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The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis.
J Cutan Pathol. 1997 Aug; 24(7):416-24.JC

Abstract

Pityriasis rubra pilaris (PRP) is an idiopathic erythematous scaling eruption which can be difficult to distinguish from psoriasis. We explored the clinical features, including background medical illnesses and potential triggers in patients with PRP, and also its histomorphologic spectrum. Patients with PRP were selected by natural language search of dermatopathology databases containing 250,000 cases accessioned over a 3-year period. A detailed medical history was obtained on each patient via office chart review and patient interviews. We compared the histologic findings in skin biopsies from patients with PRP to those of biopsies matched for site and age from control patients with psoriasis. Certain aspects of the clinical presentations encountered in the study group patients have not been previously emphasized, including eruptions compatible with a vesiculobullous disorder in 4 patients. Several patients had evidence of underlying iatrogenic or systemic immune dysregulatory states and certain triggers could be isolated such as emotional stress and prior exposure to microbial pathogens. Histomorphologic features predictive of PRP included follicular plugging, an increased granular cell layer, and acantholysis. While seen in a minority of biopsies of PRP, morphologic discriminators supportive of a diagnosis of psoriasis included neutrophils in the parakeratotic scale crust, and dermal papillae capillary ectasia with vessels lying in direct apposition to the epidermis. Other morphologic features were encountered in biopsies of PRP which have received little emphasis in the dermatopathology literature, including epithelial atrophy, significant dermal inflammation, the presence of eosinophils and plasma cells within the inflammatory infiltrates, an abnormal granular cell layer, and epidermolytic hyperkeratosis, albeit none were statistically signficant predictors of PRP over psoriasis. Nevertheless, their identification emphasizes the varied histologic spectrum of PRP. The almost ubiquitous finding of acantholysis and the presence of an abnormal granular cell layer in many biopsies of PRP suggests that abnormal keratins or defects in the retinol signalling pathway may be of pathogenetic importance.

Authors+Show Affiliations

Department of Pathology, Beth Israel Hospital, Harvard Medical School, Pathology Services Inc., Cambridge, Massachusetts 02139, USA.No affiliation info available

Pub Type(s)

Case Reports
Comparative Study
Journal Article

Language

eng

PubMed ID

9274959

Citation

Magro, C M., and A N. Crowson. "The Clinical and Histomorphological Features of Pityriasis Rubra Pilaris. a Comparative Analysis With Psoriasis." Journal of Cutaneous Pathology, vol. 24, no. 7, 1997, pp. 416-24.
Magro CM, Crowson AN. The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. J Cutan Pathol. 1997;24(7):416-24.
Magro, C. M., & Crowson, A. N. (1997). The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. Journal of Cutaneous Pathology, 24(7), 416-24.
Magro CM, Crowson AN. The Clinical and Histomorphological Features of Pityriasis Rubra Pilaris. a Comparative Analysis With Psoriasis. J Cutan Pathol. 1997;24(7):416-24. PubMed PMID: 9274959.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. AU - Magro,C M, AU - Crowson,A N, PY - 1997/8/1/pubmed PY - 1997/8/1/medline PY - 1997/8/1/entrez SP - 416 EP - 24 JF - Journal of cutaneous pathology JO - J. Cutan. Pathol. VL - 24 IS - 7 N2 - Pityriasis rubra pilaris (PRP) is an idiopathic erythematous scaling eruption which can be difficult to distinguish from psoriasis. We explored the clinical features, including background medical illnesses and potential triggers in patients with PRP, and also its histomorphologic spectrum. Patients with PRP were selected by natural language search of dermatopathology databases containing 250,000 cases accessioned over a 3-year period. A detailed medical history was obtained on each patient via office chart review and patient interviews. We compared the histologic findings in skin biopsies from patients with PRP to those of biopsies matched for site and age from control patients with psoriasis. Certain aspects of the clinical presentations encountered in the study group patients have not been previously emphasized, including eruptions compatible with a vesiculobullous disorder in 4 patients. Several patients had evidence of underlying iatrogenic or systemic immune dysregulatory states and certain triggers could be isolated such as emotional stress and prior exposure to microbial pathogens. Histomorphologic features predictive of PRP included follicular plugging, an increased granular cell layer, and acantholysis. While seen in a minority of biopsies of PRP, morphologic discriminators supportive of a diagnosis of psoriasis included neutrophils in the parakeratotic scale crust, and dermal papillae capillary ectasia with vessels lying in direct apposition to the epidermis. Other morphologic features were encountered in biopsies of PRP which have received little emphasis in the dermatopathology literature, including epithelial atrophy, significant dermal inflammation, the presence of eosinophils and plasma cells within the inflammatory infiltrates, an abnormal granular cell layer, and epidermolytic hyperkeratosis, albeit none were statistically signficant predictors of PRP over psoriasis. Nevertheless, their identification emphasizes the varied histologic spectrum of PRP. The almost ubiquitous finding of acantholysis and the presence of an abnormal granular cell layer in many biopsies of PRP suggests that abnormal keratins or defects in the retinol signalling pathway may be of pathogenetic importance. SN - 0303-6987 UR - https://www.unboundmedicine.com/medline/citation/9274959/The_clinical_and_histomorphological_features_of_pityriasis_rubra_pilaris__A_comparative_analysis_with_psoriasis_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0303-6987&date=1997&volume=24&issue=7&spage=416 DB - PRIME DP - Unbound Medicine ER -