Pityriasis Rubra Pilaris Workup

  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 20, 2012
 

Laboratory Studies

No specific laboratory tests are available to confirm the diagnosis of pityriasis rubra pilaris (PRP). The diagnosis is usually made on the basis of a correlation between clinical findings and histologic findings.[13]

Perform tests for electrolyte abnormalities, hypoalbuminemia, secondary bacterial infection in the skin, and possible sepsis.

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Procedures

Perform a biopsy.

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Histologic Findings

Histologic features are not pathognomonic, but they are useful to rule out other possible papulosquamous and erythrodermic disorders. Features on light microscopy include hyperkeratosis with alternating orthokeratosis and parakeratosis forming a checkerboard pattern in the stratum corneum, focal or confluent hypergranulosis, follicular plugging with perifollicular parakeratosis forming a shoulder effect, thick suprapapillary plates, broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic perivascular infiltration. Acantholysis has been reported as an additional histologic finding in pityriasis rubra pilaris.[14] Acantholysis may be restricted to adnexal epithelium. The presence of acantholysis, hypergranulosis, follicular plugging, and the absence of dilated capillaries and epidermal pustulation may help distinguish pityriasis rubra pilaris from psoriasis.[15]

Features on electron microscopy include a decreased number of keratin filaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipidlike vacuoles, large numbers of lamellar granules, and a focal split in the basal lamina at the dermoepidermal junction.

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Contributor Information and Disclosures
Author

Philip D Shenefelt, MD, MS  Professor, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine; Past Chief, Section of Dermatology, James A Haley Veteran Affairs Medical Center

Philip D Shenefelt, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Physician Executives, American Contact Dermatitis Society, American Medical Association, American Society of Clinical Hypnosis, Florida Medical Association, Noah Worcester Dermatological Society, and Society for Clinical and Experimental Hypnosis

Disclosure: Nothing to disclose.

Specialty Editor Board

Mark G Lebwohl, MD  Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Amgen/Pfizer Honoraria Consulting; GlaxoSmithKline Honoraria Consulting; Novartis Honoraria Consulting; Ranbaxy Honoraria Lectures; Pfizer Honoraria Consulting; BioLineRX, Ltd. Honoraria Consulting; Celgene Corporation Consulting; Clinuvel None Investigator; Eli Lilly & Co. None Investigator; Genentech Honoraria Consulting

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Margaret H. Rinker, MD, to the development and writing of this article.

References

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Reddish orange plaques on the trunk.
Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.
Plantar keratoderma with an orange hue on the soles.
Palmar keratoderma with an orange hue on the palms.
 
 
 
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