eMedicine Specialties > Dermatology > Malignant Neoplasms

Langerhans Cell Histiocytosis: Follow-up

Author: Christopher R Shea, MD, Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago
Coauthor(s): Markus D Boos, PhD, Medical Scientist Training Program, Pritzker School of Medicine, University of Chicago
Contributor Information and Disclosures

Updated: Aug 26, 2009

Follow-up

Complications

  • Complications appear in 30-50% of patients with Langerhans cell histiocytosis (LCH).
    • The most common complications are orthopedic disabilities, hearing impairment, diabetes insipidus, skin scarring, and neuropsychologic defects (eg, anxiety, depression, decreased intellect).11,44,45
    • Patients with multisystem disease, craniofacial involvement, long-standing disease, or reactivation may be at increased risk of developing diabetes insipidus.46,47 Regular neurological examinations and whole brain MRI are recommended to evaluate for CNS Langerhans cell histiocytosis lesions and evidence of neurodegeneration in patients with newly recognized diabetes insipidus.48
    • Less common sequelae include chronic pulmonary dysfunction, liver cirrhosis, secondary malignancies such as acute lymphoblastic leukemia or solid tumors, and growth retardation.25

Prognosis

  • The clinical course of Langerhans cell histiocytosis is variable. Patients with unifocal Langerhans cell histiocytosis generally have an excellent prognosis.
    • After initial bone scanning and radiographic survey to assess the extent of the disease, follow-up studies after treatment should be performed at 6-month intervals for 3 years.
    • If no additional lesions are present at 1 year, the development of subsequent lesions is unlikely.
    • A full recovery is also expected in cases of solitary lymph node involvement or isolated skin disease.
  • Multifocal Langerhans cell histiocytosis has a variable prognosis, especially in patients at the extremes of age with pulmonary involvement. The prognosis is worse than in patients with unifocal disease but better than those with disseminated disease. Sixty percent of patients with multifocal disease have a chronic course, 30% of patients undergo complete remission, and 10% of patients with multifocal Langerhans cell histiocytosis die from the disease.49
    • Response to chemotherapy in the first 6 weeks (induction therapy) is among the most important prognostic indicators for multifocal Langerhans cell histiocytosis. A good response to chemotherapy during this period is associated with significantly improved survival.23,35
    • Conversely, hematologic involvement or involvement of organs such as the lungs, spleen, and liver is associated with worse long-term outcomes.23
  • Letterer-Siwe disease (disseminated) has a high mortality rate. The prognosis in these patients depends on the patient's age, the extent of disease, and the degree of organ dysfunction. The mortality rate is 50% or higher.
  • The congenital form of histiocytosis tends to resolve spontaneously within weeks to months.50
    • Although the absence of systemic disease at presentation and the tendency of resolution of the disease are favorable, long-term follow-up care to detect evidence of relapse or progression in these patients is suggested.50
    • Relapse in these patients has been reported up to 5 years after the initial disappearance of the disease.
    • Cutaneous lesions usually disappear by 3 months, leaving residual hypopigmentation.
  • Infrequently, a case originally diagnosed as chronic focal Langerhans cell histiocytosis may progress to multifocal or even disseminated disease.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose cases of Langerhans cell histiocytosis, resulting in delayed treatment
  • Failure to consider cases of Langerhans cell histiocytosis among the differential diagnoses when cutaneous lesions do not respond to topical corticosteroids and failure to perform a biopsy in this case
  • Failure to recognize that a cutaneous eruption of Langerhans cell histiocytosis can closely simulate an eczematous rash, resulting in misdiagnosis
  • Note: Skin involvement occurs in more than 80% of patients with disseminated disease and in 30% of patients with less extensive multisystem disease.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, M. Angelica Selim, MD, and previous Chief Editor, William D. James, MD, to the development and writing of this article.



More on Langerhans Cell Histiocytosis

Overview: Langerhans Cell Histiocytosis
Differential Diagnoses & Workup: Langerhans Cell Histiocytosis
Treatment & Medication: Langerhans Cell Histiocytosis
Follow-up: Langerhans Cell Histiocytosis
Multimedia: Langerhans Cell Histiocytosis
References
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Further Reading

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Keywords

Langerhans cell histiocytosis, LCH, histiocytosis X, Langerhans cell granulomatosis, type II histiocytosis, nonlipid reticuloendotheliosis, Langerhans cells, LCs

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Contributor Information and Disclosures

Author

Christopher R Shea, MD, Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago
Christopher R Shea, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, Arthur Purdy Stout Society, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois Dermatological Society, International Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Markus D Boos, PhD, Medical Scientist Training Program, Pritzker School of Medicine, University of Chicago
Disclosure: Nothing to disclose.

Medical Editor

Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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