Eosinophilic granuloma is an uncommon disease characterized by a rapid production of specialized histiocytes found in the skin. It is most often a disease of the adult population that is localized in the lungs or bones.

The cause is unclear. Potential causative agents that have been considered include viruses and tobacco smoke. It most commonly develops in patients between 20 and 40 years of age. It is clearly seen most frequently in males. Large majorities (90 to 100%) of patients are cigarette smokers.

Despite diffuse involvement of the lungs, eosinophilic granuloma commonly produces only minor symptoms. There may be a non-productive cough, mild dyspnea on exertion, or both. Fever and weight loss occurs in less than 10 to 15% of patients. Spontaneous pneumothorax is seen in up to 10% of patients, predominantly young males. The physical examination is normal in most patients.

Chest x-rays are abnormal. High resolution computed tomography (CT) scan is of greater value than standard radiographs in suggesting the diagnosis. Pulmonary function tests (PFT) reveal both restrictive and obstructive lung disease. Routine blood work is usually normal with the exception of increased erythrocyte sedimentation rate (ESR). Bronchial lavage may support the diagnosis in some cases. Open lung biopsy is used most often to obtain tissue.

The effectiveness of drugs has never been demonstrated clearly. Given the close link between tobacco use and eosinophilic granuloma, every effort should be made to ensure that patients with this disease stop smoking.

Spontaneous recovery with little or no respiratory dysfunction is common.

• May be entitled to special monthly compensation where the Veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Special Monthly Compensation (SMC).

• Review special provisions regarding the evaluation of specific respiratory conditions under 38 CFR 4.96(a) - Rating co-existing respiratory conditions and 38 CFR 4.96(d) - Special provisions for the application of evaluation criteria for diagnostic codes 6600, 6603, 6604, 6825–6833, and 6840–6845.