Autopsy and Case Reports
Autopsy and Case Reports
Article / Clinical Case Report

Indeterminate cell histiocytosis successfully treated with phototherapy

Maria Claudia Nogueira Zerbini, Mirian Nacagami Sotto, Fernando Peixoto Ferraz de Campos, Andre Neder Ramires Abdo, Juliana Pereira, José Antônio Sanches Junior, Jade Cury Martins

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First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome.


Histiocytosis, Skin Diseases, Phototherapy


1. Thomas R, Lipsky PE. Dendritic cells: origin and differentiation. Stem Cells. 1996;14(2):196-206. PMid:8991539.

2. Toebak MJ, Gibbs S, Bruynzeel P, Scheper J, Rustemeyer T. Dendritic cells: biology of the skin. Contact Dermat. 2009;60(1):2-20. PMid:19125717.

3. Bakry OA, Samaka RM, Kandil MA, Younes SF. Intermediate cell histiocytosis with naïve cells. Rare Tumors. 2013;5(1):e13. PMid:23772299.

4. Ventura F, Pereira T, Luz Duarte M. Intermediate cell histiocytosis in association with acute myeloid leukemia. Dermatol Res Pract 2010;2010:569345-9.

5. Weiss LM, Chan JKC, Fletcher CDM. Other rare dendritic cell tumors. In: Swerdlow SH, Campo E, Harris NL et al. editors. World Health Organization classification of tumors of the hematopoietic and lymphoid tissues. Lyon: WHO Press; 2008. chap. 14; p. 365.

6. Vener C, Soligo D, Berti E, et al. Indeterminate cell histiocytosis in association with later occurrence of acute myeloblastic leukaemia. Br J Dermatol. 2007;156(6):1357-61. PMid:17459045.

7. Kolde G, Brocker EB. Multiple skin tumors of indeterminate cells inadult. J Am Acad Dermatol. 1986;15(4 Pt 1):591-7. PMid:3095403.

8. Wood GS, Hu CH, Beckstead LH, Turner RR, Winkelmann RK. The intermediate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis. J Dermatol Surg Oncol. 1985;11(11):1111-9. PMid:3902927.

9. Berti E, Gianotti R, Alessi E. Unusual cutaneous histiocytosis expressing an intermediate immunophenotype between Langerhans’ cells and dermal macrophages. Arch Dermatol. 1988;124(8):1250-3. PMid:3401031.

10. Thomas R, Davis LS, Lipsky PE. Isolation and characterization of human peripheral blood dendritic cells. J Immunol. 1993;150(3):821-34. PMid:7678623.

11. Lenz A, Heine M, Schuler G, Romani N. Human and murine dermis contain dendritic cells: isolation by means of a novel method and phenotypical and functional characterization. J Clin Invest. 1993;92(6):2587-96. PMid:8254016.

12. O’Doherty U, Peng M, Gezelter S, et al. Human peripheral blood contains two dendritic cell sub-sets: one mature and one immature. Immunology. 1994;82(3):487-93. PMid:7525461.

13. Peters JH, Gieseler R, Thiele B, Steinbach F. Dendritic cells: from ontogenetic orphans to myelomonocytic descendants. Immunol Today. 1996;17(6):273-8. PMid:8962630.

14. Rodrígues-Jurado R, Vidaurri-de la Cruz H, DuránMckinster C, Ruíz-Maldonado R. Indeterminate cell histiocytosis. Clinical and pathological study in a pediatric patient. Arch Pathol Lab Med. 2003;127(6):748-51. PMid:12741905.

15. Hashimoto K, Fujiwara K, Mehregan A. Current topics of immunohistochemistry as applied in skin tumors. J Dermatol. 1993;20(9):521-32. PMid:7693783.

16. Ratzinger G, Burgdorf WHC, Metze D, Zelger BG, Zelger B. Indeterminate cell histiocytosis: fact or fiction? J Cutan Pathol. 2005;32(8):552-60. PMid:16115054.

17. Miracco C, Raffaelli M, de Santi MM, Fimiani M, Tosi P. Solitary cutaneous reticulum cell tumor. Enzyme immunohistochemical and electron-microscopic analogies with IDRC sarcoma. Am J Dermatopathol. 1988;10(1):47-53. PMid:2845833.

18. Rezk S, Spagnolo D, Brynes R, Weiss L. Indeterminate cell tumor: a rare dendritic neoplasm. Am J Surg Pathol. 2008;32(12):1868-76. PMid:18813122.

19. Flores-StadlerEM, Gonzales-Crussi F, GreeneM, Thangavelu M, Kletzel M, Chou PM. Indeterminate-cell histiocytosis: immunophenotypic and cytogenetic findings in an infant. Med Pediatr Oncol. 1999;32(4):250-4.<250::AIDMPO2>3.0.CO;2-#. PMid:10102017.

20. Calatayud M, Güell JL, Gris O, Puig J, Arrondo E, Huguet P. Ocular involvement in a case of systemic indeterminate cell histiocytosis: a case report. Cornea. 2001;20(7):769-71. PMid:11588435.

21. Bettington A, Lai J, Kennedy C. Indeterminate dendritic cell tumour presenting in a patient with follicular lymphoma. Pathology. 2011;43(4):372-5. PMid:21566494.

22. Toth B, Katona M, Harsing J, Szepesi A, Karpati S. Indeterminate cell histiocytosis in a pediatric patient: successful treatment with Thalidomide. Pathol Oncol Res. 2012;18(2):535-8. PMid:21688087.

23. Fournier J, Ingraffea A, Pedvis-Leftick A. Successful treatment of indeterminate cell histiocytosis with lowdose methotrexate. J Dermatol. 2011;38(9):937-9. PMid:21366676.

24. Bard S, Torchia D, Connelly E, Duarte A, Badiavas E, Schachner L. S100-negative indeterminate cell histiocytosis in an Africa American child responsive to narrow ultraviolet B. Pediatr Dermatol. 2011;28(5):524-7. PMid:21916959.

25. Ishibashi M, Ouchi T, Tanikawa A, Ishiko A. Indeterminate cell histiocytosis successfully treated with ultraviolet B phototherapy. Clin Exp Dermatol. 2008;33(3):301-4. PMid:18261137.

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