Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature

Author:

Numbere Numbereye1ORCID,Pukhalskaya Tatsiana1,Bowman Blythe2,Campbell Katelynn3,Smoller Bruce4ORCID

Affiliation:

1. Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, New York, USA

2. Dermatology Group of Arkansas, Little Rock, Arkansas, USA

3. Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA

4. Department of Pathology and Laboratory Medicine and Department of Dermatology, University of Rochester Medical Center, 601 Elmwood Ave, Box 626, Rochester, New York, USA

Abstract

Progressive nodular histiocytosis (PNH) is a rare condition characterized by progressive eruption of multiple yellowish-brown papules and nodules on the skin and mucous membranes. We present the case of a 37-year-old Caucasian man with gradually increased appearance of nodular lesions on the forehead and right temple. These lesions were initially diagnosed as xanthomas and did not respond to intralesional injections of triamcinolone. Additional biopsy revealed an intense dermal infiltrate of foamy mononuclear epithelioid cells with a minor admixture of plasma cells, lymphocytes, and scattered multinucleated giant cells. On immunohistochemical staining, the lesional cells were positive for CD163 and CD68 and negative for CD1a, thus confirming a mononuclear-macrophage lineage. The clinical presentation and the histological impression lead to the diagnosis of PNH. This condition could be challenging, mimicking microscopically similar lesions of the non-Langerhans cell histiocytosis group. Although uncommon, PNH stands out due to its clinical and microscopic features and should be taken into consideration in the differential diagnosis of cutaneous histiocytoses.

Publisher

Hindawi Limited

Subject

General Medicine

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