eMedicine Specialties > Dermatology > Papulosquamous Diseases

Pityriasis Rotunda

Author: Jaggi Rao, MD, Associate Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, University of Alberta; Consulting Staff, Dermatology and Dermatologic Surgery, University of Alberta and Acne Clinics of Canada
Coauthor(s): Andrei I Metelitsa, MD, Chief Resident, Division of Dermatology and Cutaneous Sciences, University of Alberta, Canada; Andrew Lin, MD, FRCPC, Associate Professor, Department of Internal Medicine, Division of Dermatology, University of Alberta
Contributor Information and Disclosures

Updated: Mar 12, 2009

Introduction

Background

Pityriasis rotunda (PR) is an idiopathic, chronic dermatosis that features characteristic discrete, round, scaly, pigmented patches.1,2,3,4,5,6,7,8,9,10 Pityriasis rotunda may be associated with systemic diseases (eg, hepatocellular carcinoma) in certain racially predisposed groups.

Pathophysiology

The pathophysiology of pityriasis rotunda is unknown. It may be a variant of ichthyosis vulgaris.

Frequency

United States

Pityriasis rotunda is very uncommon in America. A review of English-language literature revealed only 5 case reports from America, which described 6 patients.1,11,12,13,14

International

Pityriasis rotunda is a well-known condition in South Africa, Japan, and Italy. Pityriasis rotunda was seen in 65 (1.01%) of 6388 South African medical inpatients. Japanese investigators reported 181 cases of pityriasis rotunda in 1960. Forty-two cases, all from the Italian island of Sardinia, were reviewed in 1997.15 In 1989, one review of the English-language literature discussed 89 previously reported pityriasis rotunda patients. Cases of pityriasis rotunda have been reported in Israel, England, Egypt, Portugal, Tanzania, and India.2,16,17,18,19,20,21

Mortality/Morbidity

Lesions of pityriasis rotunda are not associated with mortality, and most are asymptomatic. Pityriasis rotunda can occur with serious underlying systemic diseases, such as hepatocellular carcinoma.22,23

Race

Most cases of pityriasis rotunda from South Africa, and all 6 cases from America, have occurred in blacks.

  • In the 1960 review of 181 cases in the French-language literature (performed by Japanese investigators), 175 patients were from Japan, 4 were from Korea, and 2 were from Manchuria.24
  • Cases have been reported among West Indians living in England.18
  • Other than the cluster of 42 patients (some familial) reported from the Italian island of Sardinia, pityriasis rotunda appears to be very uncommon among whites.15,25

Sex

No sex predilection has been demonstrated in several large series.

  • One series had 77 males and 63 females; another had 73 males and 101 females.
  • The report of Sardinian patients had 22 males and 20 females.15

Age

Lesions are often first noted in adulthood, usually when aged 20-45 years, but a patient as old as 76 years has been reported. In the review of 42 cases from Sardinia, the average age of onset was estimated to be 3-7 years.

Clinical

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.

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.3,26

  • 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.
Nearly perfectly round, slightly hyperkeratotic, ...

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

Nearly perfectly round, slightly hyperkeratotic, ...

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


Causes

The cause of pityriasis rotunda is unknown. Some authors believe it is a variant of ichthyosis vulgaris because both have similar histologic findings.27 Some authors believe pityriasis rotunda is caused by malnutrition, but this is not universally accepted.28 In familial cases, an autosomal dominant mode of transmission has been proposed.3,29,30,31 The presence of systemic disease appears to be common in black South African and Japanese patients, but is much less likely among white patients.32

  • 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
      • Tuberculosis33
      • 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.34
  • 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.35
  • 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.29,36
  • American patients: Pityriasis rotunda has been reported in 6 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 patient did not have any associated medical conditions. The sixth patient had associated sarcoidosis.1,11,12,13,14

More on Pityriasis Rotunda

Overview: Pityriasis Rotunda
Differential Diagnoses & Workup: Pityriasis Rotunda
Treatment & Medication: Pityriasis Rotunda
Follow-up: Pityriasis Rotunda
Multimedia: Pityriasis Rotunda
References

References

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  3. Grimalt R, Gelmetti C, Brusasco A, Tadini G, Caputo R. Pityriasis rotunda: report of a familial occurrence and review of the literature. J Am Acad Dermatol. Nov 1994;31(5 Pt 2):866-71. [Medline].

