RESUMEN
Primary cutaneous lymphomas represent a diverse spectrum of T-cell and B-cell lymphomas with their primary skin manifestation. Among these, mycosis fungoides (MF) and Sézary syndrome (SS) represent classic forms of cutaneous T-cell lymphomas (CTCLs). This report details the case of a 67-year-old female who presented with longstanding pruritic skin lesions, initially misdiagnosed and managed as eczema. The diagnostic process ultimately revealed the presence of Sézary cells in the peripheral blood smear (PBS). The SS diagnosis was confirmed based on CD4 positivity and CD7 negativity as determined by flow cytometry. The disease was staged as IVA1 (T2N0M1B2). The patient exhibited partial improvement with oral corticosteroid therapy. This report underscores the critical importance of integrating clinical evaluation and blood findings to distinguish between MF and SS. The progression of a circulating clone signals a poor prognosis, requiring surveillance and consideration of targeted therapies to enhance patient outcomes and improve their quality of life. Early detection remains paramount in the management of these rare cutaneous lymphomas, which are associated with unique therapeutic challenges.
RESUMEN
Hereditary angioedema (HAE) is a rare condition characterized by recurrent episodes of angioedema without urticaria or pruritus. Untreated angioedema can cause significant work absenteeism and, in rare cases, be lethal due to laryngeal involvement and suffocation. The authors report a case of a patient with laryngeal involvement who was unaware of the severity of their condition. Effective medical training in patient empowerment is essential, and it is an irreplaceable element in healthcare, as it contributes to therapeutic success.