Progressive Histiocytosis: Description of a Case of Slow-Course Non-Langerhans Cell Histiocytosis

Abstract

We describe the case of a 37-year-old male patient who in 1992 started developing a skin eruption characterized by dark red to red-yellow papulonodular lesions that showed no tendency to spontaneous resolution. Visceral organs were not involved and the lipid pattern was basically normal. Histological examination revealed an infiltrate in the mid and upper dermis mainly consisting of mononucleated and multinucleated histiocytes with an abundant eosinophilic cytoplasm and a ground glass appearance, admixed with numerous xanthomatized cells and Touton and foreign-body giant cells. Immunohistochemical studies showed positivity for CD68 and negativity for CD1a and S-100 protein, whereas at the electron microscopy level the only peculiar finding was the presence of many desmosome-like junctions. The authors believe this to be a borderline form between papular xanthoma and reticulohistiocytosis.

Progressive nodular histiocytosis: a case report and literature review

“…Intracytoplasmic granules with a unique and highly complex ultrastructure have also been described 6 . Marked vacuolization was a striking feature in our case and in some others 2,11,12 . The most important EM finding in our patient and other reported cases was the absence of Birbeck granules, which are characteristics of Langerhans cells 2,6,10 …”

Progressive nodular histiocytosis

“…Unlike other patients in the literature [15,[18][19][20] our patients fortunately did not have large or severely disfi guring facial nodules. They did have involvement of the joint fl exures, which has been uncommon in other reports [5,21]. Only one other patient had involvement of the palms and soles [22].…”

Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview

“…In fact, the ECD [6,11,12], xanthogranuloma disseminatum [1,2,3,4,5,13] and juvenile xanthogranulomas [14] may have similar distributions in their CNS lesions. Finally, the XD diagnosis in the present patient is supported by the pattern of skin lesions and the absence of other symptoms/involvement as in the ECD.…”

“…Non-Langerhans histiocytosis (NLH) is a group of rare disorders characterised by the infiltration of lipoid granulomas with CD68+ and CD1a- macrophages in different tissues [1,2]. Among the areas of the central nervous system (CNS) involved in all the subtypes of NLH, infiltration of the pituitary gland is a frequent feature often expressed as diabetes insipidus.…”