  4. L'Henaff N, Combemale P. [Pityriasis rotunda. Review of the literature]. Ann Dermatol Venereol. 1993;120(4):305-9. [Medline].

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  7. Toyama T. Uber eine bisher noch nicht beschriebene dermatose: "pityriasis circinata". Arch Dermatol Syphiligr. 1913;116:243-58.

  8. Toyama T. Uber eine schuppende, pigmentierte: kreisrunde hautaffektion. Jpn Z Derm Urol. 1906;6:2.

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  11. Finch JJ, Olson CL. Hyperpigmented patches on the trunk of a Nigerian woman. Pityriasis rotunda (PR). Arch Dermatol. Nov 2008;144(11):1509-14. [Medline].

  12. Mafong EA. Pityriasis rotunda. Dermatol Online J. Oct 2002;8(2):15. [Medline].

  13. Porges DY, Scott RA. Pityriasis rotunda with HTLV-1 associated tropical spastic paraparesis. NY. 1992;184A.

  14. Rubin MG, Mathes B. Pityriasis rotunda: two cases in black Americans. J Am Acad Dermatol. Jan 1986;14(1):74-8. [Medline].

  15. Aste N, Pau M, Aste N, Biggio P. Pityriasis rotunda: a survey of 42 cases observed in Sardinia, Italy. Dermatology. 1997;194(1):32-5. [Medline].

  16. Gibbs S. Pityriasis rotunda in Tanzania. Br J Dermatol. Sep 1996;135(3):491-2. [Medline].

  17. Gupta S. Pityriasis rotunda mimicking tinea cruris/corporis and erythrasma in an Indian patient. J Dermatol. Jan 2001;28(1):50-3. [Medline].

  18. Sarkany I, Hare PJ. Pityriasis rotunda (pityriasis circinata). Br J Dermatol. May 1964;76:223-8. [Medline].

  19. el-Hefnawi H, Rasheed A. Pityriasis rotunda. "Pseudo-ichtyose acquise en taches circulaires": report and study of first case in UAR. Arch Dermatol. Jan 1966;93(1):84-6. [Medline].

  20. Hasson I, Shah P. Pityriasis rotunda. Indian J Dermatol Venereol Leprol. Jan-Feb 2003;69(1):50-1. [Medline].

  21. Segal R, Hodak E, Sandbank M. Pityriasis rotunda in a Caucasian woman from the Mediterranean area. Clin Exp Dermatol. Jul 1989;14(4):325-7. [Medline].

  22. Berkowitz I, Hodkinson HJ, Kew MC, DiBisceglie AM. Pityriasis rotunda as a cutaneous marker of hepatocellular carcinoma: a comparison with its prevalence in other diseases. Br J Dermatol. Apr 1989;120(4):545-9. [Medline].

  23. DiBisceglie AM, Hodkinson HJ, Berkowitz I, Kew MC. Pityriasis rotunda. A cutaneous marker of hepatocellular carcinoma in South African blacks. Arch Dermatol. Jul 1986;122(7):802-4. [Medline].

  24. Ito M, Tanaka T. Pseudo-ichthyose acquise en taches circulaires. Ann Dermatol Syphiligr. 1960;87:26-37.

  25. Kahana M, Levy A, Ronnen M, Schewach-Millet M, Stempler D. Pityriasis rotunda in a white patient. Report of the second case and review of the literature. J Am Acad Dermatol. Aug 1986;15(2 Pt 2):362-5. [Medline].

  26. Ena P, Cerimele D. Pityriasis rotunda in childhood. Pediatr Dermatol. May-Jun 2002;19(3):200-3. [Medline].

  27. Combemale P, L'Henaff N, Guennoc B. [Pityriasis rotunda]. Ann Dermatol Venereol. 1993;120(4):287-8. [Medline].

  28. Swift PJ, Saxe N. Pityriasis rotunda in South Africa--a skin disease caused by undernutrition. Clin Exp Dermatol. Sep 1985;10(5):407-12. [Medline].

  29. Lodi A, Betti R, Chiarelli G, Carducci M, Crosti C. Familial pityriasis rotunda. Int J Dermatol. Sep 1990;29(7):483-5. [Medline].

