|Year : 1987 | Volume
| Issue : 1 | Page : 22-6
Multifocal eosinophilic granuloma of bone causing unilateral proptosis
VB Pratap1, Rajat Desai1, Sandhya Chandra1, PK Aggarwal2
1 Deptt. of Ophthalmology, K.G. Medical College, Lucknow, India
2 Deptt. of Pathology, K.G. Medical College, Lucknow, India
V B Pratap
Deptt. of Ophthalmology, K.G. Medical College, Lucknow
Source of Support: None, Conflict of Interest: None
The most benign expression of the disease histiocytosis X is eosinophilic granuloma of bone. The most common presentation is a solitary granuloma as originally described by. Lichtenstein . Multiple involvement and orbital involvement is extremely rare . We present a case of diffuse eosinophilic granuloma of bone with orbital involvement but with no other significant visceral involvement.
|How to cite this article:|
Pratap V B, Desai R, Chandra S, Aggarwal P K. Multifocal eosinophilic granuloma of bone causing unilateral proptosis. Indian J Ophthalmol 1987;35:22
| Introduction|| |
The term histiocytosis includes three separate clinical entities: Hand Schüller Christian disease, Letterer-Siwe disease, and Eosinophilic granuloma of bone . The boundaries separating these from one another are so indefinite that they tend to be discussed together rather than as separate and distinct disease, a concept suggested by Farber .The name histiocytosis X may not be very appropriate cytopathologically because the eosinophil may be more preponderant in some tissue specimens than the histiocyte .
Lichtenstein and Jaffe  are responsible for the name eosinophilic granuloma. Sidney Farber  suggested that all the three conditions represented variations in degree, stage of involvement and localisation of the same basic disease process. They  later agreed with Farbers concept that the solitary lesion so commonly seen in eosinophilic granuloma was but a variant of the disseminated forms of histiocytosis X. Eosinophilic granuloma of bone, the most benign of the three, is characteristically a disease of children and young adults, mostly males. Involvement of multiple bones is rare .
| Case report|| |
A five year old male child presented with a painful, hot swelling of the right arm present for the last one year following a fracture after trivial trauma. A month later the child developed slow, painless and progressive proptosis of the right eye. Also noticeable since the last three months was a bulge in the skull in the left parietal region. The child had no other complaints.
On general examination, there was firm, non-tender mobile cervical lymphadenopathy and firm smooth, non-tender 2 finger hepatomegaly.
There was a hot, tender, firm-hard swelling of the right arm involving 2/3 of shaft of the humerus without extending to the elbow joint. A fracture had occurred in the same region one year back for which a plaster cast had been given then.
There was a firm swelling in the skull over the left parietal region over which bony defects could be palpated. There was fullness in the right zygomatic region.
Visual acuity was normal; pupillary reactions were normal. Right eye was proposed by 7 mm by Hertels exophthalmometry. Right eye was shifted down by 4 mm.
Orbital tonometry revealed a moderately increased resistance. Ocular position was straight elevation in right eye was slightly restricted. There was no palpable mass or bone erosion around the eye ball. There was no bruit. Fundus oculi right eye showed some venous fullness and disc hyperemia.
| Investigations|| |
Routine hemogram & urine examination were within normal limits.
| Skiagraphy|| |
Right arm - showed cystic areas of rarefaction in the humerus. Skull showed coin shaped osteolytic punched out area.
| Ultrasonography|| |
A Scan showed a mass in the upper temporal region of the orbit adjacent to the eye ball. • Bone marrow smear was within normal limits.
| Pathology|| |
An open biopsy of the arm growth was carried out. Histopathology showed clumps of histiocytes mixed with a varying number of lymphocytes, eosinophils and an occasional neutrophil. The histiocytes varied in size, but were usually relatively large with a pale cytoplasm. The findings were suggestive of Histiocytosis X.
| Discussion|| |
The ophthalmologist does not very frequently get to examine a patient of histiocytosis for the simple reason that its ophthalmic manifestations are extremely rare . In two surveys of orbital tumors done in children (Porterfield 214 cases, and Youssefi 62 cases , not a single case of histiocytosis was reported . In the Mayo Clinic series of the 115 cases of histiocytosis, only 5 had orbital involvement . Involvement of multiple bones in eosinophilic granuloma is very unusual and occurred in less than 20% of patients reported by Sbarbaro & Francis sub . 96% of these were less than 20 years of age. McGarran & Spady observed only one patient in a series of 28 patients with more than one lesion of the bone simultaneously .
Eosonophilic granuloma may involve any bone in the skeletal system, but most of the lesions are found in the skull, ribs, spine and long bones . Our case demonstrated lesions in the humerus and the skull.
The classic radiological appearance which we demonstrated in our single case, is that of sharply defined areas of bone erosion  which generally begins in the medullary region  but expands to involve the inner side of the cortex . Pathological fractures are not uncommon ,. If Lichtenstein concept of the diseases is to be accepted, then eosinophilic granuloma, whether solitary or diffuse, must be considered the most favourable expression of the disease histiocytosis X(6). In multifocal eiosinophilic granuloma of the orbit, involvement of the orbital roof, lateral wall and sphenoid bone may occur .
| Summary|| |
Eosinophilic granuloma is the most benign of the triad of the disease histiocytosis X. It usually affects children of age 4 years or less, with a male preponderance and rarely produces proptosis. Multifocal presentation is rare. Prognosis is fair and the tumour responds both to radiation therapy and chemotherapy, the modality chosen depend on the merits of the particular case 
| References|| |
Lichtenstein L., Jaffe HL, Am.J.Pathol, 16:596, 1940.
Sbarbaro JL. Jr., Francis KC, JA1A, 178 : 706, 1961.
Farber S, Am. J. Pathol, 17 : 625, 1941.
Henderson JW, Farrow Gm, 382, 1973.
IlcGarran Iii, J.bone Joint Surg. 42-A:979, 1960.
Rosai J, Aorfman RF, Cancer, 30 : 1174, 1972.
Feldmen RB et al, Am.J.Ophthalmol., 100 : 318, 1985.
Jaffe HL, Lichtenstein L, Arch. Pathol., 37, 99, 1944.
Lichtenstein L., Arch. Pathol. 56 : 84, 1953.
Friendly DS, Font RL, Rao NA, Arch. Ophthalmol., 95 : 2006, 1977.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]