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Dermatology > PAPULOSQUAMOUS DISEASES
Pityriasis Rotunda
Article Last Updated: Feb 22, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
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 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
Coauthor(s):
Andrew Lin, MD, FRCPC, Associate Professor, Department of Internal Medicine, Division of Dermatology, University of Alberta;
Andrei I Metelitsa, MD, Resident, Division of Dermatology and Cutaneous Sciences, University of Alberta, Canada
Editors: James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, University of Virginia Medical Center; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio; 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; 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
Author and Editor Disclosure
Synonyms and related keywords:
pityriasis circinata, pseudo-ichtyose acquise en taches en circulaires, pseudoichtyose acquise en taches en circulaires, PR, hepatocellular carcinoma, HCC, ichthyosis vulgaris, internal malignancy
Background
Pityriasis rotunda (PR) is an idiopathic, chronic dermatosis that features characteristic discrete, round, scaly, pigmented patches. It may be associated with systemic diseases (eg, hepatocellular carcinoma) in certain racially predisposed groups.
Pathophysiology
The pathophysiology of PR is unknown. It may be a variant of ichthyosis vulgaris.
Frequency
United States
PR is very uncommon in America. A review of English-language literature revealed only 4 case reports from America, which described 5 patients.
International
PR is a well-known condition in South Africa, Japan, and Italy. It was seen in 65 (1.01%) of 6388 South African medical inpatients. Japanese investigators reported 181 cases in 1960. Forty-two cases, all from the Italian island of Sardinia, were reviewed in 1997. In 1989, one review of the English-language literature discussed 89 previously reported patients. Cases have been reported in Israel, England, Egypt, Portugal, Tanzania, and India.
Mortality/Morbidity
Lesions of PR are not associated with mortality, and most are asymptomatic. PR can occur with serious underlying systemic diseases, such as hepatocellular carcinoma.
Race
Most cases from South Africa, and all 5 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.
- Cases have been reported among West Indians living in England.
- Other than the cluster of 42 patients (some familial) reported from the Italian island of Sardinia, PR appears to be very uncommon among whites.
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.
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.
History
- Patients are usually asymptomatic.
- In one review of 64 cases, all patients were undergoing assessment for an underlying medical condition and the lesions of PR were noted incidentally.
- Few could remember the duration of the lesions, which varied from 1 week to 2 years.
Physical
Two clinical types of PR 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 familial. Type 2 affects white patients younger than 40 years who do not have any associated systemic diseases and who present with multiple hypopigmented patches (>30) that are familial.
- 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.
Causes
The cause of PR is unknown. Some authors believe it is a variant of ichthyosis vulgaris because both have similar histologic findings. Some authors believe PR is caused by malnutrition, but this is not universally accepted. In familial cases, an autosomal dominant mode of transmission has been proposed. The presence of systemic disease appears to be common in black South African and Japanese patients, but is much less likely among white patients.
- Black South African patients
- In one series of 10 black South African patients with PR, 7 had hepatocellular carcinoma. PR 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, PR has also been associated with the following:
- Chronic myeloid leukemia
- Squamous cell carcinoma of the hard palate
- Tuberculosis
- Liver disease
- Cardiac disease
- Nutritional disease
- Various malignancies
- Pulmonary disease
- Chronic renal failure
- Osteitis
- Chronic diarrhea
- Scleroderma
- Resolution of PR has been noted with treatment of the underlying malignancy.
- 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 PR had multiple myeloma, while another had myeloma and hepatic cirrhosis.
- 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, PR 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.
- American patients: PR has been reported in 5 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
Erythrasma
Parapsoriasis
Psoriasis, Plaque
Tinea Corporis
Tinea Cruris
Tinea Versicolor
Other Problems to be Considered
Ichthyosis vulgaris (which also may be associated with internal malignancy)
Lab Studies
- Scrapings of skin lesions can be examined using potassium hydroxide mount and sent for fungal culture to rule out tinea corporis. Examination with a Wood light is useful in cases suggestive of extraflexural erythrasma.
Imaging Studies
- Use history and physical examination findings directed toward any underlying systemic diseases to guide further laboratory and imaging studies.
