Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series

Objective Acute mastoiditis is commonly attributed to infection. Rarely do clinicians encounter cases that do not respond to traditional antibiotics or surgical management. The goal of this study was to systematically review the literature to characterize diseases masquerading as acute infectious mastoiditis. Data Sources PubMed, Embase, and Scopus. Review Methods A systematic review was performed to identify all publications that reported on diseases with presentations mimicking acute mastoiditis, defined as postauricular redness, swelling, and tenderness. We included clinical prospective studies, retrospective studies, and case series/reports. Exclusion criteria included non-English articles, letters/commentaries, abstracts, and review articles. Results Out of 3339 results, 35 studies met final inclusion criteria. In children, 11 diseases were reported to mimic mastoiditis, including solid tumors, hematologic diseases, and autoimmune/inflammatory diseases. The most common disease in children was Langerhans cell histiocytosis, followed by rhabdomyosarcoma and acute myelogenous leukemia. In adults, 8 additional diseases were reported. The most common disease in adults was squamous cell carcinoma, followed by nasopharyngeal carcinoma and Langerhans cell histiocytosis. Presenting symptoms are reviewed, as well as characteristic radiographic, laboratory, and intraoperative features that may assist with diagnosis. A diagnostic algorithm for atypical cases of acute mastoiditis is proposed. Conclusion A small but significant group of diseases in children and adults can mimic acute mastoiditis. In such cases, history and examination alone may be insufficient to reach a diagnosis, and further investigation may be necessary. Otolaryngologists should always be mindful of the possibility that noninfectious pathologies may present with a constellation of symptoms similar to mastoiditis.

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Treatment of Langerhans cell histiocytosis bone lesions with zoledronic acid: a case series

International Journal of Hematology ◽

10.1007/s12185-011-0839-2 ◽

2011 ◽

Vol 93 (6) ◽

pp. 782-786 ◽

Cited By ~ 11

Author(s):

Shanthi Sivendran ◽

Harold Harvey ◽

Allan Lipton ◽

Joseph Drabick

Keyword(s):

Zoledronic Acid ◽

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Bone Lesions

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Langerhans cell histiocytosis in adults: a retrospective, single-center case series

Annals of Hematology ◽

10.1007/s00277-021-04694-7 ◽

2021 ◽

Author(s):

Stefano Chiaravalli ◽

Andrea Ferrari ◽

Luca Bergamaschi ◽

Nadia Puma ◽

Giovanna Gattuso ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Single Center

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Langerhans cell histiocytosis mimicking molluscum contagiosum

Journal of the American Academy of Dermatology ◽

10.1016/j.jaad.2011.09.034 ◽

2012 ◽

Vol 67 (3) ◽

pp. e117-e118 ◽

Cited By ~ 10

Author(s):

Jennifer T. Huang ◽

Jason Mantagos ◽

Roger Kapoor ◽

Birgitta Schmidt ◽

Sheilagh Maguiness

Keyword(s):

Langerhans Cell Histiocytosis ◽

Molluscum Contagiosum ◽

Langerhans Cell

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BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2016.033 ◽

2016 ◽

Vol 8 ◽

pp. 2016033 ◽

Cited By ~ 9

Author(s):

Deepak Chellapandian ◽

Polyzois Makras ◽

Gregory Kaltsas ◽

Cor Van den Bos ◽

Lamia Naccache ◽

...

Keyword(s):

Adverse Effects ◽

Langerhans Cell Histiocytosis ◽

Case Series ◽

Initial Response ◽

Langerhans Cell ◽

Bone Lesions ◽

International Studies ◽

First Line ◽

Retrospective Case ◽

Multisystem Disease

Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

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Langerhans cell histiocytosis

Blood ◽

10.1182/blood.2019000934 ◽

2020 ◽

Vol 135 (16) ◽

pp. 1319-1331 ◽

Cited By ~ 9

Author(s):

Carlos Rodriguez-Galindo ◽

Carl E. Allen

Keyword(s):

Organ Dysfunction ◽

Langerhans Cell Histiocytosis ◽

Survival Rates ◽

Local Treatment ◽

Case Series ◽

Mapk Signaling ◽

Langerhans Cell ◽

Multisystem Disease ◽

Case Series Report ◽

Early Case

Abstract Langerhans cell histiocytosis (LCH) is caused by clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in lesions that leads to a spectrum of organ involvement and dysfunction. The pathogenic cells are defined by constitutive activation of the MAPK signaling pathway. Treatment of LCH is risk-adapted: patients with single lesions may respond well to local treatment, whereas patients with multisystem disease require systemic therapy. Although survival rates for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 20%. Despite progress made in the treatment of LCH, disease reactivation rates remain above 30%, and standard second-line treatment is yet to be established. Treatment failure is associated with increased risks for death and long-term morbidity, including LCH-associated neurodegeneration. Early case series report promising clinical responses in patients with relapsed and refractory LCH treated with BRAF or MEK inhibitors, although potential for this strategy to achieve cure remains uncertain.

