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Hand-Schüller-Christian disease
Hand-Schiiller-Christian disease Joseph W. Hayduk, DEPARTMENT UNIVERSITY
B.S., D.D.S.,*
OF SURGERY, OF TEXAS
ORAL
MEDICAL
AND
Galveston, Texas MAXILLOFACIAL,
BRANCH
R
ecent evidence indicates that Hand-Schiiller-Christian disease and eosinophilic granuloma are related to Letterer-Siwe disease and should be included with the reticuloendothelioses rather than with the lipoidoses.l, ‘, 4, 7*’ The lipoidoses are a group of disorders (Niemann-Pick disease and Gaucher’s disease), also of unknown etiology, in which abnormal accumulation of lipoid OCcurs in various organs and tissues, The reticuloendothelioses are bone disorders which react by the diffuse proliferation of histiocytes of the reticuloendothelial system in response to an unknown inflammatory agent. Early investigators erroneously postulated that the Hand-Schiiller-Christian syndrome represented an error in the metabolism of cholesterol and its esters.3, 6, 9 Letterer-Siwe disease is similar to Hand-&huller-Christian disease and eosinophilic granuloma, but it occurs chiefly in infants. The prognosis is poor in that the disease is rapidly progressive and usually fatal. There is some disagreement as to whether eosinophilic granuloma is a separate disease or simply another manifestation of Hand-Schiiller-Christian disease, since tissues taken from both lesions are quite similar microscopically. Clinically, eosinophilic granuloma is characterized by a single rarefaction of usually one bone and by the absence of visceral involvement. The prognosis is good. Local treatment with curettage and/or x-ray therapy have proved to be curative in many instances. In 1953 Lichtenstein suggested the term histiocytosis X as an all-inclusive name, in view of the large numbers of histiocytes present in each of the three disease processes. These lesions are also found to contain eosinophils, plasma cells, lymphocytes, and polymorphonuclear leukocytes. Certain histiocytes are filled with lipid and are referred to as “foam eelIs,” and the diagnosis of Hand“Former Resident Sts., Camp Hill, Pa.
in Oral
and
Maxillofacial
Surgery.
Present
address:
35th
and
Market
29
30 Hayduk
03, O.M. & O.P. January, 1967
Xchiiller-Christian disease is based on t,he demonstration of these “foam cells,“ which differentiate it from eosinophilic granuloma. HISTORICAL DATA
In 1893 Hands presented a case of skull defects, exophthalmos, and polpuria, (diabetes insipidus) in a 3-year-old child. Because of the soft, yellow, caseous material in the skull defects, he thought the disease to be a manifestation of tuberculosis. Schiiller,n in 1915, and Christian, in 1919, presented simi1a.r cases which they thought resulted from pituitary dysfunction. In 1928 Rowland” coordinated the clinical findings of the three investigators with his own histopathologic observations and coined the term Hand-Schiiller-Christian disease. CLINICAL PICTURE
Classically, but seldom observed, Hand-Schiiller-Christian disease exhibits a triad of defects in membranous bones, exophthalmos, and diabetes insipidus. The patient may also exhibit anemia, malnutrition, and jaundice, while the spleen, liver, kidneys, and lymph nodes may be enlarged. The cxophthalmos is caused by the deposition of foam cells in the fatty bursa back of the eye, and the diabetes insipidus is most likely due to pit.uitary infiltration with the same foam cells. It is not necessary for all the symptoms of the triad to be present in order t)o make a positive diagnosis of Hand-Schiiller-Christian disease. Conversely, .Abbassy and associates1o reported an atypical case in which the first signs were sudden blindness and increased intracranial tension, with skull lesions appearing later; diabetes insipidus and exophthalmos finally appeared, but not until 21/2 years later. The literature is filled with cases presenting only one or two portions of the triad. More commonly bone lesions occur with other less significant features (sore mouth, loose teeth, swollen jaws, gingival necrosis, and soft-tissue swellings over osseouslesions) .lj 2l 5l ’ Involvement of the facial bones, frequently associated with swelling and tenderness, often causes facial asymmetry. Disseminated skin manifestations include papular rashes, seborrhea of t,he scalp, and petechial eruptions. The lipid substances in the blood are not significantly altered, and the cholesterol level invariably lies within normal limits.‘> 2 The blood picture is also relatively normal, although anemia is common in the advanced stages. Diagnosis by sternal marrow aspiration is seldom successful.I X-RAY FINDINGS
