was 50%.\nThe scalp showed slight, diffuse erythema and scaling.\nAn orange-red, waxy palmoplantar keratoderma and follicular papules manifested in the area of the knuckles.\nIndividual islands were absent on the trunk.\nPsoriasis and progressive symmetrical erythrokeratoderma were considered as differential diagnoses to classic PRP.\nA drug reaction was considered unlikely; at that time, the only medication taken regularly was citalopram 20 mg\/d.\nThe histologic findings of a punch biopsy confirmed the diagnosis of PRP: there was alternating parakeratosis and orthokeratosis at vertical and horizontal levels, irregular psoriasiform hyperplasia, thickened papillary dermal plates, a preserved granular layer, and a perivascular lymphocytic infiltrate in the superficial dermis. <\/p>\n\n

Pityriasis rubra pilaris (PRP) usually occurs between the ages of 40 and 60 [4].\nThe course and clinical symptoms vary greatly from individual to individual.\nRegardless of the subtype of PRP (I-VI), 90% of patients develop flat, erythematous plaques during the course of the disease, in a fifth of cases with an emphasis on the elbows and knees [5].\nScaling of the skin occurs in around 90% of cases.\nPalmoplantar keratoderma and diffuse alopecia are seen in around three quarters of PRP patients and a large proportion have nail involvement (pachyonychia, dyschromasia or onycholysis).\nAround 80% are affected by itching and around half of PRP patients suffer from burning of the skin [5]. <\/td><\/tr><\/tbody><\/table><\/figure>\n\n

Multi-track empirical treatment: patience paid off <\/h3>\n\n

The patient initially received acitretin 10 mg\/d twice daily plus betamethasone valerate ointment 0.1% [1].\nOne month later, the rash had spread further and the erythroderma increased.\nIn addition, there was nocturnal itching combined with feelings of tension on the skin and chills.\nThe acitretin dose was then increased to 20 mg\/d and combined with ustekinumab (45 mg, s.c.) as an add-on.\nThe usual psoriasis regimen was used: after the loading dose, ustekinumab was initially administered at four-week intervals and then every 12 weeks.\nAfter the second dose of ustekinumab, there was a gradual improvement and three months later the skin symptoms had almost disappeared.\nThe palmoplantar keratoderma regressed completely.\nOnly the scalp continued to show erythema, scaling, itching and some alopecia.\nThe dose of ustekinumab was then increased to 90 mg, which reduced the symptoms on the scalp.\nThe only side effect was some sticky palms due to the acitretin, which was subsequently reduced to 10 mg\/d.\nThe patient was advised to maintain this regimen of low-dose acitretin and ustekinumab 90 mg (every 12 weeks).\nThe laboratory values (complete blood count, liver function, lipids) remained within the normal range.\nThe plan is to slowly phase out the therapy over 1-2 years. <\/p>\n

\n
\"\"<\/a><\/figure>\n<\/div>\n

Discussion <\/h3>\n\n

There are still many unanswered questions about the aetiopathogenesis of PRP.\nThe therapeutic procedure is largely based on case series, recommendations and individual experience.\nIn adults with PRP who do not respond adequately to topical preparations (emollients, urea, topical steroids), systemic treatment with retinoids such as oral acitretin is considered a first-line therapy [2].\nHowever, treatment-refractory courses are not uncommon [1].\nRelatively high doses of acitretin are required for an adequate response and undesirable side effects often limit patient compliance.\nMore recently, it has emerged that PRP shares some molecular, histologic and clinical features with psoriasis [3].\nIn this context, some of the biologics approved for psoriasis have been used in off-label use with PRP, often resulting in good efficacy and tolerability, as in the example of the present case report [1\u20133]. <\/p>\n\n