  30. Friedmann AC, Ameen M, Swale VJ. Familial pityriasis rotunda in black-skinned patients; a first report. Br J Dermatol. Jun 2007;156(6):1365-7. [Medline].

  31. Guberman D, Lichtenstein DA, Gilead L, Vardy DA, Klaus SN. Familial pityriasis rotunda. Acta Derm Venereol. Mar 1997;77(2):162. [Medline].

  32. Kurzrock R, Cohen PR. Cutaneous paraneoplastic syndromes in solid tumors. Am J Med. Dec 1995;99(6):662-71. [Medline].

  33. Weiss RM. Pigmented lesions in a patient with pulmonary tuberculosis. Pityriasis rotunda. Arch Dermatol. Aug 1991;127(8):1221, 1224. [Medline].

  34. Leibowitz MR, Weiss R, Smith EH. Pityriasis rotunda. A cutaneous sign of malignant disease in two patients. Arch Dermatol. Jul 1983;119(7):607-9. [Medline].

  35. Etoh T, Nakagawa H, Ishibashi Y. Pityriasis rotunda associated with multiple myeloma. J Am Acad Dermatol. Feb 1991;24(2 Pt 1):303-4. [Medline].

  36. Piga S, Cottoni F, Meloni GF. Pityriasis rotunda and G6PD deficiency. Int J Dermatol. Oct 1992;31(10):745. [Medline].

  37. Agostini G, Hoang-Xuan D, Rybojad M, Puissant A. [Toyama's pityriasis rotunda]. Ann Dermatol Venereol. 1988;115(11):1226-7. [Medline].

  38. Alberti A, Conte R, Reggiani M. [Pityriasis rotunda: study of 2 family groups. Preliminary report]. G Ital Dermatol Venereol. Apr 1988;123(4):159-60. [Medline].

  39. Aste N, Biggio P. [Pityriasis rotunda in south-central Sardinia. Preliminary note]. G Ital Dermatol Venereol. Sep-Oct 1986;121(5):331-4. [Medline].

  40. Aste N, Pau M, Aste N, Biggio P. Case report. Pityriasis versicolor mimicking Pityriasis rotunda. Mycoses. Apr 2002;45(3-4):126-8. [Medline].

  41. Desruelles F, Gari-Toussaint M, Lacour JP, Marty P, Le Fichoux Y, Ortonne JP. Tinea versicolor mimicking pityriasis rotunda. Int J Dermatol. Dec 1999;38(12):948-9. [Medline].

  42. Ena P, Siddi GM. Pityriasis versicolor resembling pityriasis rotunda. J Eur Acad Dermatol Venereol. Jan 2002;16(1):85-7. [Medline].

  43. Griffin LJ, Massa MC. Acquired ichthyosis and pityriasis rotunda. Clin Dermatol. Jan-Mar 1993;11(1):27-32. [Medline].

  44. Ikada J, Oki M. Concurrent pityriasis rotunda and acquired ichthyosis with IgG myeloma. Br J Dermatol. Nov 1974;91(5):585-6. [Medline].

  45. Lewis AD. Pityriasis rotunda: a dermatophytosis?. Arch Dermatol. Apr 1987;123(4):426-7. [Medline].

Further Reading

Keywords

pityriasis circinata, tinea circinata, pseudo-ichtyose acquise en taches en circulaires, pseudoichtyose acquise en taches en circulaires, PR, hepatocellular carcinoma, HCC, ichthyosis vulgaris, internal malignancy

Contributor Information and Disclosures

Author

Jaggi Rao, MD, Associate Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, University of Alberta; Consulting Staff, Dermatology and Dermatologic Surgery, University of Alberta and Acne Clinics of Canada
Jaggi Rao, MD 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 Dermatology Association, Canadian Medical Association, Canadian Medical Protective Association, Pacific Dermatologic Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Andrei I Metelitsa, MD, Chief Resident, Division of Dermatology and Cutaneous Sciences, University of Alberta, Canada
Andrei I Metelitsa, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Canadian Dermatology Association, and Canadian Medical Protective Association
Disclosure: Nothing to disclose.

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 and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, 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 Medical Association, American Society of Dermatopathology, Medical Society of Virginia, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

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