Radiographically, the bony lesions are entirely destructive. The bone adjacent to the areas of destruction is relatively normal or, in some cases,has some radiolucency which falls short of actual destruction.3 The individual lesions are quite irregular in shape but grossly may appear round or ovoid. When the calvaria have been extensively involved, these have been referred to as geo-
Volume 23 Number 1
Hand-Schiiller-Christian
disease
31
graphic skull lesions. On a proportionate basis, the jaw lesions tend to be smaller than the skull lesions, The bone about the apices of the molar teeth seems to have some predilection for the disease process. Also, where an unerupted tooth is present, a tooth follicle has been known to be invaded, and often this may mimic a dentigerous cyst. The lesion is always osteolytic, and it is characteristic that it never produces new bone. A review of 600 cases at the Mayo Clinic revealed that many lesions of Hand-Schiiller-Christian disease were erroneously diagnosed as Vincent’s infection, aphthous or herpetic stomatitis, and various other oral conditions. Likewise, severe periodontoclasia or multiple bony lesions should be looked upon with great suspicion, especially in children. The following case report concerns a male child with Hand-Schiiller-Christian disease with typical bone and skull defects and accompanying diabetes insipidus. The case is unique, however, in that almost the entire mandibular architecture was lost to the disease process in a period of approximately 9 months. The case, advanced as it may be, has been held in check by one of the new antineoplastic drugs. CASE REPORT A thin 3-year-old white boy, was admitted to the John Sealy Hospital on Nov. 30, 1962, with a chief complaint of “drinking too much water. ” His mother stated that about 6 months prior to admission the child fell and hit his left temporal region. A physician prescribed medication, and the lump seemingly resolved although the area remained tender. About 3 weeks prior to admission the child began to drink excessive amounts of water (four 8 ounce glasses per hour, if permitted). ‘The mother also noted increased nocturia and anorexia. On Nov. 6, 1962, he was taken to a local physician because of sore teeth and a rash which appeared on the arms and legs. This was diagnosed as a %ose infection,” and the patient was dismissed. A week later the child was taken to another physician, who recommended hospitalization and a bone biopsy. Physical
examination
Upon admission to the hospital, the patient was given a complete physical examination. The vital signs were within normal limits, and the examination findings were unremarkable except for a 3 by 3 cm. soft area over the left eye, which was not tender. X-ray
examination
A skull series revealed destructive changes consistent with a diagnosis of HandSchiiller-Christian disease (Fig. 1). The chest film disclosed a bony lesion in the seventh rib, and films of the pelvis also revealed two small lesions. The long-bone films were unremarkable. A bone-marrow aspiration demonstrated eosinophilia and an increase in plasma cells and reticulum cells which was compatible with a diagnosis of histiocytosis. The complete blood count was unremarkable. The cholesterol was found to be 265 mg. per cent. Electrolytes were within normal limits, as were the urinalysis, blood urea nitrogen, calcium, and phosphorus. The VDRL determination was also negative. A bone biopsy of the rib lesion was consistent with a diagnosis of Hand-Schiiller-Christian disease. Once the diagnosis of Hand-Schiiller-Christian disease with secondary diabetes insipidus was made, the patient was started on a regimen of intramuscular Pitres&n and radiation therapy to the skull, rib, and pelvic lesions. He was given 869 r (266 kv.) to the left temporal,
Fig. CxkHive right
1. Lateral oblique mentgclnograni of mandii,le during deSkUCtiVe ChangeS in ~'itrnus and I~oriy of mandible.
petrous
ridge,
parents were instructed Second
hospital
first, hospital
and pelvic regions, Tllf: patient was s;ulWquently on regulating the dosage of Pitressin.
