Eosinophilic dermatosis of hematologic malignancy: Correlation of molecular characteristics of skin lesions and extracutaneous manifestations of hematologic malignancy

Abstract

Background Skin diseases are frequent in patients with chronic lymphocytic leukemia (CLL) and other hematological neoplasias. Eosinophilic dermatosis (ED) of hematologic malignancy has long been considered a nonspecific cutaneous reaction pattern. Recently neoplastic cells have been shown to be present in ED, thus challenging the classification as a nonspecific dermatosis. Methods We report five patients with ED in association with CLL. We further investigated the presence of neoplastic B‐cells in the skin infiltrate by immunohistochemistry and immunoglobulin heavy chain rearrangement and compared these to extracutaneous manifestations of CLL. Results The phenotype of the lymphocytic infiltrate was predominately CD3+ (range: 60%‐90%). CD20+ and CD79a+ lymphocytes were less frequent, accounting for up to 15% (range: absent ‐ 15%). CD23+ lymphocytes represented up to 20% (range: absent ‐ 20%) of the infiltrate. The analysis of the immunoglobulin heavy chain rearrangement in the skin specimens showed clonal rearrangements in 4/5 patients and in three of these four patients clones were identical to extracutaneous CLL manifestations. Conclusion Our data show that neoplastic B‐cells are very frequently found in ED when systematically evaluated. This findings support the hypothesis that leukemic cells play a pathogenetic role in ED of hematologic malignancy.

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

“…One of the most intriguing debates concerns the role of leukaemic cells in EDHM. Many studies of patients with B-cell chronic lymphatic leukaemia with EDHM demonstrated the presence of neoplastic B cells within the inflammatory infiltrate of the skin lesions [110,113,121]. However, this is not a specific finding of EDHM, as these cells can be similarly detected in many inflammatory skin diseases as well as in close proximity of skin cancers occurring in patients with B-cell chronic lymphatic leukaemia [122].…”

“…3 a, b) [ 103 , 112 ]. Additional histopathologic features include dermal oedema, dermal-epidermal detachment, eosinophilic spongiosis and parakeratosis [ 113 ]. In patients presenting with blisters and histological evidence of dermal-epidermal detachment, direct immunofluorescence of perilesional skin or serum tests usually fail to identify autoantibodies against BP180 or other proteins of the dermal–epidermal junction [ 114 ].…”

Hematologic-Related Malignancy-Induced Eosinophilic Dermatosis (He Remained): An eosinophilic dermatosis predominantly associated with chronic lymphocytic leukemia

“…3,4 The world literature on eosinophilic dermatosis of hematologic malignancy (EDHM) was recently reviewed 5 ; to the best of my knowledge, 206 patients with this condition have been described (Table I). [3][4][5][6][7] EDHM has been observed more frequently in men (55% [107 of 193 patients]) than in women (45% [86 of 193 patients]); sex was not provided for 13 patients.…”

“…However, lymphomas, other leukemias, and other hematologic disorders have also been observed in these patients (Table I). [3][4][5][6][7] To date, this condition has not been described in individuals with solid tumors.…”

“…4,5 Researchers have identified that up to 20% of lymphocytes in the infiltrate of lesions were B cells and proposed that these leukemic B cells may have an etiologic role in the pathogenesis of this condition. 6 The dermatosis and associated pruritus often respond to systemic corticosteroids or antineoplastic therapy; however, recurrence of lesions and symptoms are typical. 3,4 Investigators recently treated an 85-year-old man with chronic lymphocytic leukemia-associated EDHM and a numeric rating scale itch intensity of 6 of 10 with standard atopic dermatitis dosing regimen of dupilumab (600 mg induction dose, followed by 300 mg every 2 weeks thereafter).…”