Familial Pityriasis Rubra Pilaris

Vanderhooft, Sheryll L.; Francis, Julie S.; Holbrook, Karen A.; Dale, Beverly A.; Fleckman, Philip

doi:10.1001/archderm.1995.01690160076012

Cited by 54 publications

(24 citation statements)

References 31 publications

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“…However, familial forms with autosomal dominant or recessive inheritance have been reported. Acquired PRP usually develops in adulthood, whereas familial PRP usually presents during childhood (3)(4)(5). According to Griffiths' classification, familial PRP frequently belongs to type V (atypical juvenile) (6)(7)(8).…”

Section: Discussionmentioning

confidence: 99%

Genetic Analysis of CARD14 in Non-familial Pityriasis Rubra Pilaris: A Case Series

Hong

Chen²,

Chen³

et al. 2014

Acta Derm Venerol

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Section: Discussionmentioning

confidence: 99%

Genetic Analysis of CARD14 in Non-familial Pityriasis Rubra Pilaris: A Case Series

Hong

Chen²,

Chen³

et al. 2014

Acta Derm Venerol

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“…Direct immunofluorescence tests with antibodies to human immunoglobulins G, A and M and C3 were negative in all 15 cases investigated [17]. Western blot analysis of skin from 3 family members diagnosed with familial type PRP found an abnormal expression of keratins 6, 16 and 17 [18]. The diagnostic values of the two latter investigations remain unclear.…”

Section: Histopathologymentioning

confidence: 99%

Pityriasis Rubra Pilaris: Treatment with Biologics – A New Promising Therapy?

2012

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Pityriasis rubra pilaris (PRP) includes a spectrum of rare chronic inflammatory disorders with papulosquamous eruptions of unknown cause. Different etiologies have been proposed such as vitamin A metabolism dysfunction, association with autoimmune disorders, infection or malignancies. However, PRP seems to be a polygenic skin disorder. Classical systemic treatment is empirical and includes retinoids and methotrexate; however, only few series on treatments exist. Recently there has been an increasing number of reports documenting that new biologicals and in particular TNF-α blockers are safe and effective.

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“…Eine familiäre Form mit autosomal-dominanter Vererbung, variabler Expression und reduzierter Penetranz [10,15] wird ebenso wie eine zellulär entzünd-liche Komponente diskutiert [3]. Weiterhin wird auch über das paraneoplastische Auftreten einer Pityriasis rubra pilaris z.…”

Section: Diskussionunclassified

Pityriasis rubra pilaris

Beckmann

Neis

2006

Hautarzt

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Familial Pityriasis Rubra Pilaris

Cited by 54 publications

References 31 publications

Genetic Analysis of CARD14 in Non-familial Pityriasis Rubra Pilaris: A Case Series

Genetic Analysis of CARD14 in Non-familial Pityriasis Rubra Pilaris: A Case Series

Pityriasis Rubra Pilaris: Treatment with Biologics – A New Promising Therapy?

Pityriasis rubra pilaris

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