admi*nion
tlisc*hargetl,
showing
ant1 the
admission
The patient returned to the outpatient clinic on Oct. 1, 1963 (9 months later). The mother reported that the child, now 4 years old, was becoming more difficult to manage and “appears on a downhill course.” He had not gained n-right since being discharged from the ]~ospita], and a soft spot was noted over the left clavicle, (This spot had appeared 3 weeks earlier.) The mother reported an increase in the patient’s water intake since January, 1963, and stated 8.1~0that a ‘(fern” teeth had been lost in t,he last 6 months. The patient was admitted to John Aealy Hospital on Oct. 1, 1963. Physical examination revealed a malnourished, pale, weak child with dry skin, seborrhea of the scalp, a* asymmetrical face with left frontal prominence, loose mandibular incisor teeth, fetid breath, and a small mass over the left clavicle. Repeat s-ray films showed that the skull lesions had somewhat increased in size (Fig. 2) and that the entire mandible was absent except for a small horseshoe-like ring, 0,.5 cm. in diameter, representing the remnants of the mandible (Fig. 3). The mandibular teeth mere no longer embedded in bone but were freely movable upon digital gingivae and the malocpressure, thus accounting for the red, tender, swollen, hyperplastic elusion. Oral hygiene was nonexistent. On Oct. 14, 1963, the patient developed gingival pain, at which time all the mandibular t,ceth were noted to have Class II1 mobility. The patient was immediately placed upon a full liquid diet, but this increased neither his appetite nor his weight. On Oct. 1’7, 1963, he reportedly “lost a few more mandibular teeth.” He was returned to a normal soft diet, but his weight remained between 25 and 26 pounds. Further x-ray therapy of the bony lesions was discouraged, and on Oct. 4, 1963, VeIban (vinblastine sulfate, Lilly), an antineoplastio drug derived from periwinkle, was started. He was given 4 mg. once weekly, provided t,he white blood count remained above 4,000 per cubic millimeter (total dose, 48 mg. or 4 mg. per kilogram of body weight). Oral examination on Jan. 20, 1964, revealed that several teeth were almost exfoliated and in danger of being aspirated. On Feb. 4, 1964, the patient was taken to the operating room, where the remaining mandibular teeth and the loose maxillary molars were removed under general anesthesia. Gauze sponges were applied for hem&&s, and the patient was Bleeding was minimal, and healing returned to the postanesthetic room in good condition.
Volume 23 Number 1
Hand-Schiiller-Christian
disease 33
E
Zllld
Figs. 8 and J. Posteroanterior and lateral skull views showing further mandibular lesions. Almost all of mandible is gone by second hospital
increase in alkull admission.
was rapid. Postoperatively, the boy’s appetite improved noticeably, although he no longer had mandibular teeth. Follow-up roentgenograms on June 1, 1964, revealed that the bony skull lesions had not but neither had they progressed. Of course, it must be rememb reg1 *essed significantly, disease often is quite variable and the total effect of Velbe m is thal ; Hand-Sehiiller-Christian still unknown. Of greater significance was the finding that the patient had gained weight (10 (which appear ‘S to Pou nds) for the first tune in 2 years and that his morale had improved benefit of the Velban therapy). he ttn additional on an outpatient basis. Six The patient was discharged in June, 1964, and followed
w
34
Hayduk
month follow-up roentgenograms revealed patient is still alive, more than 2 years disease was confirmed.
no remarkable bon)- changes. At this time after the diagnosis of Hand-Schiiller-Christian
the
DISCUSSION
It is hardly necessary to belabor further the point that Hand-SchiillerChristian disease, Letterer-Siwe disease, and eosinophilic granuloma are related clinically and pathologically. The relationship is now almost universally accepted.ll Often oral symptoms are the first hint of various systemic diseases,and when multiplicity or severity of lesions is noted, especially in children, the dental surgeon should becomeextremely suspicious. Upon checking the literature, one finds that the mortality rate for HandSchiiller-Christian disease ranges from 15 to 70 per cent, but this high percentage is based on those casesfrom the literature which are heavily weighted by patients with extensive disease involvement. Age, sex, extent, and localization are important influences upon the prognosis. Therefore, those patients in whom a number of organ systems are involved present a poorer prognosis than those with involvement of only one organ system. It is not true that the disease is completely progressive. However, medical care and research are necessary if any hope is to be realized. Laheyl’ reports that only about one third of the diagnosed casesactually terminate fatally. However, the true prognosis will not be known until more caseshave been followed and the cause of the diseasebecomesknown. SUMMARY
This article has presented the classification of Hand-Schiiller-Christian disease and discussed the use of a new antineoplastic drug (Velban) in its treatment. Also, the case of a 4-year-old boy with an extremely advanced mandibular lesion has been described. Appreciation Sealy Hospital,
is extended to Mack I. M&lain, for his interest and advice.
