Progressive nodular histiocytosis

Abstract

SummaryProgressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis (NLCH). It can be distinguished from other types by the occurrence of yellow to red-brown papules and nodules measuring a few centimeters in size. Histologically the nodules represent spindle-cell xanthogranulomas. We report on two new cases of PNH with novel clinical symptoms. In the first case, the patient had microcytic anemia due to massive iron deposition in the nodules and a progressively worsening limp due to involvement of the soles. The complex therapeutic challenge was effectively managed by successive surgical ablation of all visible lesions. The second patient had epibulbar infiltrates. The clinical variability of PNH leads to considerable overlap with other forms of NLCH.

Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report

“…Mucosal involvement, primarily seen in xanthoma disseminatum, can occur in any form. [8] Laryngeal affection encountered in our patient is considered an unusual feature. Indirect laryngoscopy showed nodularity and telangiectasia over vocal cords.…”

“…Mucosal involvement has been occasionally reported, including the publication by Taunton and colleagues who were the first to describe a patient of PNH with conjunctival lesions. [8] Karimi et al . and Glavin et al .…”

“…The presence of histiocytic infiltrates features in all forms of histiocytoses and is characterized by spindle-shaped, vacuolated, stellate, and foamy cells, all of which express immunohistochemical macrophage markers such as CD68. [8]…”

A Case with Juvenile Xanthogranuloma and Progressive Nodular Histiocytosis Overlap

“…Progressive nodular histiocytosis has been said to be a distinct entity because of clinical features including the simultaneous presence of two different lesions: superficial, yellow‐reddish papules up to 10 mm in diameter and dermal nodules 1 to 5 cm in diameter, more common on the trunk . Histopathology of papular lesions in PNH usually demonstrates a dermal infiltrate composed of macrophages with abundant vacuolated, clear cytoplasm and a variable number of multinucleated Touton giant cells.…”

“…Progressive nodular histiocytosis has been said to be a distinct entity because of clinical features including the simultaneous presence of two different lesions: superficial, yellow-reddish papules up to 10 mm in diameter and dermal nodules 1 to 5 cm in diameter, more common on the trunk (3,4). Whereas skin lesions in JXG tend to disappear in most patients, lesions in PNH usually progress and do not resolve, although two instances of spontaneous regression of PNH have been reported (6,7).…”