Efficacy of vinblastine and prednisone in multicentric reticulohistiocytosis with onset in infancy

Abstract

Multicentric reticulohistiocytosis (MRH) is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. MRH can coexist with various autoimmune disorders, tuberculosis, and malignancy. It usually occurs in the elderly and is very rare in children. This is probably the first case in which disease manifestation appeared in infancy in the form of skin lesions. The patient had recurrent ulceration of cutaneous lesions, which is unusual in MRH. Early diagnosis and aggressive treatment are essential to prevent progressive irreversible course and development of arthritis mutilans. Various drugs, such as steroids, nonsteroidal anti-inflammatory drugs, immunosuppressants, interleukin inhibitors, and tumor necrosis factor-α antagonist, have been tried with variable responses. The present case responded well to vinblastine and steroid, which have not been reported previously. Here, we document a case of MRH with early onset in infancy along with role of vinblastine and prednisone in its treatment.