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Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study

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Abstract

Cutaneous manifestation is a common presentation of LCH and is usually a leading clue for the disease diagnosis. Having cutaneous lesions did not show a significantly early age onset at diagnosis compared to those without skin lesions, P value = 0.71. In the present study, cutaneous findings were found as 77.7%. Seborrheic dermatitis-like lesions were the most common cutaneous type (42.8%), followed by papules/nodules/masses (28.5%), petechiae/hemorrhagic lesions (17.8%), and eczematous lesions (10.7%). Time to diagnosis of LCH presented with seborrheic dermatitis-like lesions was significantly longer than other cutaneous presentations, P value = 0.0011.

Conclusion: Patients with LCH who had the manifestations of seborrheic dermatitis-like lesions can have diagnosis delayed due to the difficulty in distinguishing these lesions from normal seborrheic dermatitis lesions. Petechiae/hemorrhagic cutaneous signs in addition to the normal seborrheic dermatitis is the clue for early detection of the disease. To improve early detection of LCH, general pediatricians should be alerted to be aware of these skin symptoms, and if they persist, a dermatologist, pediatric if available, should be immediately consulted.

What is Known?

Cutaneous manifestation is a common presentation of LCH and is usually a leading clue for the disease diagnosis.

What is New?

Patients with LCH who have the manifestations of seborrheic dermatitis-like lesions can have a delayed diagnosis due to the difficulty in distinguishing normal from seborrheic dermatitis lesions.

Petechiae/hemorrhagic cutaneous signs in addition to the normal seborrheic dermatitis are the clue to the early disease detection.

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Abbreviations

LCH :

Langerhans cell histiocytosis

S-S LCH :

Single-system Langerhans cell histiocytosis

M-S LCH :

Multisystem Langerhans cell histiocytosis

References

  1. Techasatian L, Waraasawapati S (2017) Multiple yellow-red papules on the head and neck in a 3-month-old boy. Pediatr Int Off J Jpn Pediatr Soc 59(1):118–119

    Article  Google Scholar 

  2. Mandel VD, Ferrari C, Cesinaro AM, Pellacani G, Del Forno C (2014) Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with the same cutaneous manifestations but different clinical course. J Dermatol 41(12):1098–1101

    Article  PubMed  Google Scholar 

  3. Wollina U, Langner D, Hansel G, Schönlebe J (2016) Cutaneous Langerhans cell histiocytosis: the spectrum of a rare cutaneous neoplasia. Wien Med Wochenschr 1946:5

    Google Scholar 

  4. Ng SS-Y, Koh MJ-A, Tay Y-K (2013) Cutaneous Langerhans cell histiocytosis: study of Asian children shows good overall prognosis. Acta Paediatr Oslo Nor 1992 102(11):e514–e518

    Google Scholar 

  5. Morren M-A, Vanden Broecke K, Vangeebergen L, Sillevis-Smitt JH, Van Den Berghe P, Hauben E et al (2016) Diverse cutaneous presentations of Langerhans cell histiocytosis in children: a retrospective cohort study. Pediatr Blood Cancer 63(3):486–492

    Article  CAS  Google Scholar 

  6. Minkov M, Prosch H, Steiner M, Grois N, Pötschger U, Kaatsch P et al (2005) Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer 45(6):802–807

    Article  CAS  PubMed  Google Scholar 

  7. Flores-Terry MA, Sanz-Trenado JL, García-Arpa M, Cortina-de la Calle MP (2018) Cutaneous Langerhans cell histiocytosis presenting in adulthood. Actas Dermosifiliogr

  8. Allen CE, Merad M, McClain KL (2018) Langerhans-cell histiocytosis. N Engl J Med 379(9):856–868

    Article  CAS  PubMed  Google Scholar 

  9. Tran G, Huynh TN, Paller AS (2018) Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol 78(3):579–590.e4

    Article  CAS  PubMed  Google Scholar 

  10. Chong VC-L, Tan CL, Chee Y-L, De Mel S (2018) A young patient with a lytic skull lesion. J Clin Pathol 26

  11. Zhu H, Ma Y, Sun L, Zhang R, Lv L, Wang A (2018) Langerhans cell histiocytosis with lymph node involvment presenting as erythroderma. Acta Derm Venereol

