Primary Histiocytic Dermatoses

Abstract

The physiology of the histiocyte (macrophage) in health and disease is reviewed briefly. An overview of the so-called primary malignant, pseudomalignant, and benign histiocytic disorders, excluding histiocytosis X, is presented. The malignant histiocytosis with erythrophagocytosis, the pseudomalignant histiocytic diseases (such as sinus histiocytosis with massive lymphadenopathy and regressing atypical histiocytosis), and the solitary lesions with histologic malignant and atypical storiform histiocytosis are described. Two groups of adult histiocytic diseases are reviewed; one is characterized by nonfamilial and familial histiocytic dermatoarthritis and the other by multiple widespread benign lesions, such as xanthoma disseminatum, generalized eruptive histiocytoma, nodular non-X histiocytosis, and various xanthomatous eruptions associated with paraproteinemia. Finally, multiple benign cutaneous histiocytic lesions of childhood, such as juvenile xanthogranuloma and congenital self-healing histiocytosis, are included.

Xanthoma disseminatum: a rare normolipemic xanthomatosis

“…The third group comprises patients with neither abnormal lipoprotein structure and compostion nor underlying systemic disease, where supposed local abnormalities in the dermal tissue may play a role. This includes xanthomas following distinct skin diseases such as erythroderma and epidermolysis bullosa dystrophica as well as hereditary and non-hereditary tendinous and tuberous xanthomas without lipoprotein abnormalities [14,21], papulonodular diffusely distributed cutaneous xanthoma [34,38], generalized eruptive xanthomas [3], juvenile xanthogranuloma [39], and XD [2,19,29,33,36,41]. Due to similiar clinical course and ultrastructural findings in the latter three disorders, generalized eruptive xan- thoma, juvenile xanthogranuloma, and XD may be seen as variants of one disease and by some authors are summarized as non-X histiocytosis [8,10,12,16].…”

Xanthoma disseminatum with respiratory tract involvement and fatal outcome

“…Xanthoma disseminatum is one of several heterogeneous conditions known collectively as the primary histiocytic dermatoses1 2 or cutaneous syndromes of non-X histiocytoses,3 so called to differentiate them from the more recognised condition histiocytosis-X (HX). The condition is caused by a proliferation of non-X histiocytic cells, probably from a macrophage/monocyte origin,4 and was probably first described in 1867 by von Grafe5 and subsequently as a distinct entity by Montgomery and Osterberg in 1938 6.…”

“…8 9 More commonly a persistent mucocutaneous form occurs and treatment with steroids, immunosuppressive agents, and radiotherapy is usually unsuccessful1 2 8-10 despite the successful use of agents such as etoposide, the vinca alkaloids, and methylprednisolone in Langerhans' cell histiocytosis 11. Selective cutaneous lesions in XD may be ablated by dermabrasion or electrodesiccation 1.…”

“…It is characterised by widespread cutaneous xanthomas1 2and may affect the mucous membranes of the mouth and upper respiratory tract, but involvement of the lower respiratory tract is extremely uncommon. We present a case of XD affecting the skin, upper airways, and also the lower respiratory tract leading to death by respiratory failure.…”

Histiocitose cutânea não-Langerhans como causa de diabetes insípido central

“…Trata-se de um grupo heterogêneo de doenças caracterizadas por lesões cutâneas polimorfas histioxantomatosas, podendo ocorrer ou não o acometimento sistêmico (4). Entre as diversas variantes de histiociose não-Langerhans, destaca-se o xantoma disseminado, entidade que em cerca de 40% dos casos associa-se ao diabetes insípido (5). No entanto, existem casos descritos na literatura que sugerem, assim como no presente relato, a associação do DIC com outros tipos de histiocitoses de células não-Langerhans (6).…”