Oral Vitamin A as an Adjuvant Treatment for Refractory Pityriasis Rubra Pilaris (PRP) Hanif Sri Utami 1* , Benny Nelson 1 , Eyleny Meisyah Fitri 1 , Windy Keumala Budianti 1 , Endi Novianto 1 1 Department of Dermatology and Venereology Faculty of Medicine Universitas Indonesia/ Dr. CiptoMangunkusumoNational CentralGeneral Hospital, Indonesia Keywords: Pityriasis Rubra Pilaris, Retinol, Therapy, Vitamin A Abstract: Pityriasis rubra pilaris (PRP) is a rare and chronic papulosquamous disorder of unknown etiology that often progresses to erythroderma and causes a disabling palmoplantar keratoderma. Genetic factors with an autosomal dominant pattern of inheritance have been supposed to play a critical role for the induction of PRP. Vitamin A deficiency was also believed to be related to the disorder. Treatment of PRP is mainly anecdotal, based on case reports and case series, a feature shared by many disorders in dermatology due to their rarity. Currently, oral retinoids and methotrexate are the first line of therapy in patients with PRP. Response to therapy varies in each patient. We report a 46-year-old male patient with erythroderma and hyperkeratotic palms and soles since two years ago. Histopathological findings were consistent with PRP. The patients had been treated with several systemic therapies, yet showed poor clinical response. Clinical improvement was seen after 16 weeks addition of oral vitamin A at a dosage of 200.000 IU daily in concurrent with 10 mg weekly methotrexate. Evaluation of potential adverse effects was closely monitored.Oral vitamin A, an old regimen, seems to be a favorable adjuvant treatment for refractory PRP. 1 INTRODUCTION Pityriasisrubrapilaris (PRP) is a rare and chronic skin disorder that often progresses to erythroderma and causes a disabling keratoderma of the palms and soles. The exact cause of this skin disorder remains elusive. The impact of this skin disorder is often devastating on patients’ quality of life. There are six different types of PRP based on clinical presentation, age of onset, and prognosis. The most common clinical features are follicular hyperkeratosis, progressing to salmon-colored erythroderma with islands of normal skin (nappesclaires). Palms and soles are frequently involved as waxy and thick hyperkeratotic plaque. These typical features occur in more than 50% of the patients of PRP and the disease resolution usually occurs after an average period of 3 years.(Moretta G et al, 2017;Sehgal VN et al., 2008). The management of PRP has always been challenging. The management is predominantly based on case reports with limited number of patients. To date, no randomized controlled trial had been conducted due to the scarcity of the disease. The classical therapies include systemic therapies such as retinoids, methotrexate, cyclosporine, and fumaric acid. Psoralen plus ultraviolet A (PUVA) photochemotherapy is also in use. However, response to therapy varies from person to person, and it is not uncommon that patients show poor response to multiple therapies (Ross NA et al., 2016). Oral vitamin A (retinol) at a dosage of 150,000 to 300,000 IU per day was reported beneficial in some patients. Retinoids, its synthetic derivatives, are considered to be the first line therapy. Isotretinoin, acitretine, and etrenitate have been reported helpful in some patients of PRP. They help regulate the proliferation and differentiation of the epithelial cells. Compared to retinol, the administration of these synthetic agents requires lower dose. 4 However, the prescription of these synthetic agents remains restricted in Indonesia, so that we try oral vitamin A as an adjunctive treatment while monitoring the side effects. 2 CASE A 46-year-old male patient visited the Dermatovenerology clinic, Cipto Mangunkusumo National Central General Hospital with erythroderma Utami, H., Nelson, B., Fitri, E., Budianti, W. and Novianto, E. Oral Vitamin A as an Adjuvant Treatment for Refractory Pityriasis Rubra Pilaris (PRP). DOI: 10.5220/0009987102950298 In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 295-298 ISBN: 978-989-758-469-5 Copyright c  2020 by SCITEPRESS – Science and Technology Publications, Lda. All rights reserved 295