  12. Stein SL, Paller AS, Haut PR, Mancini AJ (2001) Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. Arch Pediatr Adolesc Med 155(7):778–783

    Article  CAS  PubMed  Google Scholar 

  13. El Fekih N, Kamoun I, Jones M, Remmeh S, Zéglaoui F, Ben Slama C et al (2013) Histiocytosis X revealed by diabetes insipidus and skin lesions. Am J Dermatopathol 35(5):606–608

    Article  PubMed  Google Scholar 

  14. Rigaud C, Barkaoui MA, Thomas C, Bertrand Y, Lambilliotte A, Miron J et al (2016) Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30-year nationwide cohort of 1478 patients under 18 years of age. Br J Haematol 174(6):887–898

    Article  CAS  Google Scholar 

  15. Ahuja A, Uppe A, Nair G (2018) Multisystem involvement of Langerhans cell histiocytosis. J Assoc Physicians India 66(4):75–76

    PubMed  Google Scholar 

  16. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M et al (1997) Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 29(3):157–166

    Article  CAS  Google Scholar 

  17. Behera B, Malathi M, Thappa DM, Gochhait D, Srinivas BH, Toi PC (2018) Dermoscopic features of three cases of Langerhans cell histiocytosis. Indian J Dermatol Venereol Leprol 84(6):730–735

    Article  PubMed  Google Scholar 

  18. Krooks J, Minkov M, Weatherall AG (2018) Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol 78(6):1047–1056

    Article  PubMed  Google Scholar 

  19. Bellinato F, Maurelli M, Colato C, Balter R, Girolomoni G, Schena D (2018) BRAF V600E expression in juvenile xanthogranuloma occurring after Langerhans cell histiocytosis. Br J Dermatol

  20. Varga E, Korom I, Polyánka H, Szabó K, Széll M, Baltás E et al (2015) BRAFV600E mutation in cutaneous lesions of patients with adult Langerhans cell histiocytosis. J Eur Acad Dermatol Venereol 29(6):1205–1211

    Article  CAS  Google Scholar 

  21. Rizzo FM, Cives M, Simone V, Silvestris F (2014) New insights into the molecular pathogenesis of langerhans cell histiocytosis. Oncologist 19(2):151–163

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Chan MMH, Tan DJA, Koh MJ-A, Tan LS (2018) Blistering Langerhans cell histiocytosis. Lancet Oncol 19(9):e500

    Article  PubMed  Google Scholar 

  23. Dodd E, Hook K (2016) Topical Imiquimod for the treatment of childhood cutaneous Langerhans cell histiocytosis. Pediatr Dermatol 33(3):e184–e185

    Article  PubMed  Google Scholar 

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Acknowledgements

We would like to acknowledge Prof. James Arthur Will, for editing the manuscript via publication clinic KKU, Thailand.

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Authors and Affiliations

Authors

Contributions

L. Techasatian contributed to the conception and design of the study, data analysis, interpretation of findings, drafting the article, revising the article, and final approval of the version submitted. S. Poompuen contributed to study conception and data collection. J. Chaiyarit contributed to data processing and data analysis, critical revision of the article, and final approval of the version submitted.

Corresponding author

Correspondence to Leelawadee Techasatian.

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Ethical approval and informed consent

The study was approved by the institutional review board of Faculty of Medicine, Khon Kaen University, Thailand (IRB no. #HE591399), before enrolling any participants.

Conflict of interest

The authors declare that they have no conflict of interest.

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Communicated by Peter de Winter

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Poompuen, S., Chaiyarit, J. & Techasatian, L. Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study. Eur J Pediatr 178, 771–776 (2019). https://doi.org/10.1007/s00431-019-03356-1

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