Cutaneous Histiocytosis Syndromes

Abstract

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.

Congenital self-healing non-Langerhans cell histiocytosis

“…It was not clear if there was any association with the histiocytic skin eruption. Pneumonia has been described in histiocytosis X [14]. Clinically our patient also differs from the cases of self-healing reticulohistiocytosis by striking and plentiful erosive or vesiculobullous lesions and only one large nodule in the vulval area.…”

Juvenile xanthogranuloma: a rare benign histiocytic disorder

“…Spontaneous regression usually occurs within 6 months to 3 years [5, 35]. Recurrence of JXG has been rarely reported.…”

Diffuse xanthogranulomatous dermatitis and systemic Langerhans cell histiocytosis: A novel case that demonstrates bridging between non-Langerhans cell histiocytosis and Langerhans cell histiocytosis

“…Controversy continues as to whether they are reactive or neoplastic; however, the former hypothesis is currently favored. 4,[18][19][20][21][22]25 The case presented herein is unusual because it does not easily fit into either the non-LCH or LCH category. Initially the eruption was postulated to be reactive to some component in the hip transplant, but a negative lymphocyte transformation assay result almost eliminates that possibility.…”

“…The aforesaid conditions not only differ in their clinical presentation, they also exhibit slightly different histologic findings (Table I), [2][3][4][5][6]11,13,[16][17][18][19][20][21][22][23][24] and immunohistochemical staining patterns (Table II). [3][4][5][6][7]11,14,[16][17][18]22,25 On the other hand, despite vast clinical differences, all nonpulmonary LCH are composed of a clonal proliferation of LCs.…”