Atypical presentation of isolated orbital Langerhans cell histiocytosis.
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Abstract:
A 9-year old female presented with one month of waxing and waning upper eyelid swelling. An excisional biopsy via anterior orbitotomy was performed.To describe a patient presenting atypically with symptoms concerning for orbital cellulitis who was diagnosed with Langerhans cell histiocytosis (LCH).Description of case report.We report a case of a 9-year old female with one month of periorbital edema and erythema suspected to be orbital cellulitis. A complete ophthalmological exam, subsequent imaging, and an excisional biopsy revealed the diagnosis of LCH. With a confirmed diagnosis, the patient started chemotherapy indicated by the Histiocyte Society Evaluation and Treatment Guidelines.Langerhans cell histiocytosis (LCH) embodies a spectrum of diseases with the primary pathologic process being the abnormal proliferation of polyclonal Langerhans cells. In children with isolated bony involvement, the most common presenting symptom is pain. Rarely is orbital involvement with associated periorbital edema and erythema the primary presentation.Keywords:
Langerhans Cell Histiocytosis
Erythema
Presentation (obstetrics)
Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.
Langerhans Cell Histiocytosis
Rare disease
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Girl
Exophthalmos
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This paper describes the clinical investigation, and light and electron microscope findings in a Pakistani girl who developed bilateral orbital and eyelid swellings in association with lymphadenopathy.The findings do not correlate with any of the previously recognized causes of these symptoms although similarities with some of the lymphoreticular disorders, in particular Hand-Schuller-Christian disease and the recently described sinus histio- cytosis with massive lymphadenopathy (Rosai and Dorfman, I969) are present.The nature of the condition and its differential diagnosis and treatment are discussed.Case history An Ix-year-old Pakistani girl, who was born in Birmingham but subsequently visited Pakistan, presented in April, I970, with recurrent eyelid swellings and progressive proptosis.She also com- plained of irritation and inability to close the left eye and recent onset of increasing deafness.Similar lid swellings had previously been excised in I962 and I966 at the Queen Elizabeth Hos- pital, Birmingham, when she was aged 3 and 7 years respectively.Apart from these complaints the patient was well, having no history of fever, ear discharge, or loss of weight or appetite.Two other members of the patient's family (Fig. i), in which first-cousin marriages are common, E' 2 Male unaffected * Male affected 0 Female unaffected II e : t (;;) < Female affected ® Female presumed affected
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Eyelid Diseases
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Purpose: To describe and illustrate recent reclassification and clinical descriptions of the histiocytoses, diagnosis and management, and effects on the eye and adnexaePatients and methods: Literature review with an illustrative case series of seven patients with histiocytosis and ophthalmic involvement from a single tertiary centre.Results: Skin lesions with signs including eyelid mass, orbital bone destruction, bizarre keratic precipitates, limbal or iris mass, haemorrhagic anterior uveitis, subretinal lesions of uncertain etiology, ischaemic retinopathy, optic neuropathy, and apparent steroid-resistant inflammations should be comprehensively investigated.Conclusions: Ophthalmic involvement in histiocytosis is rare. Clinical manifestations vary from limited single-organ disease to lethal systemic infiltration or malignancy. Radiology may identify lesions for biopsy. Histology with appropriate markers is essential. Oncology advice may be required.
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Langerhans Cell Histiocytosis
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Eosinophilic granuloma
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Histiocytosis X
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Langerhans Cell Histiocytosis
Mandible (arthropod mouthpart)
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Indeterminate cell histiocytosis is a rare neoplastic disease characterized by proliferation of dendritic cells that share morphologic and immunophenotypic features of Langerhans cells and non-Langerhans histiocytes. ICH lesions are typically restricted to the skin; however, there have been rare reports of extracutaneous and systemic involvement.We describe a case of a 13-year-old female presenting with complaints of worsening lower back pain for 1 year. CT and MRI of the lumbar spine demonstrated a well-defined bony, lytic, expansile lesion of the posterior and mid-endplate of the L4 vertebra with mass effect on the thecal sac. The patient underwent L3-L5 decompression and fusion with surgical excision of the vertebral body tumor. Microscopic examination of the lesion showed benign fibrohistiocytic proliferation with giant cells, and immunohistochemical staining revealed a phenotype consistent with indeterminate cell histiocytosis (S100+ CD1a+ langerin-).ICH is an extraordinarily rare neoplastic disease of dendritic cells that has a poorly understood pathogenesis. This case expands the spectrum of potential ICH extracutaneous involvement to now include the spine, a location previously undocumented in the literature in the pediatric population.
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Background: Langerhans cell histiocytosis (LCH) is a group of disorders that encompasses a wide range of disease spectrum from localized spontaneous remission to systemic involvement. It is uncommon in neonatal period. Cutaneous manifestation is the most common presentation in neonatal onset LCH. Skin lesions vary from single solitary nodule to generalized cutaneous eruption. Case Report: The present authors reported three cases that include two males and one female. All cases were presented with generalized, discrete erosive papules with hemorrhagic crusts since birth. In all cases, physical examinations were normal. Laboratory investigations including complete blood cell count, chemistry panel, urinalysis, and liver function tests were unremarkable. Bone survey shown no osteolytic lesion. Skin biopsy with immunohistologic staining was performed and confirmed diagnosis of LCH in all cases. Skin lesions improved in few weeks later with no other systemic symptoms in all cases. At the six-week follow-up, one case developed high fever and hepatosplenomegaly. A few months later, it was diagnosed as LCH with systemic involvement by a pediatric oncologist. Conclusion: Cutaneous manifestation is the common presentation of LCH in neonate. Although the majority of patients are benign, skin-only and self-limiting clinical course, careful physical examination and long-term follow-up for systemic involvement are mandatory. Keywords: Langerhans cell histiocytosis, Cutaneous manifestation, Neonate Received 18 Jul 2019 | Revised 27 Sep 2019 | Accepted 30 Sep 2019
Langerhans Cell Histiocytosis
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