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Periodontal manifestations of Langerhans cell histiocytosis: a systematic review

Clinical Oral Investigations ◽

10.1007/s00784-021-03873-0 ◽

2021 ◽

Author(s):

Julia C. Difloe-Geisert ◽

Selina A. Bernauer ◽

Noémie Schneeberger ◽

Michael M. Bornstein ◽

Clemens Walter

Keyword(s):

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Treatment Modalities ◽

Bone Lesions ◽

Bias Assessment ◽

Initial Symptoms ◽

Treatment Measures ◽

Oral Symptoms ◽

Tooth Extractions

Abstract Objectives To explore the evidence of periodontal manifestations and treatment modalities in patients with Langerhans cell histiocytosis (LCH). Material and methods A systematic literature search was performed and the criteria for PRISMA and risk of bias assessment were applied. Human clinical studies (≥10 patients) presenting patients with LCH and periodontal findings were considered for inclusion. Results From 298 titles identified, six case series with a total of 1278 patients suffering from LCH were included. In these studies, oral symptoms were reported in a frequency ranging from 10 to 100%. Overall, in 216 patients (17%), oral symptoms were observed. Out of these patients, 49–100% demonstrated periodontal symptoms. The most common oral findings were pain, swelling, tooth loss/mobility, and bone lesions. Specific periodontal findings comprised varying frequencies of gingival ulcerations, increased pocket depths, and gingival bleeding. Treatment measures constituted of surgical curettage of bone lesions, soft tissue excision and/or tooth extractions, radiotherapy, systemic chemotherapy, or a combination of these approaches. Healing without recurrence of oral lesions was reported in most of the cases. Conclusions The available evidence on periodontal manifestations in LCH patients is heterogeneous. Several oral and periodontal findings were reported and may occur as initial symptoms and/or at later stages of the disease. Clinical relevance The dentist should be aware of possible oral involvement of systemic diseases such as LCH, and these manifestations may mimic periodontal disease.

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Case series of cutaneous Langerhans cell histiocytosis in Indonesian children; The clinicopathological spectrum

Dermatology Reports ◽

10.4081/dr.2020.8777 ◽

2020 ◽

Vol 12 (2) ◽

Author(s):

Irianiwati Widodo ◽

Nita Sahara ◽

Ery Kus Dwianingsih ◽

Paranita Ferronika

Keyword(s):

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Multisystem Disease ◽

Systemic Involvement ◽

Clonal Proliferation ◽

S 100 ◽

Microscopic Features ◽

Skin Biopsies ◽

Histological Reactions

Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of Langerhans cells, which are immunoreactive to S-100 and CD-1a/ CD207 (Langerin). Cutaneous involvement is the most common presentation of LCH in children. It is suggested that the patients with single-system LCH limited to the skin have a better prognosis than those with systemic involvement. Three histologic reactions of cutaneous LCH have been reported and are associated with the clinical types of LCH. These histological reactions include: proliferative, granulomatous, and xanthomatous. This study presents the clinicopathological features of ten cutaneous LCH cases collected from Dr. Sardjito General Hospital Yogyakarta Indonesia between 2014-2018. The ten cases showed various clinical features, in which some features mimic other diseases. The microscopic features of skin biopsies showed granulomatous reaction in 80% of cases and proliferative reaction in the other 20%. Five patients (50% of cases) who died had systemic manifestation of thrombocytopenia, anemia, icterus, hepatosplenomegaly, and revealed the granulomatous type from their skin biopsy specimens. The clinical recognition of LCH and subsequent histological reaction determination are important since some cases may develop multisystem disease and have a poor prognosis.

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Multifocal Osseous Tuberculosis Mimicking Langerhans’ Cell Histiocytosis: A Case Series

Indian Journal of Radiology and Imaging ◽

10.1055/s-0041-1734347 ◽

2021 ◽

Author(s):

Rashmi Singh ◽

Priyanka Naranje ◽

Devasenathipathy Kandasamy ◽

Manisha Jana ◽

Aditya K. Gupta ◽

...

Keyword(s):

Computed Tomography ◽

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Abscess Formation ◽

Common Cause ◽

Atypical Manifestations ◽

Skeletal Lesions

AbstractLangerhans cell histiocytosis (LCH) is a common cause of multifocal lytic skeletal lesions in children. However, multifocal osseous tuberculosis can affect children and mimics LCH on imaging, especially in endemic regions. We report cases with atypical manifestations of multifocal osseous tuberculosis which were presumptively diagnosed as LCH. The findings of our series of cases suggest that on computed tomography (CT) irregular sclerotic margins, abscess formation, sclerosis of involved bone, and button sequestrum point toward a diagnosis of multifocal osseous tuberculosis, especially in endemic regions.

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