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 histiocytic neoplasm affecting the skin and mucous membranes with no signs of spontaneous regression of the lesions. Patients typically have hundreds of lesions of superficial xanthomatous papules to nodules and deep larger fibrous nodules, which can be life-threatening if laryngeal mucosa was involved. So far, few cases of PNH have been reported and no effective treatment has been shown to reverse the progressive clinical course.

Case presentation

Here we describe a case of PNH presenting as diffuse, progressively enlarging papules, nodules and even leonine appearance in a young woman. The diagnosis was confirmed by histological and immunohistochemical findings. Next-genera

SUBMITTER: Wang JN 

PROVIDER: S-EPMC8430172 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature