MyMedR

Childhood Langerhans cell histiocytosis: a disease with many faces

Leung AKC, Lam JM, Leong KF

World J Pediatr, 2019 Dec;15(6):536-545.

PMID: 31456157 DOI: 10.1007/s12519-019-00304-9

BACKGROUND: Langerhans cell histiocytosis (LCH) is a group of diseases characterized by the proliferation and accumulation of Langerhans cells. Clinical presentations of LCH vary widely.

DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.

RESULTS: Generally, patients with LCH can be divided into two groups based on the extent of involvement at diagnosis, namely, single-system LCH and multisystem LCH. The involvement may be unifocal or multifocal. Patients with isolated bone lesions typically present between 5 and 15 years of age, whereas those with multisystem LCH tend to present before 5 years of age. The clinical spectrum is broad, ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition. Clinical manifestations include, among others, "punched out" lytic bone lesion, seborrheic dermatitis-like eruption, erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches, and eczematous lesions, diabetes insipidus, hepatosplenomegaly, cytopenias, lymphadenopathy, and an acute fulminant disseminated multisystem condition presenting with fever, skin rash, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. The diagnosis is clinicopathologic, based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue. Positive CD1a, S100, and/or CD207 (Langerin) immunohistochemical staining of lesional cells is required for a definitive diagnosis. Watchful waiting is recommended for patients with skin-only LCH. Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids, topical nitrogen mustard, oral methotrexate, or oral hydroxyurea. The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine. Mercaptopurine is added for patients with risk organ involvements.

CONCLUSIONS: Because of the broad spectrum of clinical manifestations and the extreme diversity of disease, LCH remains a diagnostic dilemma. Morphological identification of LCH cells and positive immunochemical staining with CD1a, S100, and/or CD207 (Langerin) of lesional cells are necessary for a definitive diagnosis.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/diagnosis*

Fulltext Langerhans cell histiocytosis with extensive spinal and thyroid gland involvement presenting with quadriparesis: an unusual case in an adult patient

Mohd Ariff S, Joehaimey J, Ahmad Sabri O, Zulmi W

Malays Orthop J, 2011 Nov;5(3):28-31.

PMID: 25279033 MyJurnal DOI: 10.5704/MOJ.1111.004

Langerhans cell histiocytosis of the spine frequently presents as a solitary lesion and rarely results in neurological deficit. Involvement of thyroid tissue is also rare, even in multifocal disease presentations. We present an unusual variant of Langerhans cell histiocytosis in a 37-year-old man presenting with profound quadriparesis and incidental thyroid involvement. MR imaging and skeletal radiographs revealed widespread involvement of the spine. The patient underwent surgical resection of the tumour and stabilization of the spine. Diagnosis was confirmed by histological and immunohistochemistry analysis. Langerhans cell histiocytosis should be included in the differential diagnosis of radiolucent lesions of the spine in adults.

Matched MeSH terms: Histiocytosis, Langerhans-Cell*

Chronic disseminated histiocytosis X: a case report

Ong ST, Lian CB

J Clin Pediatr Dent, 1992;17(1):33-5.

PMID: 1290758

A case of chronic disseminated histiocytosis X (Hand-Schuller-Christian Disease) of the mandible is presented. Multi-modal approach to management is discussed.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/diagnosis; Histiocytosis, Langerhans-Cell/surgery*

Fulltext Langerhans cell histiocytosis of maxillary sinus

Azreen AB, Kwan CY, Prepagaren N

Med J Malaysia, 2012 Dec;67(6):627-8.

PMID: 23770962 MyJurnal

Langerhan cell histiocytosis is a rare disease and usually occurs in paediatric age group. This disease may involve single or multiple organs system and has an unpredictable course of disease. The involvement of head and neck region are almost 90% of cases, however maxillary sinus involvement is very rare. We report a case of 2 year old boy presented with multi organ LCH (orbit, skull, sinus and liver). The mainstay treatment for this high risk multi organ LCH group is chemotherapy. Unfortunately, although with the advancement of treatment, their mortality rate is still high.

