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Pityriasis Rotunda Clinical Presentation

  • Author: Jaggi Rao, MD, FRCPC; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jul 15, 2016
 

History

Pityriasis rotunda patients are usually asymptomatic. In one review of 64 cases, all patients were undergoing assessment for an underlying medical condition and the lesions of pityriasis rotunda were noted incidentally. Few could remember the duration of the lesions, which varied from 1 week to 2 years.

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Physical

Two clinical types of pityriasis rotunda are described. Type 1 affects Asian or black patients older than 60 years old who tend to have associated malignancies or systemic disease. These patients present with few hyperpigmented patches (< 30) that are usually nonfamilial. Type 2 affects white patients younger than 40 years who do not have any associated malignancy or systemic diseases and who present with multiple hypopigmented patches (>30) that are familial.[3, 27]

Lesions range from pink to light-brown, are usually perfectly round but sometimes can be oval, and appear as well-demarcated patches that show fine scaling. They range from 0.5-20 cm and are generally isolated, but the merging of lesions results in a polycyclic configuration.

Several lesions are usually present, ranging from 4-80. One patient had more than 100 lesions.

Lesions are usually present on the trunk, buttocks, and upper and lower extremities. See the image below.

Nearly perfectly round, slightly hyperkeratotic, h Nearly perfectly round, slightly hyperkeratotic, hyperpigmented, asymptomatic plaque on the trunk.
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Causes

The cause of pityriasis rotunda is unknown. Some authors believe it is a variant of ichthyosis vulgaris because both have similar histologic findings.[28] Some authors believe pityriasis rotunda is caused by malnutrition, but this is not universally accepted.[29] In familial cases, an autosomal dominant mode of transmission has been proposed.[3, 30, 31, 32] The presence of systemic disease appears to be common in black South African and Japanese patients, but is much less likely among white patients.[33, 34, 35]

Black South African patients

In one series of 10 black South African patients with pityriasis rotunda, 7 had hepatocellular carcinoma. Pityriasis rotunda occurred in 15.9% of 63 unselected black South African patients with hepatocellular carcinoma, which is significantly greater than its prevalence in 63 matched controls with each of the following:

  • Active tuberculosis (4.8%)
  • Chronic benign hepatic disease (3.2%)
  • Other malignancy (0%)

In South African patients, pityriasis rotunda has also been associated with the following:

  • Chronic myeloid leukemia
  • Squamous cell carcinoma of the hard palate
  • Tuberculosis [36]
  • Liver disease
  • Cardiac disease
  • Nutritional disease
  • Various malignancies
  • Pulmonary disease
  • Chronic renal failure
  • Osteitis
  • Chronic diarrhea
  • Scleroderma

Resolution of pityriasis rotunda has been noted with treatment of the underlying malignancy.[37]

Japanese patients

In the report of 181 patients (largely Japanese) in the French-language literature, 4 cases were associated with carcinoma of the stomach, 3 with carcinoma of the liver, 1 with carcinoma of the lung, 3 with unspecified malignant tumors.

In the same series, one Japanese patient with pityriasis rotunda had multiple myeloma, while another had myeloma and hepatic cirrhosis.[38]

White patients

The association with systemic disease appears much less common among white patients. However, some cases are familial, suggesting an autosomal dominant pattern of inheritance.

In one Sardinian family, pityriasis rotunda occurred in 3 siblings, whose father had typical ichthyosis vulgaris.

In another Sardinian family, 5 of 18 affected individuals had favism (deficiency of glucose-6-phosphate dehydrogenase). Such an association is likely coincidental because favism is common among individuals from that family's place of origin.[30, 39]

American patients

Pityriasis rotunda has been reported in 7 African Americans. One patient had metastatic adenocarcinoma, one had diabetes and unexplained thrombocytopenia, while another had human T-cell lymphotropic virus type 1–associated tropical spastic paraparesis. The fourth had mild hypertension. The fifth and sixth patients did not have any associated medical conditions. The sixth patient had associated sarcoidosis.[1, 11, 12, 13, 14, 15]

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Contributor Information and Disclosures
Author

Jaggi Rao, MD, FRCPC Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, Director of Dermatology Residency Program, University of Alberta Faculty of Medicine and Dentistry

Jaggi Rao, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Medical Association, Pacific Dermatologic Association, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Coauthor(s)

Andrew Lin, MD, FRCPC Associate Professor, Department of Internal Medicine, Division of Dermatology, University of Alberta

Andrew Lin, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Andrei I Metelitsa, MD, FRCPC Assistant Clinical Professor, Section of Dermatology, University of Calgary Faculty of Medicine, Canada

Andrei I Metelitsa, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Medical Protective Association, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

James W Patterson, MD Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

References
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Nearly perfectly round, slightly hyperkeratotic, hyperpigmented, asymptomatic plaque on the trunk.
Classic histology for pityriasis rotunda demonstrating mild hyperkeratosis and a reduced granular layer in the epidermis.
 
 
 
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