Unraveling Histiocytic Disorders: Two Cases From Resource-Limited Settings

Histiocytic disorders include a range of uncommon illnesses marked by the buildup of cells that have developed into macrophages, dendritic cells, or monocytes in diverse tissues and organs. Over 100 distinct subtypes have been documented, exhibiting a diverse array of clinical symptoms, presentations, and histologic features that can be confused with other clinical conditions leading to delayed diagnosis. They affect both children and adults, generating a variety of clinical symptoms that can be limited to one position, numerous areas within one system, or affect many systems in the body. We provide our experiences handling two cases of histiocytic disease in underdeveloped countries where targeted treatment choices are unavailable. The first patient was a 32-year-old female with polyuria, skin lesions, and macroadenoma on MRI. Histopathological and immune-histochemical assessment of skin lesions confirmed the diagnosis of Langerhans cell histiocytosis. The patient responded well to steroids and oral and intrathecal methotrexate. The second patient was a 38-year-old male with recurrent skin lesions followed by retro-orbital discomfort and proptosis. The retro-orbital mass was surgically excised followed by a recurrence a few months later. Histopathological and immune-histochemical assessment confirmed the diagnosis of extranodal Rosai-Dorfman disease. He showed a partial response to methotrexate followed by a remarkable response to rituximab. Because of the wide variety of clinical symptoms, the diversity of experts engaged in assessing and treating these patients, and the scarcity of cases accessible, there is still a need to develop an evidence-based technique for evaluating and treating this complicated disease, especially in resource-limited settings.