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Pityriasis rubra pilaris - External and Internal Eye
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Pityriasis rubra pilaris - External and Internal Eye

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Contributors: Vivian Wong MD, PhD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

This summary discusses pityriasis rubra pilaris in adults. Pityriasis rubra pilaris in children is addressed separately.

Pityriasis rubra pilaris (PRP) is a papulosquamous disorder with characteristic clinical features. There are both acquired and heritable forms. It is characterized in the acquired form by a fairly rapid onset of extensive, confluent, scaling plaques in association with thick keratoderma of the hands. It often begins in the scalp and rapidly involves the trunk and extremities.

The heritable form of the disease begins in early childhood, while the incidence of the acquired form peaks in both childhood and mid-to-late adulthood. In addition to the generalized disease, a limited form is seen in both children and adults. Human immunodeficiency virus (HIV)-associated PRP can be associated with nodulocystic acne and responds to antiretroviral therapy.

Peripheral ulcerative keratitis and cicatricial ectropion have been seen with PRP. Blurred vision and dry eyes may be reported.

For more information, see OMIM.

Codes

ICD10CM:
L44.0 – Pityriasis rubra pilaris

SNOMEDCT:
3755001 – Pityriasis rubra pilaris

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Psoriasis – See Diagnostic Pearls.
  • Seborrheic dermatitis – Much more responsive to standard therapies. PRP is often resistant to conventional therapies for seborrheic dermatitis. In addition, the progression of body surface involvement will distinguish PRP from seborrheic dermatitis.
  • Mycosis fungoides / Sézary syndrome – Generalized lymphadenopathy, circulating malignant lymphocytes as determined by flow cytometry, leonine facies, and a CD4/CD8 ratio greater than 10 as determined by flow cytometry.
  • Erythrokeratodermia variabilis – More than 90% of patients present within the first year of life.
  • Drug eruption – Often presents with urticarial, exanthematous, or vesicular / bullous lesions. In addition, systemic symptoms are more pronounced, including fever, lymphadenopathy, and facial edema. Eosinophilia on CBC and histology are often seen (but not an invariable finding). Medication history will help.
  • Atopic dermatitis – Patients are often aware of their atopic history, and the condition commonly starts in childhood. Look for lichenified plaques on the flexural surfaces and neck.
  • Caspase recruitment domain family member 14 gene (CARD14)-associated papulosquamous eruption refers to a distinctive phenotype with overlapping features of psoriasis and PRP. The patients typically present early in life and report a family history of psoriasis or PRP. The cheeks, chin, and ears are typically affected. This disease is difficult to treat using conventional therapies, while treatment with ustekinumab appears to be effective.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 08/30/2018
Last Updated: 09/06/2018
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Pityriasis rubra pilaris - External and Internal Eye
See also in: Overview
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Pityriasis rubra pilaris : Confluent configuration, Erythroderma, Follicular configuration, Ichthyotic scaly plaque, Palms and soles, Widespread
Clinical image of Pityriasis rubra pilaris
A close-up of an area of normal skin ("island of sparing") surrounded by a deep pink, scaly plaque.
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