Pityriasis Rotunda Clinical Presentation
- Author: Jaggi Rao, MD, FRCPC; Chief Editor: Dirk M Elston, MD more...
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.
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.
The cause of pityriasis rotunda is unknown. Some authors believe it is a variant of ichthyosis vulgaris because both have similar histologic findings. Some authors believe pityriasis rotunda is caused by malnutrition, but this is not universally accepted. 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
Chronic renal failure
Resolution of pityriasis rotunda has been noted with treatment of the underlying malignancy.
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.
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]
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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