Matched MeSH terms: Histiocytosis, Langerhans-Cell*

Fulltext Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

Abdul Aziz DA, Abdul Rahman NA, Tang SF, Abdul Latif H, Zaki FM, Annuar ZM, et al.

BMJ Case Rep, 2011;2011.

PMID: 22674944 DOI: 10.1136/bcr.09.2011.4734

Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/radiography; Histiocytosis, Langerhans-Cell/surgery*

Histiocytosis X and vulvar ulceration

Wong KK, Lin HP, Looi LM

Int J Gynaecol Obstet, 1992 Oct;39(2):131-4.

PMID: 1358712

Vulvar ulceration is a rare manifestation of histiocytosis X. A 13-year-old girl had a nonhealing vulvar ulcer for 1 year. She had been in remission from histiocytosis X and the ulcer was not recognised as a sign of disease recurrence until tissue biopsy was obtained for histopathological and immunohistochemical studies. This article stresses the importance of establishing an accurate diagnosis when chronic vulvar ulcers are encountered and reviews the literature on this uncommon presentation of histiocytosis X.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/diagnosis; Histiocytosis, Langerhans-Cell/pathology

Fulltext Periodontal disease as the initial oral manifestation of langerhans cell histiocytosis - a case report

Ahmad AR, Ismail SM, Zain RB, Mohamad Zaini Z

Ann Dent, 2009;16(1):31-36.

MyJurnal

Langerhans Cell Histiocytosis (LCH) refers to a group of lesions presenting with a spectrum of clinical. features but sharing similar histology. These lesions are rare and treatment has been quite variable with current treatment protocol recommended being dependent on whether it is a unifocal or multi focal bone disease or a multi focal multisystem disease. However, the clinical presentations of LCH are variable and the decision to place into the appropriate clinical types may sometimes be masked by the non-discovery of all the lesions. In the oral maxillofacial area, the clinical features of these lesions may further pose a problem by nondescript manifestations as dental/periodontal/oral mucosal disorders. These oral findings may sometimes lead to inappropriate choice of treatment and delay in the diagnosis of all the lesions involved. This paper describes one such case where LCH manifest itself as a periodontal disease thus leading to delay in identifying all the sites involved and consequently a delay in id~ntifying the appropriate clinical type.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext A gluteal mass of langerhans cell histiocytosis mimicking malignancy in a two-year-old boy: a case report

Ibrahim ZA, Lung WS, Long PK

Malays J Med Sci, 2009 Jan;16(1):48-50.

PMID: 22589649 MyJurnal

Langerhans cell histiocytosis is a disease primarily affects the bone. More than 50 percent of the disease occurs between the age of 1 and 15. We reported a case of a 2 year old boy who presented with a gluteal mass. Radiographic imaging showed an osteolytic lesion suspicious of malignancy. However, the histological diagnosis was Langerhans cell histiocytosis.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Histiocytosis X: evidence for a genetic etiology

Hanapiah F, Yaacob H, Ghani KS, Hussin AS

J Nihon Univ Sch Dent, 1993 Sep;35(3):171-4.

PMID: 8246038

Histiocytosis X is a rare disorder with no particular predilection for race, age or sex. Since its discovery by Hand in 1893, the etiology has remained unknown, although viruses, bacteria and genetic factors have been implicated. Familial occurrence of this disease is very rare, and only a handful of such cases have been reported. The present study adds further evidence to support the influence of genetic factors in the etiology of histiocytosis X.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/genetics*

Fulltext Multisystem Langerhans cell histiocytosis presenting as an oral lesion

George KT, Anand R, Ganasalingam S, Zain RB

J Oral Maxillofac Pathol, 2013 Jan;17(1):106-9.

PMID: 23798841 DOI: 10.4103/0973-029X.110694

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which the pathologic Langerhans cells infiltrate and destroy the tissues. Patients with LCH present varied clinical manifestations. Cutaneous lesions in LCH manifest as vesiculopapular eruptions that often mimic various infectious diseases particularly in infants. We present a case of a female infant with an ulcerative lesion intraorally. The baby was asymptomatic otherwise. A detailed history revealed the presence of cutaneous lesions that was overlooked by her parents.

