What is Eosinophilic Granuloma?
Human body consists of Langerhans cells that are antigen presenting cells or accessory cells which forms antigen complex at their cell surface that are recognized by T- cells. These cells help in immune system of the body and are present in all the layers of epidermis. These cells sense the presence of foreign pathological bodies and communicate it to the immune cells of the body. They are found in skin, vaginal lining, oral mucosa, and other tissues like lymph tissue. Eosinophilic granuloma is a rare, noncancerous type of Langerhans cell histiocytosis characterized by abnormal proliferation of these dendritic Langerhans cells that are derived from the bone marrow. These cells then migrate from skin to lymph nodes and may affect one single bone and convert into multiple system disorder. Eosinophilic granuloma occurs in both humans and animals and is often referred as rodent ulcer because of the pattern of the lesion. Langerhans cell histiocytosis has three clinical variants namely: Eosinophilic granuloma,
Letterer-Siwe disease and Hand-Schuller-Christian disease. Eosinophilic granuloma is a benign version of Langerhans cell histiocytosis affecting the children, adolescents, and young population.
There are various types of Eosinophilic granuloma depending upon the occurrence. These are:
- Pulmonary histiocytosis X (PHX)/ pulmonary Langerhans cell histiocytosis is a lung disease that is caused due to tobacco smoking primarily affecting the adults and young aged generation.
- Eosinophilic granuloma of skull bone is more commonly seen in children and young adults and presents with single of multiple sites of bone destruction in skull.
Causes of Eosinophilic Granuloma
Exact cause of Eosinophilic granuloma is unknown, and the research is in progress; however, theories suggest the condition to occur because of some specific gene mutation. This gene mutation is somatic in nature and does not show genetic inheritance.
Symptoms of Eosinophilic Granuloma
The symptoms of eosinophilic granuloma include:
- Single or solitary bone lesions or multiple bone lesions. Solitary lesions are more common.
- Following the multiple lesions, new bone lesion develops in a span of one to two years.
- Commonly involved bones are skull, mandible, ribs, spine, pelvis and long bones.
- Condition most commonly affects the bones than lungs, skin and lymph areas.
- Localized pain in the affected area.
- Tenderness and swelling in the area involved.
- Fever and fatigue
- Difficulty in weight bearing
- Back pain
- Neck pain
- Skin rash
- Limited body movement
- Blood reports present increased white blood cell (WBC) count (leukocytosis).
Diagnosis of Eosinophilic Granuloma
Clinical manifestation and signs can help in achieving the differential diagnosis.
Bone lesion biopsy can be performed for more appropriate diagnosis of Eosinophilic granuloma. It is a process where a sample of bone tissue is obtained under anesthesia by small incision and the sample is then sent to the labs. This helps in distinguishing the benign and malignant tumors and eliminate the other possible differential diagnosis such as Multiple myeloma, Fibroma, Osteosarcoma, Osteomyelitis, Osteoblastoma, Rickets disease, Ewing’ sarcoma etc.
Blood test presents increased WBC- leukocytosis.
Prognosis of Eosinophilic Granuloma
Eosinophilic granuloma presents a good prognosis; however, it may show spontaneous regression. The condition is highly radiosensitive.
Treatment for Eosinophilic Granuloma
Eosinophilic granuloma lesions regress on its own within a period of two to three months; however, it may take more than a year or two to suppress. Long standing lesions are painful and are treated with corticosteroid injections. Enlarged tumors may require surgical intervention for partial or complete removal.
- Satter, Elizabeth K., et al. “Eosinophilic Granuloma: Clinical Presentation, Diagnosis, and Treatment of a Rare Langerhans Cell Histiocytosis.” Pediatric Dermatology, Volume 35, Issue 1, 2018, pp 23-28. DOI: 10.1111/pde.13308
- Zhang, Ling, et al. “Eosinophilic Granuloma in Children: Experience From a Medical Center in Taiwan.” Medicine, Volume 97, Issue 31, 2018, e11655. DOI: 10.1097/MD.0000000000011655
- Goyal, Gaurav, et al. “Langerhans Cell Histiocytosis (Histiocytosis X) with Eosinophilic Granuloma: A Rare Presentation.” Journal of Clinical and Diagnostic Research, Volume 11, Issue 11, 2017, pp ED15-ED17. DOI: 10.7860/JCDR/2017/26642.10917
- Kim, Brian, et al. “Eosinophilic Granuloma of the Cervical Spine.” Global Spine Journal, Volume 7, Issue 1, 2017, pp 108S-112S. DOI: 10.1177/2192568216686516