629603 case-report2016
CMSXXX10.1177/1203475416629603Journal of Cutaneous Medicine and SurgerySchreiber et al
Case Report
Generalized Recalcitrant Pruritus as the Presenting Manifestation of Hypereosinophilic Syndrome
Journal of Cutaneous Medicine and Surgery 1–3 © The Author(s) 2016 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1203475416629603 jcms.sagepub.com
Ariane Schreiber1, Martin LeBlanc1, Marie-Marthe Thibeault1, and Steve Mathieu1
Abstract Background and Objective: Pruritus is a frequent occurrence in dermatology, and investigation is often unrevealing. The authors report the case of a 65-year-old man presenting with generalized recalcitrant pruritus as the presenting manifestation of hypereosinophilic syndrome. Methods and Results: A 65-year-old man developed intractable pruritus. Results of polymerase chain reaction clonal rearrangement were positive and led to the diagnosis and treatment. The patient also developed massive pulmonary embolism, which can be caused by chronic eosinophilia. Conclusions: This case highlights the importance of investigating patients with pruritus and unexplained persistent eosinophilia. Hypereosinophilic syndrome must be included in the differential diagnosis, which in this case presented initially as intractable pruritus. Polymerase chain reaction clonal rearrangement was key in reinforcing the diagnosis. Keywords hypereosinophilic syndrome, generalized pruritus, eosinophilia, pulmonary embolism, PCR clonal rearrangement, hypereosinophilic syndrome lymphocytic variant
Introduction Hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent nonreactive peripheral blood hypereosinophilia (HE) with eosinophilia-associated organ damage. Organ damage and clinical symptoms are presumed to be due to eosinophilic infiltration and toxic mediators. Recognition of the different HES phenotypes is critically important, as this can orient treatment and outcomes.
Case Report We report the case of a 65-year-old man presenting with generalized recalcitrant pruritus. The patient had prior membranous glomerulonephritis with now stable chronic kidney failure and mild proteinuria and steroid-induced diabetes. He had been taking systemic steroids for his chronic pruritus for nearly 3 years. The systems review was also positive for hypertension, dyslipidemia, and tobacco use. Medications included low prednisone doses ranging from 5 to 10 mg daily or every other day, which is ongoing; insulin; hydroxyzine; ezetimibe; amlodipine; candesartan; metoprolol; simvastatin; and esomeprazole. Physical skin examination included xerosis, excoriations, and dermographism. There was no lymphadenopathy or hepatosplenomegaly. The first blood
count revealed eosinophilia (white blood cell count 5.4 × 109/L, eosinophil count 1.00 × 109/L). The eosinophilia was variable, with a maximum of 2.754 × 109/L (normal range,
CMSXXX10.1177/1203475416629603Journal of Cutaneous Medicine and SurgerySchreiber et al
Case Report
Generalized Recalcitrant Pruritus as the Presenting Manifestation of Hypereosinophilic Syndrome
Journal of Cutaneous Medicine and Surgery 1–3 © The Author(s) 2016 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1203475416629603 jcms.sagepub.com
Ariane Schreiber1, Martin LeBlanc1, Marie-Marthe Thibeault1, and Steve Mathieu1
Abstract Background and Objective: Pruritus is a frequent occurrence in dermatology, and investigation is often unrevealing. The authors report the case of a 65-year-old man presenting with generalized recalcitrant pruritus as the presenting manifestation of hypereosinophilic syndrome. Methods and Results: A 65-year-old man developed intractable pruritus. Results of polymerase chain reaction clonal rearrangement were positive and led to the diagnosis and treatment. The patient also developed massive pulmonary embolism, which can be caused by chronic eosinophilia. Conclusions: This case highlights the importance of investigating patients with pruritus and unexplained persistent eosinophilia. Hypereosinophilic syndrome must be included in the differential diagnosis, which in this case presented initially as intractable pruritus. Polymerase chain reaction clonal rearrangement was key in reinforcing the diagnosis. Keywords hypereosinophilic syndrome, generalized pruritus, eosinophilia, pulmonary embolism, PCR clonal rearrangement, hypereosinophilic syndrome lymphocytic variant
Introduction Hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent nonreactive peripheral blood hypereosinophilia (HE) with eosinophilia-associated organ damage. Organ damage and clinical symptoms are presumed to be due to eosinophilic infiltration and toxic mediators. Recognition of the different HES phenotypes is critically important, as this can orient treatment and outcomes.
Case Report We report the case of a 65-year-old man presenting with generalized recalcitrant pruritus. The patient had prior membranous glomerulonephritis with now stable chronic kidney failure and mild proteinuria and steroid-induced diabetes. He had been taking systemic steroids for his chronic pruritus for nearly 3 years. The systems review was also positive for hypertension, dyslipidemia, and tobacco use. Medications included low prednisone doses ranging from 5 to 10 mg daily or every other day, which is ongoing; insulin; hydroxyzine; ezetimibe; amlodipine; candesartan; metoprolol; simvastatin; and esomeprazole. Physical skin examination included xerosis, excoriations, and dermographism. There was no lymphadenopathy or hepatosplenomegaly. The first blood
count revealed eosinophilia (white blood cell count 5.4 × 109/L, eosinophil count 1.00 × 109/L). The eosinophilia was variable, with a maximum of 2.754 × 109/L (normal range,
