Disseminated cutaneous leishmaniasis in a patient with Sézary syndrome
Authors: Priscila Giavedoni, Ignasi Pau-Charles, José M Mascaró, Mercé Alsina-Gibert, Adriana García-Herrera, Teresa Estrach
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: A 66-year-old Caucasian man was diagnosed with Sézary syndrome (SS) in 2007. After chlorambucil, prednisone, and bexarotene treatment, only a partial response was achieved. In May 2012, he was hospitalized with necrotizing pneumonia. He presented with typical features of SS: erythroderma, pruritus, and enlarged lymph nodes. Laboratory studies showed total leukocytes 8.2 × 109/L (normal range: 4-11 × 109/L), 84.5% neutrophils, and 8.2% lymphocytes, of which 35% were Sézary cells. Increased lactate dehydrogenase, 520 U/L (normal range: 250-450 U/L), was also seen. The patient had numerous asymptomatic hyperpigmented macules on his trunk and limbs, which appeared over the previous 6 months. Histopathologic examination of lesions showed acanthosis, epidermotropism, and a mild perivascular atypical lymphoid infiltrate in the upper dermis. Lymphocytes were hyperchromatic, with indented cerebriform nuclei, and expressed CD2, CD3, CD4, and CD5, with partial loss of CD7. Five months later, the patient was readmitted with fever, malaise, and progression of skin lesions. Cutaneous examination revealed numerous widespread erythematous papules and nodules (Fig 1). A skin biopsy showed a dense lymphohistiocytic infiltrate in the upper and middle dermis with intracellular microorganisms suggestive of Leishmania amastigotes (Fig 2). Disseminated cutaneous leishmaniasis was diagnosed and confirmed by positive polymerase chain reaction for Leishmania infantum. Bone marrow aspirate was negative for Leishmania. Liposomal amphotericin B (4 mg/kg/day intravenously) was given for 10 days without clinical improvement, so treatment was switched to meglumine antimonate (20 mg/kg/day intravenously). After the first dose, the patient's condition worsened; fever and flare of cutaneous lesions developed. Jarisch-Herxheimer reaction was diagnosed and the patient was treated with prednisone (1 mg/kg/day). Myalgias, impaired renal function, and leucopenia developed. Even though treatment was discontinued 13 days later, skin lesions progressively improved. Two months later the patient died due to progression of the lymphoproliferative process.Fig 2Disseminated cutaneous leishmaniasis in patient with Sézary syndrome. Large numbers of Leishmania amastigotes within the macrophages (arrows). (Hematoxylin and eosin; original magnification: ×640.)View Large Image Figure ViewerDownload Hi-res image Download (PPT) SS is a primary cutaneous T-cell lymphoma associated with dysregulation of both cellular and humoral immunity.1Olsen E.A. Rook A.H. Zic J. Kim Y. Porcu P. Querfeld C. et al.Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC).J Am Acad Dermatol. 2011; 64: 352-404Abstract Full Text Full Text PDF PubMed Scopus (132) Google Scholar Staphylococcus aureus has been identified as the most common pathogenic organism in cutaneous infections and bacteremias in patients with SS. Disseminated herpes infections are also frequently found with progressive cell-mediated immunity decline.1Olsen E.A. Rook A.H. Zic J. Kim Y. Porcu P. Querfeld C. et al.Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC).J Am Acad Dermatol. 2011; 64: 352-404Abstract Full Text Full Text PDF PubMed Scopus (132) Google Scholar Leishmaniasis due to L. infantum is endemic in Spain, both in immunocompetent and in immunosuppressed patients, and is transmitted by mosquitoes.2Cortes S. Chicharro C. Cruz I. Cristovão J.M. Cañavate C. Campino L. Genetic diversity of human zoonotic leishmaniasis in Iberian peninsula.Zoonoses Public Health. 2011; 58: 234-237Crossref PubMed Scopus (6) Google Scholar Disseminated cutaneous leishmaniasis is rarely seen in SS. To our knowledge, there is only one report of cutaneous leishmaniasis in a patient with SS.3Mahé A. Gessain A. Huerre M. Pratlong F. Dedet J.P. Erythrodermic diffuse cutaneous leishmaniasis with Sézary syndrome.Lancet. 1996; 348: 405-406Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Cutaneous leishmaniasis caused by dermotropic species may disseminate in immunosuppressed patients and present different clinical characteristics. Poor response to treatment is common and relapses are frequent,4Van Griensven J. Carrillo E. López-Vélez R. Lynen L. Moreno J. Leishmaniasis in immunosuppressed individuals.Clin Microbiol Infect. 2014; 20: 286-299Abstract Full Text Full Text PDF PubMed Scopus (199) Google Scholar depending on the Leishmania strain.5Masmoudi A. Hariz W. Marrekchi S. Amouri M. Turki H. Old World cutaneous leishmaniasis: diagnosis and treatment.J Dermatol Case Rep. 2013; 7: 31-41Crossref PubMed Scopus (64) Google Scholar Differential diagnosis of disseminated cutaneous eruptions in immunocompromised patients must include other infectious diseases such as leishmaniasis, histoplasmosis, sporotrichosis, and paracoccidioidomycosis. Histopathologic examination is necessary for the diagnosis of cutaneous leishmaniasis, although the parasites may not be found. If clinical suspicion is high, polymerase chain reaction may be useful.5Masmoudi A. Hariz W. Marrekchi S. Amouri M. Turki H. Old World cutaneous leishmaniasis: diagnosis and treatment.J Dermatol Case Rep. 2013; 7: 31-41Crossref PubMed Scopus (64) Google Scholar