Clinical and Experimental Dermatology 1989; 14: 325-327.

Pityriasis rotunda in a Caucasian woman from theMediterranean area

R.SEGAL, E.HODAK AND M.SANDBANK Department of Dermatology, Beilmson Medical Center, PetahTiqva, and Tel Aviv University Sackler School of Medicine, Israel

Accepted for publication 23 January 1989

Summary

Pityriasis rotunda (PR) has been known to affect,, almostexclusively, Japanese and South African Bantu, often inassociation with various systemic diseases. However, theoccurrence of PR in Caucasian patients is extremely rare,and has been previously described in only three reports.

We hereby report a case of an apparently healthyCaucasian woman from Israel having clinical and histo-logical features characteristic of PR.

Pityriasis rotunda (PR) is a rare disorder of keratinizationcharacterized by strikingly well demarcated, perfectlyround scaly brownish patches of variable number anddiameter located mainly on the trunk and extremities.The exact place of this disorder within the nosology of thecutaneous dermatoses is not yet fully established. How-ever, most authors regard it as a special circumscribedform of acquired ichthyosis with a histological resem-blance to ichthyosis vulgaris.

For more than half a century following the firstdescription of this disorder in 1906' cases reported werealmost exclusively from the Far East, particularly fromJapan, or in South African Negroids, with a few cases ofWest Indian Negroids, usually in association with variousdebilitating diseases. The tirst report of a Caucasianpatient appeared in the literature in 1966 and since thenthere have been only two additional reports of PR inCaucasian patients.'"*

Case report

A 4()-year-old female (the mother of three children) whowas born in Morocco and had been living in Israel for 38years presented with a widespread, asymptomatic erup-tion of 7 years duration. The past and family history wereunremarkable. The eruption first appeared on the sides ofthe trunk, gradually spreading to the entire trunk andproximal parts of the extremities. The patient reported animprovement during the summer, attributing this tosunbathing.

Correspondence: R.Segal, Department of IJermatoIogy, BeilinsonMedical Center, Petah Tiqva, 49 100, Israel,

The patient's complexion was light and she had blueeyes. On the trunk, arms and thighs (Fig. 1) there weresharply demarcated, superficial plaques, round or oval inshape and light brown in colour but darker than thesurrounding skin, which were uniformly covered withichthyosiform scales. The diameter of the lesions rangedfrom 5 to 15 cm. There was a tendency to confluence butwith preservation of the typical borders (Fig. 2). Theremainder of the physical examination was unremarkable.Routine laboratory tests (ESR, complete blood count,urinalysis, renal and liver function) as well as chest X-raywere all within normal limits. Gynaecological examin-ation, including a cervical smear and ultrasound

Figure 1. Sharply demarcated superficial round to oval pigmentedscaly plaques on back of patient.

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326 R.SEGAL, E.HODAK AND M.SANDBANK

Figure 2. Plaques showing tendcncv to cuntlucnce while preservingthe typical borders.

Figure 3. Microscopic examination showing lamellar hvperkcratosis,a keratotic plaque, mild atrophy of the stratum malpighi and a sparsegranular layer. In the dermis slight perivascular monohistiocyticinfiltrate is seen (H & E x 47).

examination, were also normal. Potassium hydroxideexamination for fungi as well as Wood's lamp examin-ation gave negative results. Biopsy (Fig. 3) revealedlamellar hyperkeratinization, keratotic plaques, and mildatrophy of the malphigian layer. The granular layer was

thin and absent in places. In the basal cell layer there wasan increase ir: 'iigmentation. In the dermis there was aslight perivascular monohistiocytic infiltrate.

The patient refused to undertake a trial with oralsystemic Tigason and was treated with topical keratolyticand emollient agents. During the 1-year follow-up thedisease has been observed to progress, with no otherillness detectable.

Discusssion i

Pityriasis rotunda (PR) is generally regarded as a specialform of acquired circumscribed ichthyosis. The histo-logical features are essentially those seen in ichthyosisvulgaris, showing hyperkeratosis with a thin or absentgranular layer and mild lymphohistiocytic perivascularinfiltrate in the upper dermis. The clinical appearance is,however, unique,^ characterized by round, usually darkbrown patches. Most patients have multiple lesions withup to 28 patches that vary in diameter from 05 to 28 cmand occasionally become confluent. Although some of themorphological features are suggestive of tinea corporis,pityriasis versicolor or extraflexural erythrasma, allattempts to demonstrate an infective agent have beenunsuccessful,^ ^ as was experimental transfer of scales.''

The disease is noted for its peculiar racial andgeographical distribution and an association with aserious systemic disease has frequently been reported.

The first description of PR was made by Toyama in1906' in Tokyo, subsequently, similar cases werereported from Japan, Korea and Manchuria.''"" Toyamanoted that a high proportion of patients had tuberculosis,dysentery or endometriosis.^ In the review by Ito andTanaka,' more than one-third of the patients had anassociated disease—in most tuberculosis or a disorder ofthe female genital tract.

During the past three decades, the English literaturehas contained reports of PR in South AfricanNegroids,'''^'^'^ West Indian Negroids in London** and,recently, the first report of two American Negroids,'^making a total of 95 cases. Among these, 92 were found tobe associated with another disease, as follows: tuberculo-sis, '" 25 cases; cirrhosis,*''-'"' 16 cases; hepatocellularcarcinoma,'^ 10 cases; other forms of malignancy,'"'^ 10cases; malnutrition,'" eight cases; and other chronicdiseases including: cardiac disease, pulmonary disease,diabetes mellitus, hypertension. Most of these authorsregard PR as a form of acquired ichthyosis and a markerof a serious chronic illness.

The first description of PR in a Caucasian patient wasreported in 1966 by el-Hefnawi and Rasheed.^ Thepatient was a healthy 24-year-old Egyptian male with a10-year history of typical lesions of PR, exhibitingexacerbation in the winter. In 1986, Kahana and col-leagues"* reported a 19-year-old Israeli brunette of

PITYRIASIS ROTUNDA 327

Sephardic Jewish origin who had classical clinical andhistological features of PR of 7 years' duration, withimprovement in the summer. No internal disorder hadbeen found. In the same year, Zina and colleagues'described the occurrence of PR in another Mediterraneanlocation (Sardinia) but their report differs from theclassical description of PR since the disease appeared inseveral members of the same family (apparently healthychildren aged 11, 13 and 14 years), suggesting a heredi-tary disorder and, furthermore, the father suffered fromichythyosis vulgaris. The clinical appearance of theeruption in these three cases was also slightly different,the lesions being more numerous (more than 40) and mostbeing hypochromic. Itisof interest that these authors citeBonu (personal communication) as finding PR to becommon among Sardinians and showing a familialoccurrence.

Our patient constitutes yet another case of the rareoccurrence of PR in a Caucasian and shows similaritiesto the previous cases "* namely: Mediterranean origin;absence of family history; the classical clinical andhistological features of PR; presence of the rash for severalyears in a healthy young person having no apparentunderlying disease.

In summary, it would appear that, although PR hasbeen documented almost exclusively in certain geo-graphic and ethnic groups, most frequently among theJapanese and South African Bantu, and very often inassociation with various serious systemic diseases, it alsoexists among Caucasians. Up to now, cases have beenreported only from the Mediterranean, occurring inhealthy individuals either sporadically or as a familialtrend among Sardinians.

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