Nodal anaplastic large cell lymphoma with lymphomatoid papulosis following treatment of initially presumed atopic dermatitis with dupilumab: A case report

Dear Editor, A 38-year-old Japanese man with a three-year history of erythematous plaques on his extremities consulted our outpatient clinic. He had received topical steroid therapy for atopic dermatitis (AD) for 3 years at a private clinic and was administered dupilumab 1 year before his arrival. In addition, he had developed steroid-resistant disseminated papules half year before his initial visit. On physical examination at his initial visit, dome-like, crusted nodules were seen on his back and right breast (Figure 1A), disseminated red papules (Figure 1B) were present on his trunk and buttock, and he had severe dry skin with itch. An immunological study showed high serum levels of total IgE (11,499 IU/mL) with multiple specific antigens (class 6 for house dust, cedar, mite, etc.), and increased levels of TARC (2254 pg/mL) and soluble IL-2R (620 IU/mL). Positron emission tomography (PET)-CT at the initial visit showed no evidence of lymph node or visceral involvement. A biopsy specimen from papule on the trunk showed dense infiltration of atypical lymphocytes from the superficial dermis with glenz zone to deep dermis but without epidermotropism (Figure 1C, E). Immunohistochemical staining showed that these atypical lymphocytes were positive for CD2, CD4, and CD30 (Figure 1D) and negative for CD3, CD5, CD7, CD8, and anaplastic lymphoma kinase (ALK). On the other hands, a biopsy specimen from erythematous plaque showed spongiotic and perivascular dermatitis (Figure 1F) without CD30 positive atypical lymphocytes. The initial diagnosis was CD30+ lymphomatoid papulosis (LyP), for which topical