InsideSurgery Medical Information Blog

Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news

  • Home
  • Eponyms
  • Abbreviations
  • Terms of Use
  • Privacy Policy
  • Contact

Hand-Schuller-Christian Disease

February 18, 2010

Pathophysiology of Hand-Schuller-Christian Disease

1) Hand-Schuller-Christian disease is characterized by proliferation of histiocytes (Langerhans’ cells) 2) considered a variant of Langerhans’ cell histiocytosis (along with eosinophilic granuloma disease and Letterer-Siwe disease 3) mostly affects children 4) usually fairly indolent in nature with an intermediate prognosis between eosinophilic granuloma (best) and Letterer-Siwe (worst)

Signs and Symptoms

1) classic triad (occurs in one third of cases) – exophthalmos, diabetes insipidus, and multiple cystic effects of skull 2) lymphadenopathy 20%) 3) red crusty sores on trunk, hairline, and dorsum of hands and feet 4) bony lesions are typically the most impressive findings

Characteristic Test Findings

Radiology – 1) lytic bone lesions most commonly in the skull and in scapulae, ribs, pelvis, and jaw (causing “floating teeth”) 2) infiltrates in lung fields (20%)

Histology/Gross Pathology handSchuller

1) Langerhans cells – proliferation and contain racket-shaped Birbeck’s granules 2) nucleus on H and E stain has a “kidney bean” appearance

Associated Conditions

1) hypopituitarism 2) deafness

Inheritance/Epidemiology

1) most commonly affects children age 2-5 years 2) occurs equally in boys and girls 3) makes up 20% of all cases of Langerhans’ cell histiocytosis

Treatment

1) radiation to destroy masses of histiocytes, especially if they are in the retro-orbital area 2) corticosteroids 3) cyclophosphamide 4) diabetes insipidus does not always reverse with treatment

Tips for USMLE

1) if question mentions cells that contain granules shaped like tennis rackets or nuclei that look like kidney beans, think Hand Schuller Christian disease 2) if the question mentions a 5 year-old girl with lytic bone lesions in her skull and exophthalmos, think Hand-Schuller-Christian disease 3) Langerhans cells – another term for histiocyte

Leave a Reply Cancel reply

Your email address will not be published. Required fields are marked *

Categories

  • Acupuncture
  • Amputation Surgery
  • Anesthesia
  • Appendix Surgery
  • Articles
  • Bird Flu
  • BP Oil Spill Health Risks
  • Breast Surgery
  • Cancer
  • Cardiac Surgery
  • Cardiology
  • Case Reports
  • Clinical Trials – Published
  • Colon Surgery
  • Complementary and Alternative Medicine
  • Coronavirus
  • Critical Care
  • Dental and Oral Surgery
  • Dermatology
  • Devices and Technology
  • Drug-Drug Interactions
  • Ebola
  • Endocrinology
  • Eye Surgery
  • For Professionals
  • Gallbladder Surgery
  • Gastroenterology
  • General
  • Grand Rounds
  • Hand Surgery
  • Head and Neck Surgery
  • Healthcare Law
  • Hematology
  • Hernia Surgery
  • Hip Surgery
  • History of Surgery and Medicine
  • Hyperbaric Oxygen
  • Infectious Disease
  • Interviews
  • Liver Surgery
  • Medical Eponym Bios
  • Medical Mnemonics
  • Medical News Wire
  • Medical Trivia
  • Medical Words and Abbreviations
  • Musings
  • Nanomedicine
  • Neck Surgery
  • Neurology
  • Nutrition
  • ObGyn Surgery
  • Oncology
  • Orthopedic surgery
  • Pancreas Surgery
  • Pediatric Surgery
  • Pediatrics
  • People
  • Pharmacology
  • Plastic Surgery
  • Pulmonology
  • Rectal Surgery
  • Rheumatology
  • Small Bowel Surgery
  • Spleen Surgery
  • Stomach Surgery
  • Surgery and Medicine Quotations
  • Surgical Procedure Videos
  • Surgpedia
  • Thoracic Surgery
  • Tip of the Day
  • Transplant Surgery
  • Trauma Surgery
  • Uncategorized
  • Urology Surgery
  • USMLE
  • Vascular Surgery

Archives

© 2021 InsideSurgery.com® · All rights reserved.
This material may not be published, broadcast, rewritten, or redistributed without the express written consent of InsideSurgery.com.
This website uses cookies to improve your experience. We'll assume you're ok with this, but you can opt-out if you wish. Cookie settingsACCEPT
Privacy & Cookies Policy

Privacy Overview

This website uses cookies to improve your experience while you navigate through the website. Out of these cookies, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. We also use third-party cookies that help us analyze and understand how you use this website. These cookies will be stored in your browser only with your consent. You also have the option to opt-out of these cookies. But opting out of some of these cookies may have an effect on your browsing experience.
Necessary
Always Enabled

Necessary cookies are absolutely essential for the website to function properly. This category only includes cookies that ensures basic functionalities and security features of the website. These cookies do not store any personal information.

Non-necessary

Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. It is mandatory to procure user consent prior to running these cookies on your website.

SAVE & ACCEPT
Inside Surgery
  • Home
  • Eponyms
  • Abbreviations
  • Terms of Use
  • Privacy Policy
  • Contact