Literature:<\/p>\n\n

    \n
  1. DeBiasio C, Cyr J, Ayroud Y, Glassman SJ: A case of classic adult pityriasis rubra pilaris successfully treated with a combination of acitretin and ustekinumab: A case report.\nSAGE Open Med Case Rep, 2022 Apr 19; 10: 2050313X221093453. <\/li>\n\n\n\n
  2. Roenneberg S, Biedermann T: Pityriasis rubra pilaris: algorithms for diagnosis and treatment.\nJEADV 2018; 32(6): 889-898. <\/li>\n\n\n\n
  3. Brown F, Badri T: Pityriasis Rubra Pilaris.\n2020 Jun 29. StatPearls [Internet].\nStatPearls Publishing; 2020. <\/li>\n\n\n\n
  4. “Pityriasis rubra”, https:\/\/flexikon.doccheck.com\/de\/Pityriasis_rubra,<\/a>(last accessed 13.08.2024).<\/li>\n\n\n\n
  5. Kahlert K, et al: Pityriasis rubra pilaris – a rare inflammatory dermatosis with many facets. Act Dermatol 2019; 45: 32-39.<\/li>\n<\/ol>\n\n

    DERMATOLOGY PRACTICE 2024; 34(4): 28<\/em><\/p>\n\n

    <\/p>\n\n

    <\/p>\n\n

    Cover picture: \u00a9Kelly McGauran, wikimedia<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"

    Pityriasis rubra pilaris is a rare papulosquamous disease characterized by great variability. The clinical spectrum ranges from localized infestation of the extremities to erythrodermic forms. Treatment often proves difficult. Currently,…<\/p>\n","protected":false},"author":7,"featured_media":384992,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"pmpro_default_level":"","cat_1_feature_home_top":false,"cat_2_editor_pick":false,"csco_eyebrow_text":"Study report S. aureus infection in pediatric AD","footnotes":""},"category":[11520,11340,11508,11548,11503],"tags":[54990,12391,78056,78052,44127],"powerkit_post_featured":[],"class_list":["post-384988","post","type-post","status-publish","format-standard","has-post-thumbnail","category-cases-en","category-dermatology-and-venereology","category-education","category-rx-en","category-studies","tag-acitretin-en","tag-biologics-en","tag-combination-treatment","tag-oral-retinoids","tag-pityriasis-rubra-pilaris-en","pmpro-has-access"],"acf":[],"publishpress_future_action":{"enabled":false,"date":"2026-04-28 03:37:03","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category","extraData":[]},"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"wpml_current_locale":"en_US","wpml_translations":{"fr_FR":{"locale":"fr_FR","id":384990,"slug":"traitement-combine-reussi-pour-le-pityriasis-rubra-pilaire","post_title":"Traitement combin\u00e9 r\u00e9ussi pour le pityriasis rubra pilaire","href":"https:\/\/medizinonline.com\/fr\/traitement-combine-reussi-pour-le-pityriasis-rubra-pilaire\/"},"it_IT":{"locale":"it_IT","id":384991,"slug":"trattamento-combinato-di-successo-per-la-pitiriasi-rubra-pilaris","post_title":"Trattamento combinato di successo per la pitiriasi rubra pilaris","href":"https:\/\/medizinonline.com\/it\/trattamento-combinato-di-successo-per-la-pitiriasi-rubra-pilaris\/"},"pt_PT":{"locale":"pt_PT","id":384989,"slug":"tratamento-combinado-bem-sucedido-para-a-pitiriase-rubra-pilar","post_title":"Tratamento combinado bem sucedido para a pitir\u00edase rubra pilar","href":"https:\/\/medizinonline.com\/pt-pt\/tratamento-combinado-bem-sucedido-para-a-pitiriase-rubra-pilar\/"},"es_ES":{"locale":"es_ES","id":385136,"slug":"tratamiento-combinado-con-exito-para-la-pitiriasis-rubra-pilaris","post_title":"Tratamiento combinado con \u00e9xito para la pitiriasis rubra pilaris","href":"https:\/\/medizinonline.com\/es\/tratamiento-combinado-con-exito-para-la-pitiriasis-rubra-pilaris\/"}},"_links":{"self":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts\/384988","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/comments?post=384988"}],"version-history":[{"count":1,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts\/384988\/revisions"}],"predecessor-version":[{"id":384996,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts\/384988\/revisions\/384996"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/media\/384992"}],"wp:attachment":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/media?parent=384988"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/category?post=384988"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/tags?post=384988"},{"taxonomy":"powerkit_post_featured","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/powerkit_post_featured?post=384988"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}