Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion

Abstract

Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.

Isolated hypothalamic-pituitary langerhans’ cell histiocytosis in female adult

“…It has been referred in the literature as Histiocytosis X, Letterer-Siwe disease, Hand-Schuller-Christian syndrome, eosinophilic granuloma of bone, self-healing reticulohistiocytosis, and more recently, Langerhans' cell histiocytosis. [1][2] LCH can affect bones, skins, lungs, and any other organs, [3][4][5][6] and it may involve a single organ or system, or involve multiple organs or systems. Clonal proliferative disorder via BRAF V600E mutation, MAP2K1 mutation, or activation of MAPK/ ERK has been considered as possible pathogenesis for LCH.…”

A Case of Neurodegenerative Langerhans Cell Histiocytosis

“…Common skin manifestations are vesiculo-pustules, erythematous-brownish papules, ulcerous-crusty plaques, and a rash resembling recalcitrant seborrheic dermatitis, the last one being more erosive, ulcerative and frequent in MS-LCH 14,16,17 . When they regress, they leave behind hypochromia, atrophy, and alopecia.…”

“…When they regress, they leave behind hypochromia, atrophy, and alopecia. LCH can manifest with various and extensively investigated symptoms, diagnosed late, from a skin lesion 16 .…”