Effective treatment of progressive nodular histiocytosis by intermediate-dose cytarabine: a case report and literature review

Abstract

Background Progressive nodular histiocytosis (PNH) is a rare, clinically distinct non-Langerhans cell histiocytosis, affecting the skin and mucous membranes with no signs of spontaneous regression of the lesions, without any other system involved. Patients typically have hundreds of lesions of superficial xanthomatous papules to nodules and deep larger fibrous nodules. So far, very few universally accepted reports of PNH existed and no effective treatment have been reported to reverse the progressive clinical course. Results Twenty-five PNH cases were identified so far, with a median age of 34 years. 14 patients were male. All patients presented as papules and nodules over the skin and/or mucosal surfaces, including face (39%), fingers or palm (16%), laryngeal (12%) and eyes (8%). 7 cases (28%) reported PNH with systematic diseases. Treatment efficacy using steroids, chemotherapy or surgical excision were disappointing in previous case reports. One patient with laryngeal involvement died due to airway obstruction. In our case, traditional chemotherapy achieved favorable therapeutic effect with intermediate-dose cytarabine confirmed by clinical symptoms of the patient, as well as PET/CT imaging. Conclusions PNH is a rare, proliferative disorder with disfiguring lesions and deteriorative quality of life. Intermediate-dose cytarabine should be considered as a treatment option for PNH patients.