Eosinophilic Fasciitis as a Manifestation of a Cutaneous T-Cell Lymphoma Not Otherwise Specified

Abstract

Eosinophilic fasciitis (EF) is a rare entity characterized by symmetrical and painful thickness and induration of the skin, especially localized on forearms and thorax and generally accompanied by eosinophilia. Although several reports indicate the relationship between EF and hematological disorders such as aplastic anemia, polycythemia vera, or myelomonocytic leukemia, the association with lymphomas is extremely rare. Only a few cases of EF have been previously described preceding or concomitant to the Hodgkin disease, peripheral T-cell lymphoma, B-cell lymphoma, and mycosis fungoides. We report for the first time a 76-year-old man with an EF associated with a peripheral T-cell lymphoma not otherwise specified. We review the relationship between both conditions. In conclusion, we present a unique case of EF as a manifestation of a T-cell lymphoma not otherwise specified. The present case demonstrates the importance of clinical and radiological studies in those cases of EF to rule out a visceral, lymph node, or cutaneous lymphoma.

Review of Peripheral Blood Eosinophilia: Workup and Differential Diagnosis

“…Cutaneous T-cell lymphomas (CTCL) are characterized by clonal accumulations to T-cells in the skin [13]. This disorder is associated with erythroderma, severe pruritis, and lymphadenopathy, though symptoms can be elusive and a strong index of suspicion must be maintained to identify this condition [5].…”

“…This disorder is associated with erythroderma, severe pruritis, and lymphadenopathy, though symptoms can be elusive and a strong index of suspicion must be maintained to identify this condition [5]. CTCL is thought to induce eosinophilia due to T-helper 2 clones increasing production of cytokines specific to eosinophil proliferation [13]. CTCL tumor lines are noted to spontaneously produce IL-5 (and other cytokines), which may cause the peripheral eosinophilia in the disease [14].…”

“…In one article, 33 of 52 cases of EF presented with peripheral eosinophilia [88]. The etiology of EF varies, and reported cases have included medication exposure, autoimmune diseases, infection, initiation of hemodialysis, and even hematologic malignancies [13,88]. Definitive diagnosis requires full thickness biopsy and histopathological examination [89].…”

Eosinophilic Fasciitis with Hypereosinophilia as the Initial Clinical Manifestation of Peripheral T-Cell Lymphoma, Not Otherwise Specified

“…While autoimmune disorders and certain hematological neoplasms, including myelomonocytic leukemia, chronic lymphocytic leukemia, and malignant lymphoma, are known to trigger the onset of EF ( 4), most cases are idiopathic with no clear association. Indeed, there are few reports of T-cell lymphoma preceded or accompanied by EF (6)(7)(8)(9). According to what reports do exist, EF can occur in association with T-cell lymphoma, at either the same time as the lymphoma or months or more in advance.…”

Eosinophilia in Rheumatologic/Vascular Disorders

“…63 Associations with other hematological disorders have been reported anecdotally. [75][76][77][78][79][80][81][82][83][84][85][86][87] Pathophysiology…”