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Neonatal erythroderma
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Aparna Palit, Arun C. Inamadar
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder that may present at birth or in the early neonatal period with diffuse involvement of the skin [24]. Cutaneous involvement may be part of single-system skin-only LCH (SS-LCH), also known as congenital self-healing reticulohistiocytosis (CSHRH) or Hashimoto-Pritzker disease [24]. Otherwise it may be part of multisystem LCH (MS-LCH), also known as Letterer-Siwe disease [24].
A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
P. Daniel Nicholas, Ian Garrahy
Langerhans cell histiocytosis (LCH) is a rare malignancy characterized by lesions of monoclonal histiocytes capable of infiltrating almost any organ system. This disease is also known by its previous monikers, Histiocytosis X and eosinophilic granulomas, or by its eponymous labels, Letterer–Siwe disease, Hand–Schüller–Christian disease, and Hashimoto–Pritzker disease. Even its own name is a potential misnomer, as recent studies have suggested that the malignant cell is actually a dendritic precursor from the bone marrow instead of a transformed Langerhans cell from the epidermis [1]. Largely a disease of children, its incidence in adults approximates 1–2 cases per million with a mean age of 35 ± 14 years at diagnosis [2]. LCH is classified based on organ involvement into single-system (usually bones or lungs in adults) versus multisystem disease. The latter is further stratified into low and high-risk groups, distinguished by the involvement of risk organs (the hematopoietic system, liver, and spleen) which carries worse prognoses [3,4].
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2018
Ryosuke Tamura, Sho Maeda, Hiroto Terashi
LCH is a generic term that includes eosinophilic granuloma of the bone, Hand–Schüller–Christian disease, Letterer–Siwe disease, which was previously called Histiocytosis X, pulmonary LCH and Hashimoto–Pritzker disease. Although there are differences in the peak ages of onset, the locations of the disease and the prognosis, they are all the same disease pathologically [3]. The pathogenesis of this disease is still unknown. The incidence is low and has been reported to be one in 200,000 people [1]. In the Histiocyte Society of the World Health Organisation, LCH is classified into three types: unifocal disease, multifocal single system disease and multifocal multisystem disease [3,4]. The frequency of each is almost the same, ranging from 31% to 36%. Multifocal single system disease and multifocal multisystem disease occur in younger people with increased recurrence rates and mortality [3]. It is reported that approximately 30% of all LCH occurs in the oral and maxillofacial area, commonly in the jaw bone [5] and more commonly in the mandible than the maxilla [2].