CONCLUSION: This report tries to briefly discuss the current concepts regarding the etiology of LCH. An attempt has been made to emphasis the need for a through systemic examination. The protocol of investigative procedures to be adopted in LCH is also discussed.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext Case Report: Total hip replacement for recurrent histiocytosis X of the pelvis involving the hip joint

Wazir,N.N., Ravindran, T., Mukundala, V.V., Choon, S.K.

Malays Orthop J, 2007;1(2):21-23.

MyJurnal

In 1977, a female patient, aged 31y, presented with histiocytosis X in the right pelvis and was treated with radiotherapy. She presented again in 1999 at the age of 53y with a similar problem at the same location this time also involving the acetabulum. The affected right hip was treated with cemented total hip replacement. Two years postoperatively, the patient was pain free with no evidence of local recurrence or loosening of implant, thus demonstrating that total hip replacement is a viable treatment option for histiocytosis involving the hip.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext Concurrent Mastoid Cellulitis and Langerhans Cells Histiocytosis:  A Challenging Diagnosis

Ong HY, Goh LC, Santhi K, Sha'ariyah MM

Oman Med J, 2018 Mar;33(2):167-170.

PMID: 29657688 DOI: 10.5001/omj.2018.31

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, which commonly arises in the bone and may involve other systems. To date, the diagnosis of temporal bone LCH remains a challenge as it may masquerade as a common ear infection. We report a case of a child who presented to us with persistent bilateral ear discharge for four months and was not responding to treatment. Her condition subsequently worsened, with clinical features and radiological findings suggestive of mastoid cellulitis. Nevertheless, further histopathology study revealed LCH.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext Bilateral spontaneous persistent open pneumothorax with chylothorax

Khajotia R, Raman S

Can Fam Physician, 2012 Jul;58(7):757-60.

PMID: 22859639

Matched MeSH terms: Histiocytosis, Langerhans-Cell/diagnosis

Oral Langerhans cell histiocytosis in Malaysian children: a 40-year experience

Jalil Ab, Hin-Lau S

Int J Paediatr Dent, 2009 Sep;19(5):349-53.

PMID: 19486369 DOI: 10.1111/j.1365-263X.2009.00985.x

BACKGROUND: Oral Langerhans cell histiocytosis is generally seen in children.
OBJECTIVE: To determine the clinicopathological features of oral LCH in Malaysian paediatric patients.
METHODS: A retrospective study was carried out to determine the clinicopathological features of Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and histiocytosis X occurring in the oral cavity in children, diagnosed histologically in the main oral histopathology laboratory in Malaysia from 1967 to 2007.
RESULT: There were 17 cases (eight girls and nine boys) with age ranging from 1 to 7 years. There were ten Malays, four Chinese, two Indians, and one of other ethnicity. Thirteen cases presented as gingival swellings with six of these cases accompanied with mobility of the teeth. Nine cases involved the mandible, two in the maxilla, and two cases in both the maxilla and mandible. The radiographic findings were mentioned only in nine cases with presence of bony erosion or destruction of the jaw bones. Four cases had punched-out radiolucencies of the skull. The patients also had other systemic signs and symptoms: skin lesions (n = 5), hepatosplenomegaly (n = 2), prolonged fever (n = 2), diabetes insipidus (n = 1), and exophthalmos (n = 1). Two cases were known cases of systemic LCH.
CONCLUSION: The histopathologic features of LCH are easily recognized; however, with the development of immunostaining, the use of CD1a helps in confirming the diagnosis.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/pathology*

Histopathology of lymph nodal tuberculosis--university hospital experience

Jayalakshmi P, Malik AK, Soo-Hoo HS

Malays J Pathol, 1994 Jun;16(1):43-7.

