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Hand schuller christian disease: a rare case report with oral manifestation
Ch Lalitha1, M Manjula2, K Srikant3
1Senior Lecturer, Department of Oral Medicine and Radiology, Sri Sai College of Dental Surgery , Vikarabad,Telanagana, India .
Insights
Langerhan's Cell Histiocytosis (LCH) is a rare disorder involving abnormal Langerhan cell proliferation. This case report details a 6-year-old boy diagnosed with Hand Schuller Christian Disease, a type of LCH, presenting with eye and dental issues.
Area of Science:
- Histiocytosis
- Pediatric Pathology
- Rare Diseases
Background:
- Langerhan's Cell Histiocytosis (LCH) comprises disorders characterized by abnormal proliferation of Langerhan cells.
- LCH presents clinically as eosinophilic granuloma, Hand Schuller Christian disease, and Abt-Letterer-Siwe disease.
- Manifestations occur in both children and adults due to Langerhan cell infiltration.
Observation:
- A rare case of LCH in a 6-year-old boy is presented.
- The patient exhibited extraoral exophthalmos of the right eye and oral symptoms including tooth mobility.
- Typical radiological findings were observed.
Findings:
- Clinical, radiological, and histopathological examinations confirmed the diagnosis.
- The patient was diagnosed with Hand Schuller Christian Disease, a specific type of LCH.
- The case highlights unusual extraoral and oral manifestations.
Implications:
- This case underscores the diverse clinical presentations of LCH, particularly Hand Schuller Christian Disease.
- Early diagnosis and recognition of varied symptoms are crucial for effective management.
- Further research into LCH's varied manifestations can improve patient outcomes.
Abstract:
Langerhan's Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan's cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-Letterer- Siwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the result of the accumulation and infilteration of the langerhan cells in organs and tissues. Here is a rare case report of 6-year-old boy with extraoral manifestation of exopthalmic right eye and oral manifestation of mobility of teeth and with typical radiological findings. Basing on the clinical, radiological and histopathological examination the diagnosis of Hand Schuller Christian Disease was given.
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