Pityriasis rubra pilaris (PRP) is a rare group of hyperkeratotic, papulosquamous disease that can be acquired or inherited. There have been reported cases of rheumatologic associations, mainly arthritis and dermatomyositis. In this review article, we will explore the clinical presentation and classification, rheumatologic associations and treatment modalities of PRP. In addition, we will also report a case of PRP with seronegative arthritis.Keywords: Pityriasis rubra pilaris; Review; Arthritis; Rheumatologic; Treatment
CASE REPORTA 50-year-old East Indian male with a 2-year history of nonspecific knee arthritis presented to his primary care doctor with a new scaly and pruritic rash. It began on the extensor surfaces of the elbow and bilaterally spread onto the forearms. Similar lesions began to appear over the scalp, trunk, axilla and knee. His nails became thickened and hyperpigmented. The patient was diagnosed with psoriasis. With the appearance of the lesions, his knee pain intensified, especially when ascending the stairs. The pain would awaken him at night. During this period, he had stiffening of his extremities. Besides fatigue, he denied any recent acute illnesses, constitutional symptoms, and mucocutaneous or ocular lesions. His past medical history included hypothyroidism and seasonal allergies. He denied food, drug and environmental allergies. His medication list at presentation included levothyroxine and glucosamine and chondroitin. He smoked tobacco occasionally and owned a cat. He had not traveled outside the country recently. His mother had nonspecific arthritis. On physical examination, his vitals were normal. There were no lymphadenopathy, oral lesions and hepatosplenomegaly. Neither joint deformity nor synovitis was appreciated. He had tenderness of the right knee on palpation with mild patellar laxity. He had multiple sharply demarcated redorange scaly plaques over the extensor surfaces of the forearms, axillae, knees and trunk. The plaques were nearly confluent on his trunk with the exception of islands of normal skin. The skin of his palms was thick and dry. Bilaterally, his second and third fingernails were hyperkeratotic with pitting and hyperpigmentation.X-rays of the hands and knees were normal. Complete blood count, complete metabolic panel and urinalysis were normal. The erythrocyte sedimentary rate was 15; antinuclear antibody was 1:160 with a nucleolar pattern. Thyroid stimulating hormone, immunoglobulin and complement levels were normal. Rheumatoid factor, hepatitis panel and RPR were negative. He did not respond to the conventional psoriatic treatment with ultraviolet light therapy UVA and UVB. Eventually, a punch biopsy was performed, and a diagnosis of pityriasis rubra pilaris (PRP) was made. The patient was placed on methotrexate therapy and his arthritis and skin lesions subsequently resolved.