ABSTRACT
In the pediatric and adolescent age group, primary cutaneous lymphomas are rare, especially cutaneous B-cell lymphomas. According to the World Health Organization, the three main subtypes of primary cutaneous B-cell lymphomas are primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type. We present an extraordinary case of PCFCL arising in a 16-year-old female, only the sixth case of PCFCL to be reported in the literature in a patient less than 20 years old. Although PCMZL was considered in this case, the finding of lambda light chain restriction in the BCL-6 and CD10 positive population of lymphocytes established the diagnosis of primary cutaneous follicle center lymphoma. Not many data currently exist on the prognosis of PCFCL in young individuals, but adult PCFCL has a good prognosis with an indolent course and 5-year survival rates over 95%. Because of its uncommon manifestation in young patients, the diagnosis of PCFCL is often delayed or missed. This case is presented to raise awareness of PCFCL in the pediatric/ adolescent population and to contribute to the ongoing research of this condition.
Subject(s)
Lymphoma, B-Cell, Marginal Zone/pathology , Lymphoma, Follicular/diagnosis , Lymphoma, Large B-Cell, Diffuse/pathology , Pseudolymphoma/pathology , Skin Neoplasms/pathology , Adolescent , Awareness , Biopsy , Child , Female , Humans , Immunohistochemistry , Lymphoma, B-Cell, Marginal Zone/diagnosis , Lymphoma, Follicular/metabolism , Male , Prognosis , Pseudolymphoma/diagnosis , Young AdultABSTRACT
This report presents two cases of patients with long-standing, treatment-resistant Hailey-Hailey disease (HHD) who experienced significant symptom relief through a combination therapy of oral naltrexone and dupilumab injections. The therapeutic potential of targeting the Th2 pathway and Ca2+ signaling with dupilumab in managing HHD manifestations is highlighted. The findings suggest that Th2 blockade with dupilumab, in conjunction with naltrexone, effectively controls recalcitrant HHD, indicating a role of cytokine response in altering disease pathogenesis. This case contributes to the growing body of literature on biologic treatments for HHD and suggests avenues for further research in HHD management.