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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Hand-Schüller-Christian disease is a variant which typically affects children 1–5 years old. Multiple bones are involved, with evidence of extraskeletal involvement (classic triad of diabetes insipidus, proptosis, and lytic bone lesions). Letterer-Siwe disease is a multisystem variant which affects children less than 2 years old and is often fatal.
Pulmonary Involvement in Systemic Cancer
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Michael R. Bye, Daniel V. Schidlow
Letterer-Siwe Disease is an acute disseminated form of histiocytosis X occurring primarily in children under 3 years of age. Pulmonary involvement as described above plays a prominent clinical role in the patients,24 causing significant morbidity and mortality. In addition, a cystic dilatation of bronchiolar walls can occur, possibly as a result of a weakening of those walls by Langerhans cell invasion. This may also be the etiology of the pneumothorax occasionally seen in these young children.
Cysts and Tumours of the Bony Facial Skeleton
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Julia A. Woolgar, Gillian L. Hall
Langerhans cells, the antigen-presenting histiocytes of epithelia, occasionally give rise to bone tumours. Langerhans cell histiocytosis (histiocytosis X) (LCH) of bone may present as a single ‘eosinophilic granuloma’; or multifocally within bone and other organs, or with generalized disseminated multiorgan disease.34, 79, 107 Multifocal involvement of the craniofacial bones, orbit and posterior pituitary presents with skull defects, exophthalmus and diabetes insipidus (Hand-Schuller-Christian syndrome). The most severe form, disseminated Letterer–Siwe disease, occurs mainly in infants and has a high mortality. Unifocal and multifocal eosinophilic granuloma typically occur in older children/young adults with a 2:1 M:F ratio. The cranium and jaws, in particular the mandible, are common sites. Radiographically, the lesions are osteolytic, rounded and indistinct. Multiple jaw lesions result in the classic ‘teeth floating in air’ appearance. Loosening and exfoliation of teeth is a common presentation. Involvement of the temporal bone can present as otitis media mastoiditis. Histologically, collections of histiocytes admixed with eosinophils are seen. Immunohistochemical demonstration of the Langerhans cells surface antigen, CD1a, and S-100 protein is diagnostic and has largely replaced the electron microscopical detection of Birbeck granules. Generally, eosinophilic granuloma heals after curettage and some regress spontaneously. More disseminated disease is unpredictable. Genetic studies have shown LCH represents a clonal proliferation and the neoplasm may progress despite irradiation and/or chemotherapy.107
Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report
Published in Journal of Dermatological Treatment, 2023
Reem Diab, Mohammad Shahidi Dadras, Azadeh Rakhshan, Ali Kaddah, Fahimeh Abdollahimajd
Histiocytosis is divided into two sub-groups: Langerhans and non-Langerhans cell histiocytosis, distinguished by the immunophenotype of the affected cells (3). Langerhans cell histiocytosis (LCH) includes Letterer–Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, congenital self-healing, and adult LCH, staining positively for CD1a, S100 protein, and CD207 (langerin), and negatively for CD68, CD163, and factor XIIIa (3). Non-Langerhans cell histiocytosis (non-LCH) is divided into three subgroups: primarily cutaneous, cutaneous with systematic involvement, and primarily extra-cutaneous in addition to skin involvement (4). Non-LCH includes juvenile xanthogranuloma, xanthoma disseminatum, benign cephalic histiocytosis, progressive nodular histiocytosis, spindle cell xanthogranuloma, and generalized eruptive histiocytosis, which are of dendritic origin; HPMH was later added to this group (5).
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].
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy
Published in Expert Opinion on Pharmacotherapy, 2018
Langerhans cell histiocytosis (syn.: Letterer–Siwe disease, Hand–Schüller–Christian disease, eosinophilic granuloma; histiocytosis X) is a disease, whose pathobiology still represents an incomplete jigsaw and continues to intrigue and fascinate both biologists and clinicians. Its pathology is characterized by mixed cellular infiltrates and granuloma formation containing the diagnostic hallmark, the so-called LCH cells. The LCH cells are histiocytes sharing cellular phenotype with the normal Langerhans cell, particularly their surface markers CD1a, HLA-DR, S-100, and the cytoplasmic pentalaminar body, known as Birbeck granula. New insights into the molecular biology of the histiocytic disorders were integrated into the recently published revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Based on concomitant occurrence in the same patient, overlapping pathology and shared genetic markers (e.g. BRAF V600E mutation) LCH is now grouped together with Erdheim–Chester Disease and inderterminate cell histiocytosis, to form the ‘L’ (Langerhans-related) Group of histiocytoses [1].