(Juvenile) Pityriasis rubra pilaris

Sehgal, Virendra; Srivastava, Govind

doi:10.1111/j.1365-4632.2006.02666.x

Cited by 44 publications

(63 citation statements)

References 110 publications

(210 reference statements)

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“…PRP occurs in three distinct peaks, during the first decade, second decade, or in adulthood (40-60 years) [1,3]. Most commonly, PRP develops as an acquired disorder [1,3]; however, it has been estimated that, as in our case, 6.5% of PRP cases are due to familial inheritance [1]. The inheritance pattern is generally autosomal dominant with variable expressivity, though an autosomal recessive pattern has also been reported [3].…”

Section: Discussionmentioning

confidence: 53%

“…Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous disorder of unclear etiology [1,2]. PRP occurs in three distinct peaks, during the first decade, second decade, or in adulthood (40-60 years) [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…PRP has been reported in association with various malignancies, immunologic abnormalities infections, and trauma [1][2][3][4]. When associated with such inciting events, PRP may develop because of an abnormal immune response elicited against an antigenic trigger [3,4].…”

Section: Discussionmentioning

confidence: 99%

“…When associated with such inciting events, PRP may develop because of an abnormal immune response elicited against an antigenic trigger [3,4]. Because of the efficacy of retinoids in treating PRP, vitamin A and retinol binding protein deficiencies have been postulated to play a role in PRP [1,4]. These proposed mechanisms have yet to be validated.…”

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

A Novel Case of Recalcitrant Juvenile Pityriasis Rubra Pilaris with Response to Bexarotene

Word¹,

Patel²,

M³

2011

J Clin Exp Dermatol

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Pityriasis rubra pilaris (PRP) is generally treated with single or combination regimens of topical agents, systemic retinoids, methotrexate, biologics, various other immunosuppressant agents, and light therapy; however, cases of PRP that are not amenable to these therapies can be challenging to control. We present the case of a male with biopsy-proven juvenile PRP followed for ten years, with failure to respond to all traditional treatment regimens. Significant improvement in the patient's cutaneous and rheumatological symptoms occurred when the patient was treated with bexarotene (Targretin ® ). To our knowledge, this represents the first reported successful treatment of PRP with bexarotene. Thus, treatment with bexarotene may represent a beneficial option for cases of PRP recalcitrant to traditional treatment modalities.

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Section: Discussionmentioning

confidence: 53%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

A Novel Case of Recalcitrant Juvenile Pityriasis Rubra Pilaris with Response to Bexarotene

Word¹,

Patel²,

M³

2011

J Clin Exp Dermatol

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“…Although PRP may occur at any age (10), it most commonly aff ects those in their fi rst, second, fi fth or sixth decades of life (1). Usually these are sporadic acquired forms, familial forms are rare, being rather transplacentally transmitted (1), than inherited in an autosomal dominant or autosomal recessive or X-linked fashion (3,12).…”

mentioning

confidence: 99%

Pityriasis Rubra Pilaris: A Report of Two Cases and Literature Review

Paravina

Ljubenović

Binić

et al. 2015

Serbian Journal of Dermatology and Venereology

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Pityriasis rubra pilaris (PRP) is an idiopathic inflammatory hyperproliferative chronic dermatosis characterized by: perifollicular coalescing papules with central keratotic acuminate plugs gradually submerged in sheets of erythema; perifollicular erythema with islands of unaffected skin; palmoplantar keratoderma; diffuse desquamation which typically spreads from the head down to the feet. The cause of the condition is unknown, but possible etiological factors include: vitamin A deficiency, trauma, infections, autoimmune mechanisms, and malignancies. Taking into account different age of onset, clinical course, morphology and prognosis, there are six different types of the disease: two in adults (classical and atypical); three in children (classical, circumscribed and atypical); one in individuals infected with human immunodeficiency virus. This paper presents two male patients with clinical symptoms of classical PRP, 53 and 69 years of age at the onset of the disease, with rapid generalized involvement, typical erythematous perifollicular papules, islands of unaffected skin, palmoplantar hyperkeratosis with a waxy appearance and nail changes. The diagnosis was based on clinical findings and histopathologic analysis. Apart from topical therapy with emollients, corticosteroids and keratolytics, they received systemic retinoids and corticosteroids, which resulted in improvement of skin lesions. It is extremely important to consider the possible triggering factors, establish the diagnosis as soon as possible and begin proper treatment.

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Pityriasis Rubra Pilaris

Samuelov

Sprecher

2019

Harper's Textbook of Pediatric Dermatology

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(Juvenile) Pityriasis rubra pilaris

Cited by 44 publications

References 110 publications

A Novel Case of Recalcitrant Juvenile Pityriasis Rubra Pilaris with Response to Bexarotene

A Novel Case of Recalcitrant Juvenile Pityriasis Rubra Pilaris with Response to Bexarotene

Pityriasis Rubra Pilaris: A Report of Two Cases and Literature Review

Pityriasis Rubra Pilaris

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