PMID: 16329575

Fifty-nine cases of tuberculous cervical lymphadenitis were analysed histologically. Characteristic epithelioid cell granulomas were seen in all the cases with central areas of caseation necrosis in 96.6% (57/59) of these cases. The diagnosis of tuberculosis was further established by the demonstration of acid-fast bacilli (AFB) in the tissue sections in 29 cases. These AFB, although occasional, were found more frequently within the epithelioid cells as compared with other zones of the granuloma. There was no significant association between necrosis and bacillary content. We conclude that light microscopical assessment is still a useful screening method to diagnose tuberculosis in cases of cervical lymphadenopathy.

Matched MeSH terms: Histiocytosis, Langerhans-Cell/microbiology; Histiocytosis, Langerhans-Cell/pathology

Fulltext Langerhans cell histiocytosis in an adult - a rare, life-threatening and not to be missed

Lai JY, Tang MM, Priya G, Rajasuriar JS, Suganthi T

Med J Malaysia, 2014 Apr;69(2):95-7.

PMID: 25241821

Langerhans Cell Histiocytosis (LCH) is an uncommon neoplastic disorder characterized by accumulation of histiocytes in various tissues. The clinical manifestation is highly variable, ranging from an isolated skin rash or a single bony lesion, to fatal multi-organ failure. Due to its rarity and systemic involvement, the epidemiology of LCH is still not fully understood and most studies focus on the paediatric population. The extent of clinical involvement has important prognostic implications. Treatment options may be local or systemic depending on disease extent. Here we describe a rare case of adult onset LCH who presented with recurrent pneumothoraces, diabetes insipidus and papules and plaques over the scalp and forehead.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma

Addenan M, May CM, Hooi TK, Ismail F, Kamalden TA

Oman J Ophthalmol, 2018 12 7;11(3):284-287.

PMID: 30505126 DOI: 10.4103/ojo.OJO_149_2017

Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext Importance of clinical and morphological correlations in diagnosing Langerhans cell histiocytosis

Seb, Omayma, Fauzana, K., Aisyah, M.R., Noraidah, M., Noor Hamidah, H.

Medicine & Health, 2018;13(1):220-226.

MyJurnal

Langerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Adult Langerhans cell histiocytosis: An unusual cause of referred knee pain

Swarna Nantha Y, Yeoh MH, Sharif SM

Malays Fam Physician, 2019;14(3):60-64.

PMID: 32175042

Langerhans cell histiocytosis (LCH) in adults is rare and regarded as an 'orphan disease.' The systemic symptoms of LCH can mimic many other undifferentiated diseases seen at the primary care level. Failure to diagnose and delays in referral are common pitfalls in the management of this disease. We present a case of a 34-year-old woman with referred knee pain who was eventually diagnosed with multi-system LCH 4 years after the initial presentation. The mean age of presentation of LCH symptoms in adults is 33. Bone lesions are the frequent presentation of LCH in this age group. Endocrine involvement in LCH is seen in the form of diabetes insipidus (DI), which remains the most common extraskeletal presentation of LCH in adults. In the case discussed here, a definitive diagnosis of LCH was established through tissue biopsy. The spectrum of undifferentiated symptoms underscores the difficulty and delay in making a diagnosis associated with the condition. Most GPs not only face the predicament of initial recognition but also fail to merge presenting symptoms to form a purposeful referral of this elusive disease to a tertiary care unit.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

Fulltext Idiopathic thrombocytopenic purpura in childhood, Langerhans cell histiocytosis in adulthood: More than a chance association?

Kuan YC, Nurain MN

Med J Malaysia, 2017 02;72(1):50-52.

PMID: 28255140 MyJurnal

Described herein, a case of Langerhans cell histiocytosis (LCH) in an adult with Idiopathic Thrombocytopenic Purpura (ITP) diagnosed at age ten. She presented with cranial diabetes insipidus, later developed hypogonadotrophic hypogonadism and multiple cervical lympadenopathy from which histopathology of excisional biopsy confirmed LCH. Magnetic resonance imaging showed thickened pituitary stalk. Association of ITP and LCH is unknown but the question of LCH presenting as isolated thrombocytopenia in childhood only to be discovered in adulthood when there was pituitary and bone involvement remains. It reemphasizes the need for high index of suspicion and the challenges in diagnosing LCH at the outset.

Matched MeSH terms: Histiocytosis, Langerhans-Cell

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