Pityriasis rubra pilaris (PRP) is a chronic, inflammatory papulosquamous skin condition of poorly understood pathogenesis; fewer than 5000 individuals are estimated to have the disease in the United States.(1,2) PRP has been associated with infections, autoimmune diseases, and malignancies in case reports, and in a subset of individuals is due to an inherited change in the CARD14 gene. (1) Diagnosis is based on clinical presentation – typically characterized by orange-salmon–colored plaques with scales, follicular hyperkeratosis, erythroderma, and areas of sparing – along with histopathologic correlation. (1) The treatment of PRP is based on clinical experience, with no clinical trials nor FDA-approved treatment currently available.

Paraneoplastic PRP is a rare phenomenon, with only a few dozen cases reported in the literature, and only 3 current cases relating to hematological malignancy. (3) Its pathogenesis is not understood, but may be driven by overproduction in growth factors and immunological cross-reactivity triggered by the underlying cancer. (4) This phenomenon has been previously reported with different hematological malignancies but been poorly characterized given its rarity, with prior cases arising in association with myelodysplastic syndrome (MDS), acute leukemia of undifferentiated type, and chronic lymphoid leukemia (CLL). (5-6)

We conducted a retrospective chart review investigating PRP with hematological malignancy. We queried the Research Patient Data Registry of Mass General Brigham from December 1979 to October 2024 for “Pityriasis Rubra Pilaris” and “Malignant Neoplasms of lymphoid, hematopoietic and related tissue OR Myelodysplastic syndromes” in any clinical documentation. Patients that did not have either diagnosis were excluded.

Forty-eight patients were identified through the query, of which 16 were ultimately included. The majority were males (62.5%), white (93.8%), and non-Hispanic (68.8%). Of the 16 patients, 6 (37.5%) had drug-induced PRP related to malignancy treatment. 4 of these patients had CLL, 1 had ALL, and 1 had non-Hodgkin's lymphoma. The ALL patient was related to ponatinib, the non-Hodgkin's lymphoma case was related to copanlisib, 2 CLL patients were related to duvelisib, and 2 CLL were related to idealisib.

Of the 16 patients, 5 (31.3%) had evidence of possible paraneoplastic occurrence, arising in the context of non-Hodgkin's lymphoma (n=2), CLL (n=2), and multiple myeloma (n=1). Average age at PRP diagnosis for paraneoplastic cases was 65.8 years. Distribution of PRP involvement included head/neck (4, 80.0%), upper limbs (3, 60.0%), trunk (5, 100.0%), lower limbs (5, 100.0%), and genital region (1, 20.0%). All 5 (100%) had a body surface area (BSA) of >30%. All 5 occurred after hematological diagnosis, with an average latency of 21.6 months. With regards to paraneoplastic treatment, topical treatment included corticosteroids, emollients, topical urea, retinoids, and fluorouracil. Systemic treatments included methotrexate, systemic corticosteroids, and phototherapy.

Our study explored PRP arising in association with hematological malignancies, contributing to the limited literature of paraneoplastic PRP and PRP with this malignancy subtype. A substantial proportion of patients had drug-induced PRP, with a predominance of CLL and treatment with P13K inhibitors. All patients were characterized by widespread skin disease with >30% BSA and had trunk and leg involvement. All occurred after malignancy diagnosis with an average latency of two years, but some cases occurring within a few months of malignancy. Specialists should maintain a high index of suspicion for PRP as a paraneoplastic signal or drug-related adverse event. Future studies are needed to elucidate such presentations and guide management.

References:

  • Wang D, et al. Am J Clin Dermatol. 2018

  • U.S. Department of Health and Human Services. Pityriasis rubra pilaris - about the disease. Genetic and Rare Diseases Information Center.

  • Davis et al. JAAD Case Rep.

  • Chung VQ, et al. J Am Acad Dermatol. 2006

  • Kwak et al. Am J Hematol. 2021

  • Vance, et al. Advances in Skin & Wound Care 2022.

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2025
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