Progressive nodular histiocytosis: a case report with literature review, and discussion of differential diagnosis and classification

Abstract

Histiocytoses are a heterogeneous group of disorders which are difficult to categorize because of overlapping clinical and microscopic features, and incompletely understood mechanisms of cellular origin and triggers of proliferation. Progressive nodular histiocytosis (PNH) is a rare non‐Langerhans cell histiocytic (NLH) disorder. We present the case of a 34‐year‐old woman with PNH, who has involvement of the back, head and neck, face, and uniquely, extensive involvement of the posterior pharynx and larynx, which has shown progressive worsening and deformity over a 7‐year course. Clinically it is best classified as PNH, although it microscopically closely resembles juvenile and adult xanthogranulomatosis. This study reinforces the concept that the NLH is a closely related group of disorders, possibly representing a spectrum of a single underlying process.

Nonlipidized juvenile xanthogranuloma: An unusual variant with a potential diagnostic pitfall

“…Histologically, it is characterized as a well circumscribed nodule composed predominantly of spindle-shaped histiocytes arranged in a storiform pattern, intermingled with mononuclear cells showing vacuolated, xanthomatized, scalloped, or oncocytic morphologies and Touton giant cells. Mitotic activity is generally absent [20,21]. Although nonneural granular cell tumor shares some histopathological and IHC features with NJXG, the former is clearly a distinctive entity.…”

Reddish-Brown Nodules and Papules in an Elderly Man

“…2,3 Lesions tend to be diffuse and involve the face, typically with sparing of the mucous membranes, although some studies have noted involvement of oral and conjunctival mucosae. [3][4][5] Most patients with PNH have no systemic involvement, but there have been a few reported cases of associated malignancies. 5,6 The clinical course is characterized by the progressive appearance of new lesions.…”

“…One option is surgical excision, but recurrences are not uncommon. 3 One case report described carbon dioxide laser treatment of PNH nodules, but there was recurrence of lesions over the scar tissue. 6 Systemic vincristine combined with prednisolone treatment has been reported to be unsuccessful.…”

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

“…PNH is regarded as a solely cutaneous disease without a tendency for extracutaneous involvement. In general, PNH is not known to be associated with specific systemic disorders or syndromes,[7] hence systemic screening may be indicated only in the presence of symptoms. Mucosal involvement, primarily seen in xanthoma disseminatum, can occur in any form.…”

“…claim to have documented the first case with extensive laryngeal and pharyngeal involvement causing dyspnea and dysphagia due to partial airway obstruction. [17] Hence after extensive search in the literature, only one previous case of PNH with laryngeal involvement could be identified (to the best of our knowledge). 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.…”