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Hand Schüller Christian disease.
Hand Schuller Christian disease is a rare entity comprising of exophthalmos, diabetes insipidus and geographical map skull. However, the first case described by Hand[9] in 1893 had bronzed skin, hepatosplenomegaly and poor development, besides exophthalmos and geographic map skull. Schuller[17] (1915) and Christians [3],[4] (1920) had called in attention to this triad. It was Rowland[16] (1929) who gave histological description of this lesion in different organs while Green and Farber[7] (1942) demonstrated that eosinophilic granuloma of bone, Hand Schuller Christian disease and Letterer Siwe Syndrome have the same basic pathology. However, Lichtenstein[13], [14] coined the word "HistiocytosisX" to refer to full disease. Others like Henderson[10] do not believe in classifying the disease in above three forms as one form may easily change into the other. Though the exophthalmos, diabetes in sipidus and geographic map skull remain important for diagnosis, number of other features have also been described.
B . B . S . , male aged 17 years was admitted in K.E.M. Hospital on 6-7-1978 with otorrhoea of the left ear of 2 years' duration and discharging sinus on right side of neck of one month's duration. On enquiry, he also gave history of fever off and on, skin rashes on neck off and on and polyurea for 2 years. There was no history of cough with expectoration, hemoptysis or loss of weight. On examination, he was poorly built and nourished with poorly developed secondary sexual characters. His temperature, pulse, respiration and blood pressure were within normal limits. He had left otitis media, left proptosis [Fig. 1] and discrete, nontender multiple cervical nodes. He also had multiple, soft, subcutaneous, tender nodules in the scalp. Systemic examination revealed no abnormality. Following investigations were carried out: Hemoglobin was 12.2 gms%, total WBC count was 6,500/cmm with polymorphs 67% and lymphocytes 33%. Erythrocyte sedimentation rate was 2 mm/hour (Westergren). Blood urea nitrogen, blood sugar, serum electrolytes etc. were within normal limits. Urine specific gravidity was low (1003) and daily urine output was about 6-7 litres. Stool examination was normal. Cervical lymph node biopsy revealed nonspecific changes and liver biopsy was within normal limits. Biopsy of the scalp swelling showed pleomorphic infiltration of lymphocytes, plasma cells, neutrophils and predominent histiocytes. Histiocytes had abundant acidophilic cytoplasm and centrally placed indented nuclei. A few compact group of histiocytes were filled with neutral fat. PAS stain for fungus was negative and there was no suggestion of tuberculosis. A diagnosis of histiocytosis-X (H.S.C.D.) was made [Fig. 2]. Radiologically, skeletal survey revealed irregular well defined osteolytic area with thin, sclerotic borders limited to skull vault and the left petrous pyramid and sphenoid wing producing a `Bare area' on the posterior wall of the left orbit [Fig. 3],[Fig. 4] and [Fig. 5] on). Left carotid angiography was normal however, the urogram film showed distension of bilateral pelvicalyceal system, ureter and bladder due to contrast laden urine inspite of preangiogram evacuation of urine, consistent with diagnosis of diabetes insipidus.
The Hand-Schuller-Christian disease is a well described entity which has appeared sporadically till 1935. The cases have been reported from the Western literature but authentic case reports from India are few.[5], [20] This disease was classified by Obermann[15]into three main groups based upon the findings at onset in bones and soft tissue involvement. Our case fits in Obermann's B2 category.[15] Hand Schuller-Christian disease is also classified from clinicopathological classification" of histiocytes in three main subheadings. (A) Differentiated histiocytes. (B) Intermediate histiocytes. (C) Malignant histiocytes. The present case belongs to Group A (Multifocal, multisystem histiocytosis). In the present case salient features were as follows: This case had classical triad of Hand Schuller-Christian disease which is only seen in 1 out of 10 proved cases. It has skin involvement in the form of erythematous scaly rash which is seen in only 30% of patients. Poor sexual development and retarted growth is another feature. The patient also presented with chronic otitis media of the left ear. However, features like central and perihilar pulmonary involvement, extensive interstitial fibrosis, cor pulmonary, hepatosplenomegaly and thyroid involvement were not present in this case.[11], [12],[13],[14],[21] Cytologically this disease has focal accumulation within bone, skin, lung, masses of tissue macrophages, of which some have foamy appearance with variable admixture of lymphocytes, plasma cells, neutrophils and eosinophils. Eosinophilic definition is not outstanding.[2] In our case we had it in skull bones and skin only. According to the present concept, the syndrome of eosinophlic granuloma, Letterer Siwe disease and Hand Schuller Christian disease are considered a clinical variety of a common pathological process.[2], [10] The course of this disease is entirely variable'; in 35% of patients[13], [14] it gets burned out while in, 15% of patients, it is fatal. Hand-Schuller-Christian disease is placed intermediate in severity and prognosis as compared to the former and the second disease. Our case has a favourable prognosis due to non-involvement of the lung and, liver.[8] The management of this disease is on following principle, i.e. open biopsy for diagnosis, curettage, chemotherapy in acute and maintenance phase,[18] supervoltage therapy,[19] supportive therapy, control of diabetic insipidus and surgery in specific indications [5],[11],[12],[13],[14],[20],[21]
We take this opportunity to thank Dr. A. R. Chitale, Honorary Cytopathologist, Miss Mehta, P.G. student and Dr. V. V. Joshi, Associate Professor of Pathology for reviewing the slides. We also thank Dr. C. K. Deshpande, M.D., F.R.C. Path. (Lond.), Dean, K.E.M. Hospital, Bombay, for giving kind permission to publish this article.
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