Langerhans cell histiocytosis in an adult patient

Cover Page


Cite item

Full Text

Abstract

Aim: to present a clinical case of a rare dermatosis — Langerhans cell histiocytosis (LCH) in an adult patient.

Materials and methods. A clinical and laboratory examination of a 64-year-old woman who had complained of rashes on the skin of the scalp, neck, trunk and lower extremities accompanied by itching was carried out. A histological study of skin biopsy samples from the lesion area, as well as an immunohistochemical study of Langerhans cell markers — langerin and S-100 protein — were performed.

Results. Clinical manifestations of the disease, the presence of histiocytic infiltrate in the epidermis and dermis during the histological study and immunohistochemical detection of langerin infiltrate cells and S-100 protein were all consistent with the diagnosis of LCH. The therapy with methotrexate subcutaneously significantly improved the patient’s condition.

Conclusion. Verification of the LCH diagnosis requires a histological study of skin biopsy samples and an immunohistochemical study of Langerhans cell markers. The efficacy of methotrexate in the treatment of this disease has been confirmed.

About the authors

A. E. Karamova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Cand. Sci. (Med.), Head of the Dermatology Department,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

V. V. Chikin

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: chikin@cnikvi.ru

Dr. Sci. (Med.), Senior Researcher, Dermatology Department,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

L. F. Znamenskaya

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Dr. Sci. (Med.), Leading Researcher, Dermatology Department,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

M. A. Nefedova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Junior Researcher, Dermatology Department,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

V. A. Mikhina

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Dermatovenerologist, Department of Clinical Dermatology,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