- Consider investigations into the possibility of hepatocellular carcinoma in black South African patients.
Procedures
- Skin biopsy is often useful to help exclude other diseases listed in Differentials.
Histologic Findings
Mild hyperkeratosis and a reduced or absent granular layer are found.
Medical Care
Improvement has been noted with 0.1% tretinoin cream, 10% lactic acid lotion, and 5% salicylic acid ointment; however, the lesions are usually resistant to therapy. Improvement with treatment of the underlying disease has also been noted.
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Retinoids
Decrease cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. Modulate keratinocyte differentiation. Have been shown to reduce risk of skin cancer formation in renal transplant patients.
| Drug Name | Tretinoin (Avita, Retin-A) |
|---|
| Description | Inhibits microcomedo formation and eliminates lesions. Makes keratinocytes in sebaceous follicles less adherent and easier to remove. Available as 0.025%, 0.05%, and 0.1% creams. Available also as 0.01% and 0.025% gels. |
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| Adult Dose | 0.1% cream: Apply hs or qod; lower frequency of application if irritation develops |
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| Pediatric Dose | <12 years: Not established
>12 years: Administer as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Toxicity increases with coadministration of benzoyl peroxide, salicylic acid, or resorcinol; avoid topical sulfur, resorcinol, salicylic acid, other keratolytics, abrasives, astringents, spices, and lime |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | May take several wk for skin to adapt to irritative effect of tretinoin; noncompliance from warmth and redness is decreased by starting application qwk and slowly increasing to qhs; caution in eczema; do not apply to mucous membranes, mouth, and angles of nose; minimize exposure to sun and UV light |
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Drug Category: Emollients
Contain colloidal solids and various oils that act as emollients. Recommended for relief of minor skin irritations and pruritus associated with common dermatoses and dry skin conditions.
| Drug Name | Ammonium lactate (Lac-Hydrin, AmLactin) 12% cream or lotion |
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| Description | Relieves itching and aids healing of skin in mild eczemas and dermatoses; itching skin, minor wounds, and minor skin irritations. Lactic acid is an alpha-hydroxy acid with keratolytic properties. |
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| Adult Dose | Apply qd/tid |
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| Pediatric Dose | Apply as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | May cause stinging and burning at application site |
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Drug Category: Keratolytic agents
Cause cornified epithelium to swell, soften, macerate, and then desquamate.
| Drug Name | Salicylic acid 6% cream or lotion (Salex) or 5% ointment |
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| Description | By dissolving the intercellular cement substance, produces desquamation of the horny layer of skin while not affecting structure of viable epidermis.
Hydrate skin and enhance effects of medication by soaking affected area in warm water for 5 min prior to use. Remove any loose tissue with brush, washcloth, or emery board and dry thoroughly.
Improvement generally occurs in 1-2 wk. Maximum resolution expected after 4-6 wk. |
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| Adult Dose | Apply to affected area qd/bid |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; prolonged use in infants, patients with diabetes, and patients with impaired circulation; use on moles, birthmarks, warts with hair growing from them, genital or facial warts, warts on mucous membranes or irritated skin, or any area infected or reddened |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Avoid contact with mucous membranes, normal skin surrounding warts, and eyes; immediately flush with water for 15 min if contact with eyes or mucous membranes occurs; avoid inhaling vapors |
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Prognosis
- The prognosis is excellent. Lesions sometimes resolve with treatment of the associated systemic disorder.
Medical/Legal Pitfalls
- Failure to consider internal malignancy in appropriate situation
- Failure to inform patient of possible adverse effects of treatment and resistant nature of lesions
| Media file 1:
Nearly perfectly round, slightly hyperkeratotic, hyperpigmented, asymptomatic plaque on the trunk. |
 |  View Full Size Image | |
Media type: Photo
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| Media file 2:
Classic histology for pityriasis rotunda demonstrating mild hyperkeratosis and a reduced granular layer in the epidermis. |
 | View Full Size Image | |
Media type: Photo
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Pityriasis Rotunda excerpt Article Last Updated: Feb 22, 2007
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