Besnier Boeck Schaumann Disease

Definition

Definition of Besnier Boeck Schaumann
When inflammatory granular nodules form in various organs in the body, the situation is known as Besnier Boeck-Schaumann Disease. The disease is fatal and can cause following complications:

1. Cheilitis granulomatosa
2. Dactylitis
3. Hypercalciuria
4. Breathlessness
5. Dry eyes
6. Myocarditis
7. Optic neuritis
8. Splenomegaly
9. Atrioventricular node conduction block
10. Erythema nodosum
11. Restrictive cardiomyopathy
12. Saddle nose deformity
13. CSF lymphocytes
14. Hilar lymphadenopathy
15. Pyrexia of unknown origin
16. Bone cyst

Symptoms

Symptoms of Besnier Boeck Schaumann
Apart from Fatigue, Cough, Weight loss, Chest pain, Fever, Joint pain, and Erythema nodosum, the disease may also cause:

1. Expectoration
2. Coughing up blood
3. Cyanosis
4. Cutaneous polymorphic lesions
5. Enlarged liver
6. Enlarged spleen
7. Iridocyclitis
8. Scleral nodules
9. Chorioretinitis
10. Acute migratory monoarthritis
11. Polyarthritis
12. Rales
13. Reduced white blood cell count
14. Eosinophilia
15. Low blood platelet level
16. High urine calcium level
17. Breathing difficulty
18. Vision difficulty
19. Blindness
20. Bell palsy
21. Adenoapthy

Causes

Causes of Besnier Boeck Schaumann
No direct cause of sarcoidosis can been identified, though reports of cell wall deficient bacteria that may be possible pathogens have been reported. These bacteria are generally not identified in standard laboratory analysis. It has been suggested that there may be a hereditary factor because there are some families with multiple members affected with sarcoidosis. To date, no reliable genetic markers have been targeted, and an alternate hypothesis is that family members share a similar exposure to environmental pathogens. Transmission of sarcoidosis via organ transplants have also been reported. Sarcoidosis has been traced to celiac disease. Celiac disease is a condition wherein there is a chronic reaction to certain protein chains, commonly known as glutens, found in some cereal grains. This reaction causes the destruction of the villi in the small intestine, which results malabsorption of nutrients.

Diagnosis/Prognosis

Diagnosis of Besnier Boeck Schaumann
The diagnosis of the disease may depend upon the health of the patients. It may include:

1. X-ray of chest
2. Blood test
3. Examination of the eyes using a special light called a slit-lamp may reveal changes indicative of sarcoidosis
4. Bronchoscopy
5. The physical examination in sarcoidosis to reveal the characteristic skin lesions

Treatment

Treatment of Besnier Boeck Schaumann

1. Systemic or topical steroid, as does sarcoidosis that produces hypocalcaemia or destructive skin lesions.
2. Various other drugs used in addition to corticosteroids include immunosuppressant, methotrexate, azathioprine, and cyclophosphamide.
3. Other treatment may include: i) Low-calcium diet and avoidance of direct exposure to sunlight in the patients who are suffering with hypercalcemia., ii) Organ transplantation.