N. S. Battalova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Resident Physician,

Korolenko str., 3, bldg 6, Moscow, 107076

Russian Federation

References

  1. Badalian-Very G., Vergilio J. A., Degar B. A. et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116:1919–1923.
  2. Badalian-Very G., Vergilio J. A., Fleming M., Rollins B. J. Pathogenesis of Langerhans Cell Histiocytosis. Annu Rev Pathol. 2013;24(8):1–20.
  3. Girschikofsky M., Arico M., Castillo D. et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net.Orphanet. J Rare Dis. 2013;8:72.
  4. Kobayashi M., Tojo A. Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Science. 2018;109:3707– 3713.
  5. de Menthon M., Meignin V., Mahr A., Tazi A. Histiocytose à cellules de Langerhans de l’adulte. PresseMed. 2017;46(1):55–69.
  6. Allen C. E., Li L., Peters T. L. et al. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 2010;184:4557–4567.
  7. Carstensen H., Ornvold K. The epidemiology of LCH in children in Denmark. Med Pediatr Oncol. 1993;21:387–388.
  8. Aricò M., Girschikofsky M., Généreau T. et al. Langerhans cell histiocytosis in adults. Report from the international registry of the histiocyte society. Eur J Cancer. 2003;39:2341–2348.
  9. Guyot-Goubin A., Donadieu J., Barkaoui M. et al. Descriptive epidemiology of childhood langerhans cell histiocytosis in France, 2000– 2004. Pediatric Blood Cancer. 2008;51:71–75.
  10. Aricò M., Danesino C. Langerhans’ cell histiocytosis: is there a role for genetics? Haematologica. 2001;86:1009–1014.
  11. Tazi A., Bonay M., Bergeron A. et al. Role of granulocytemacrophage colony stimulating factor (GM-CSF) in the pathogenesis of adult pulmonary histiocytosis X. Thorax. 1996;51:611–614.
  12. Annels N. E., Da Costa C. E., Prins F. A. et al. Aberrant chemokine receptor expression and chemokine production by Langerhans cells underlies the pathogenesis of Langerhans cell histiocytosis. J Exp Med. 2003;197:1385–1390.
  13. Willman C. L., Busque L., Griffith B. B. et al. Langerhans’-cell histiocytosis (histiocytosis X) — a clonal proliferative disease. N Engl J Med. 1994;331:154–160.
  14. Yousem S. A., Colby T. V., Chen Y. Y. et al. Pulmonary Langerhans’ cell histiocytosis: molecular analysis of clonality. Am J Surg Pathol. 2001;25:630–636.
  15. Chakraborty R., Hampton O. A., Shen X. et al. Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. Blood. 2014;124(19):3007–3015.
  16. Rollins B. J. Genomic alterations in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015;29:839–851.
  17. Berres M. L., Lim K. P., Peters T. et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med. 2014;211:669–683.
  18. Roden A. C., Hu X., Kip S. et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol. 2014;38(4):548–551.
  19. Go H., Jeon Y. K., Huh J. et al. Frequent detection of BRAF(V600E) mutations in histiocytic and dendritic cell neoplasms. Histopathology. 2014;65(2):261–272.
  20. Haroche J., Charlotte F., Arnaud L. et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other nonLangerhans cell histiocytoses. Blood. 2012;120(13):2700–2703.
  21. Brown N. A., Furtado L. V., Betz B. L. et al. High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Blood. 2014;124:1655–1658.
  22. Nelson D. S., van Halteren A., Quispel W. T. et al. MAP2K1 and MAP3K1 mutations in Langerhans cell histiocytosis. Genes Chromosom Cancer. 2015;54:361–368.
  23. Nelson D. S., Quispel W., Badalian-Very G. et al. Somatic activating ARAF mutations in Langerhans cell histiocytosis. Blood. 2014;123(20):3152–3155.
  24. Héritier S., Saffroy R., Radosevic-Robin N. et al. Common cancerassociated PIK3CA activating mutations rarely occur in Langerhans cell histiocytosis. Blood. 2015;125:2448–2449.
  25. Héritier S., Emile J. F., Barkaoui M. A. et al. BRAF mutation correlates with high-risk langerhans cell histiocytosis and increased resistance to first-line therapy. J Clin Oncol. 2016;34:3023–3030.
  26. Kudakwashe C., Jaffe R. Langerin (CD207) staining in normal pediatric tissues. Reactive lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol. 2004;7:607–614.
  27. Egeler R. M., van Halteren A. G., Hogendoorn P. C. et al. Langerhans cell histiocytosis: fascinating dynamics of the dendritic cellmacrophage lineage. Immunol Rev. 2010;234:213–232.
  28. Marchal J., Kambouchner M., Tazi A. et al. Expression of apoptosis-regulatory proteins in lesions of pulmonary langerhans cell histiocytosis. Histopathology. 2004;45:20–28.
  29. Senechal B., Elain G., Jeziorski E. et al. Expansion of regulatory T cells in patients with langerhans cell histiocytosis. PloS Med. 2007;4:e253.
  30. Hutter C., Kauer M., Simonitsch-Klupp I. et al. Notch is active in langerhans cell histiocytosis and confers pathognomonic features on dendritic cells. Blood. 2012;120:5199–5208.
  31. Munn S., Chu A. C. Langerhans cell histiocytosis of the skin. Hematol Oncol Clin North Am. 1998;12:269–286.
  32. Favara B. E., Jaffe R. The histopathology of Langerhans cell histiocytosis. Br J Cancer. 1994;Suppl 23:S17–23.
  33. Steen A. E., Steen K. H., Bauer R., Bieber T. Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate. Br J Dermatol. 2001;145:137–140.
  34. Sakai H., Ibe M., Takahashi H. et al. Satisfactory remission achieved by PUVA therapy in Langerhans cell hisiocytosis in an elderly patient. J Dermatol. 1996;23:42–46.
  35. Imafuku S., Shibata S., Tashiro A., Furue M. Cutaneous Langerhans cell histiocytosis in an elderly man success treated with narrowband ultraviolet B. Br J Dermatol. 2007;157:1277–1279.
  36. Emile J. F., Abla O., Fraitag S. et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–2681.

Supplementary files

Supplementary Files
Action
1. JATS XML

Copyright (c) 2019 Karamova A.E., Chikin V.V., Znamenskaya L.F., Nefedova M.A., Mikhina V.A., Battalova N.S.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 60448 от 30.12.2014.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies