Severe Mucha–Habermann‐Like Ulceronecrotic Skin Disease in T‐Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant

Abstract

A 5-year-old girl with T-cell acute lymphoblastic leukemia (T-ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha-Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. This report describes a severe case of FUMHD-like eruption associated with clonal leukemic cells that improved with basiliximab, suggesting anti-CD25 therapy as a novel treatment for ulceronecrotic skin disease in the setting of high interleukin-2 levels.

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

“…Due to the rarity of the disease, most of the publications on FUMHD are individual case reports or small case series. 1, An infective or non-infective trigger 1 may lead to development of a multisystem inflammatory condition with CD8+ lymphocytic infiltration 1,[4][5][6] of the skin and a subsequent characteristic histological picture of interface dermatitis. Reported infective triggers were, e.g., Epstein-Barr virus (EBV), 15 chickenpox, 25 measles vaccination, 39 or chronic tonsillitis.…”

“…21 Clinically, FUMHD may initially mimic viral illnesses or Kawasaki syndrome. 45 Stevens-Johnson syndrome 27 and malignant conditions involving clonal T-cell proliferation 1,4,6,29,40,47 are other potential differential diagnoses of FUMHD. Treatment options comprise topical or systemic modulation or suppression of the immune system.…”

“…2 Systemic treatment includes steroids, methotrexate (MTX), and intravenous immunoglobulins. 1 More recently, targeted immunotherapy has emerged using antibodies with infliximab 1,4,5 or basiliximab, 6 blocking TNF-alpha 1,4,5 or interleukin-2 receptor 6 and thus inhibiting the involved T-cell populations. However, because of the rarity of the disease, data availability on treatment is poor.…”

A Bright Horizon: Immunotherapy for Pediatric T-Cell Malignancies

“…The addition of basiliximab improved her skin findings and allowed for intensification of therapy and bone marrow transplant. Given this case, there may be opportunity for the use of anti-CD25 agents in combination therapy for T-ALL [ 76 ]. To our knowledge, there are no open trials using this drug for T-cell malignancies.…”

Combination therapy with cyclosporine and intravenous immunoglobulin for febrile ulcerative Mucha‐Habermann disease

“…1 Nevertheless, reported treatments are heterogeneous and consist mainly of immunosuppressive therapies, such as systemic steroids, methotrexate, and IVIG in different combinations. [1][2][3][4][5][6] To the best of the authors' knowledge, this is the first report on the successful association of IVIG with cyclosporine in the management of FUMHD. The incorporation of a short course of IVIG to the therapeutic approach was effective in inducing disease remission.…”