Adult onset pityriasis rubra pilaris

Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, Delhi, India. .
Indian journal of dermatology, venereology and leprology (Impact Factor: 1.39). 07/2008; 74(4):311-21. DOI: 10.4103/0378-6323.42889
Source: PubMed


Pityriasis rubra pilaris (PRP) has always been an intriguing topic ever since its inception. It is a group of chronic disorders characterized by reddish orange plaques with pityriasiform scaling showing follicular keratoses, palmoplantar keratoderma, and sometimes, erythroderma. It occurs all over the world but with racial variations. Its incidence might vary and the age at onset, behavior, clinical appearance, and prognosis are considered to be very important for its classification. It may manifest either as Type I classical adult onset PRP, Type II atypical adult (onset) PRP, or Type VI PRP (HIV-associated PRP pityriasis rubra pilaris) in contrast to classical juvenile (Type III) and circumscribed juvenile (Type IV) encountered among children. Its diagnosis is largely clinical with microscopic pathology being a useful supplement, but it continues to be a therapeutic dilemma. We review the epidemiology of adult onset PRP here and take stock of the prevalent treatment options.

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Available from: Virendra N Sehgal, Aug 26, 2014
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    • "Spontaneous resolution occurs in 80% of patients within 1-3 years [1, 5]. It is characterized by follicular hyperkeratotic papules that coalesce into large, scaly, erythematous plaques, palmoplantar keratoderma, diffuse scaling of the scalp sometimes progressing into erythroderma [1, 6-8]. The affected skin is extremely rough to touch [3]. "
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    ABSTRACT: Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. It has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo. Herein we report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris. A 38 year old woman with classic adult type 1 pityriasis rubra pilaris (PRP) developed progressive skin thickening of the trunk, face, upper and lower extremities after 2 years of PRP treatment with topical emollients and steroids. Clinical examination and immunological findings were consistent with SSc. Co-existence of these two rare conditions is documented for the first time.
    Full-text · Article · Aug 2010 · Pan African Medical Journal
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    • "Se ha observado un caso de PRP en unos gemelos monocigotos [11] . En general, los casos familiares de PRP se desarrollan durante la infancia, mientras que los casos adquiridos lo hacen durante la quinta o sexta década de la vida [12] . "
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    ABSTRACT: La pitiriasis rubra pilaris es una dermatosis heterogénea rara en donde se asocian tres elementos semiológicos en grados diversos: una pápula córnea folicular, una queratodermia palmoplantar anaranjada y lesiones eritematoescamosas que pueden ser muy extensas con intervalos de piel sana. El origen de esta afección es confuso y en la mayoría de los casos existen factores desencadenantes traumáticos o infecciosos, que probablemente actúan en un contexto de predisposición. En otros casos se observa una asociación a desórdenes inmunológicos o bien, en casos familiares, alteraciones genéticas de la queratinización cercanos a la ictiosis. Ante esta gran variabilidad semiológica se han propuesto varias clasificaciones basadas en criterios clínicos y evolutivos. La evolución varía en función de las formas clínicas. El enfoque terapéutico no está bien codificado y no existen ensayos terapéuticos disponibles, debido a la rareza de la enfermedad. Los mejores resultados parecen obtenerse con retinoides orales, y en segunda línea se hallan el metotrexato y la ciclosporina. Los nuevos inhibidores del factor de necrosis tumoral α podrían presentar gran interés en el futuro.
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    ABSTRACT: Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis of unknown etiology, and finding a successful therapy can be challenging. PRP occurs equally in men and women. In some patients, associated autoimmune diseases, infections, or malignancies are possible trigger factors. PRP shows a bimodal age distribution, peaking in the first as well as in the fifth to sixth decade. Its classification into five subgroups is based on age at onset, clinical course, morphologic features, and prognosis. More than 50% of patients are best classified as type I with adult-onset PRP. This form is also characterized by high spontaneous remission rates (80%) within 1–3 years. Clinically, the classical adult (type I) and classical juvenile (type III) forms appear to be the same except for the patient’s age. Recently, the designation of a new category of PRP (type VI) has been proposed that is characterized by the presence of HIV infection with different clinical features and a poorer prognosis. Typical morphologic features of PRP are erythematosquamous salmon-colored plaques with well demarcated islands of unaffected skin. Often, keratoderma of the palms and soles is present. In patients with extensive disease, ectropion is a dreaded complication. Histology shows hyperkeratosis, alternating orthokeratosis and parakeratosis in a checkerboard pattern, and focal acantholytic dyskeratosis. Descriptions and therapeutic experiences are mainly based on case reports. Mostly, systemic retinoids, methotrexate, and other immunosuppressive agents as well as UV light therapy are applied, with varying response rates. In recent years, treatment with so-called ‘biologics’ is becoming more and more popular for treating recalcitrant PRP. We present a review of the clinical features, histopathologic findings, classification, differential diagnoses, and treatment of PRP.
    No preview · Article · Feb 2010 · American Journal of Clinical Dermatology
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