Paraneoplastic Pityriasis Rubra Pilaris Preceding Leukemia

Abstract

Pityriasis rubra pilaris (PRP) is a rare, chronic papulosquamous disorder that presents with scaling plaques, palmoplantar keratoderma, and keratotic follicular papules. Typically, there are distinctive unaffected areas referred to as "islands of sparing." Pityriasis rubra pilaris has been associated with various immunodeficient states and malignancies.The authors conducted a literature review using MEDLINE, PubMed, and Google Scholar, documenting all known cases of PRP associated with malignancy; 15 cases were found in the literature. They also present the case of a 49-year-old White man who, prior to referral to dermatology, was seen in urgent care for widespread pruritic rash. Physical examination in the dermatology clinic revealed confluent, scaly erythematous papules coalescing into plaques with island of sparing involving the trunk and upper and lower extremities. Bilateral palms and soles showed hyperkeratosis with fissuring. He was diagnosed with PRP after punch biopsy and began a new course of topical corticosteroid therapy. Hematology was consulted because of abnormal complete blood count results, and he was subsequently diagnosed with chronic lymphoid leukemia.Treatment of PRP is largely based on clinical experience and may involve corticosteroids, immunomodulators, or biologic therapy. The relationship between PRP and malignancy is unknown. Current theories postulate it may be driven by tumor production of functional peptides or antigen cross-reactivity between cancer cells and the skin. This is the second reported case of PRP as a manifestation of leukemia, and the first of chronic lymphoid leukemia. Although not yet understood, the documented relationship between PRP and malignancy prompts screening for cancer in all patients with new-onset PRP.