Track: Case Reports

Topic: Lymphoproliferative Disorders

Abstract:

An 81-year-old male with a history of Grover's disease and hypereosinophilic syndrome presented to the dermatology clinic for a 1-week history of a mildly pruritic rash on his arms, legs, and chest. For his Grover’s disease, the patient was previously on methotrexate and dapsone but ultimately switched to and had clearance with dupilumab 12 weeks prior to presentation with new rash. Physical examination revealed erythematous plaques with minimal scale on the bilateral upper and lower extremities. A punch biopsy was performed and revealed a spongiotic dermatitis with intraepithelial T-cell lymphocytes sprinkled in the upper layers of the epidermis and around superficial vessels. The dermis shows a superficial perivascular lymphocytic infiltrate with rare eosinophils. The lymphocytes are small and hyperchromatic. There is dermal fibrosis. CD4: CD8 ratio of 3:1. CD5 is retained. CD7 is mostly lost. CD30 stain few scattered T-lymphocytes mainly the in the upper layers of the epidermis. TCR gene rearrangement was negative for clonality. A diagnosis of dupilumab induced lymphoid reaction was made and the dupilumab was discontinued and started on topical steroids with resolution of the rash. A biopsy several months later demonstrated no intraepithelial lymphocytes. Lymphoid reactions can occur in patients with atopic dermatitis receiving dupilumab. Features of the lymphoid reaction included atypical lymphocytes throughout the upper epidermis, abnormal CD4:CD8 ratio, and CD30 overexpression.1, 2 Lymphoid reactions must be considered for a new/worsening dermatitis while on dupilumab and the medication should be discontinued due to risk of progression to a cutaneous T cell lymphoma.

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Erin Boh, MD, PhD