A Clinical and Histopathologic Study of Pityriasis Rubra Pilaris / 대한피부과학회지

BACKGROUND: Pityriasis rubra pilaris (PRP)is a rare papulosquamous disorder of unknown etiology. Although the clinical and histopathologic features of PRP are relatively well known in western patients, there are few available studies in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathologic features of pityriasis rubra pilaris(PRP) in Korea METHODS: The clinical charts, photographies and skin biopsy slides of patients clinically diagnosed as PRP at the initial visit were reviewed. Finally 30 patients were diagnosed as PRP. They were classified into 5 groups according to the Griffiths classification. And then the evaluation of age at onset, sex distribution, clinical feature of the skin lesion, associated symptoms, clinical course and histopathologic features were conducted. RESULTS: 1) The male to female ratio was 1:1.14. The age of onset was varied from 1 to 65 years with two peaks in first and fifth decades. The average ages of onset in adult type and juvenile type were 40.1 and 5.1 years respectively. 2) According to the Griffiths classification, 14(47%) of the patients had type I, 4(13%) had type III, 7(23%) had type IV, and 3(10%) had type V. However, 2(7%)patients could not be classified under any of these five types due to atypical clinical feature or insufficient follow-up periods. 3) The common clinical features during the disease course were follicular hyperkeratosis(83%), knee and/or elbow involvement(83%), palm and/or sole hyperkeratosis(80%). Erythroderma was present in 5 patients(17%) during the disease course. 4) There were no specific symptoms in most cases, but 9 patients had ben suffered from mild itching. 5) 3 patients had a positive family history, and were thought to have inherited autosomal dominant trait. 6) The frequent histopathologic findings were alternating parakeratosis and orthokeratosis (100%), superficial perivascular infiltration(100%), broad rete ridge(97%), acanthosis(93%), hypergranulosis (87%), follicular plugging(73%). CONCLUSION: We investigated the clinical and histopathological features of PRP in Korea. In our study, the clinical and histopathological features of PRP were not different from those observed in western patients.

A Clinical Study of 30 Cases of Pityriasis Rubra Pilaris / 대한피부과학회지

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare disease characterized by widespread follicular acuminate papules. Traditionally, two forms have been described : hereditary (juvenile) and acquired (generally aduIrt). A.substantial contribution, however, suggests that further subdivision is appropriate. OBJECTIVE: The purpose of this study was to investigate clinicopathologic features of PRP and to compare the results of clinical classification with Griffiths' classification. METHODS: The retrospective clinicopathologic study carried out in 30 cases of PRP over the last 30 years. RESULTS: 1. The incidence of type lV was 38.5%. For both type I and III it was 30.8%. The onset ages of the type I, III, and lV were 36.1, 6.0, and 96 years respectively. 2. The most common site of initial involvement was the palms, and the most common involvement site during the diease course was the dorsa of palms and feet. The incidence involved in the whole body was 46.6%. 3. The incidences of insidious and acute onset were 60% and 40% respectively. No case had the family history of PRP. 4. In the histopathologic study, diffuse hyperkeratosis, acanthosis, and mild dermal infiltrations were found in all cases. Follicular pluggings were observed in 93.3% of cases CONCLUSION: We investigated the clinical classifications and clinicopathologic features of PRP. We think that more studies such as prognosis and treatment of PRP should be achieved in the near future.