M.D., of the Radiology
Department,
John
REFERENCES
1. Nelson,
W. E.: Textbook
of Pediatrics,
ed. 7, Philadelphia,
1959, W. B. Saunders
Com-
2. $!‘%%e, R. W., Stuteville, 0. H., and Calandra, J. C.: Pathologic Physiology of Oral Disease, St. Louis, 1959, The C. V. Mosby Company. 3. Worth, H. M.: Principles and Practice of Oral Radiologic Interpretation, Chicago, 1963, Year Book Medical Publishers, Inc. 4. McCall, J. O., and Wald, S. S.: Clinical Dental Roentgenology, Philadelphia, 1958, W. B. Saunders Company. of Oral Pathology, Phila5. Shafer, W. G., Hine, M. K., and Levy, B. M.: A Textbook delphia, 1958, W. B. Saunders Company. of Oral Disease, St. Louis, 1955, The C. V. Mosby 6. Bernier, J. L.: The Management Company. 7. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company. 8. Blevins, C. B., Dahlin, D. C., Lovestedt, S. A., and Kennedy, R. L. J.: Oral and Dental Manifestations of Histiocytosis X, OVAL SURQ., ORAL MED. & ORAL PATE. 12: 473, 1959. of Eosinophilic Granuloma of Bone, Hand-Schiiller9. Peracchio, R. L.: A Description
Hand-Schiiller-Christian
disease 35
Christian Disease, and Letterer-Siwe Disease, ORAL SURG.,ORAL MED. & ORAL PATH. 11: 617, 1958. 10. Abbassy, A. Shafik, Massoud, Gamal-el-Dine, and Rida, Amin: Hand-Schiiller-Christian Disease, J. Pediat. 53: 233, 1958. 11. Lahey, M. I%: Prognosis in Reticuloendotheliosis in Children, J. Pediat. 60: 5, 1962.
B.S., D.D.S.,*
OF SURGERY, OF TEXAS
ORAL
MEDICAL
AND
Galveston, Texas MAXILLOFACIAL,
BRANCH
R
ecent evidence indicates that Hand-Schiiller-Christian disease and eosinophilic granuloma are related to Letterer-Siwe disease and should be included with the reticuloendothelioses rather than with the lipoidoses.l, ‘, 4, 7*’ The lipoidoses are a group of disorders (Niemann-Pick disease and Gaucher’s disease), also of unknown etiology, in which abnormal accumulation of lipoid OCcurs in various organs and tissues, The reticuloendothelioses are bone disorders which react by the diffuse proliferation of histiocytes of the reticuloendothelial system in response to an unknown inflammatory agent. Early investigators erroneously postulated that the Hand-Schiiller-Christian syndrome represented an error in the metabolism of cholesterol and its esters.3, 6, 9 Letterer-Siwe disease is similar to Hand-&huller-Christian disease and eosinophilic granuloma, but it occurs chiefly in infants. The prognosis is poor in that the disease is rapidly progressive and usually fatal. There is some disagreement as to whether eosinophilic granuloma is a separate disease or simply another manifestation of Hand-Schiiller-Christian disease, since tissues taken from both lesions are quite similar microscopically. Clinically, eosinophilic granuloma is characterized by a single rarefaction of usually one bone and by the absence of visceral involvement. The prognosis is good. Local treatment with curettage and/or x-ray therapy have proved to be curative in many instances. In 1953 Lichtenstein suggested the term histiocytosis X as an all-inclusive name, in view of the large numbers of histiocytes present in each of the three disease processes. These lesions are also found to contain eosinophils, plasma cells, lymphocytes, and polymorphonuclear leukocytes. Certain histiocytes are filled with lipid and are referred to as “foam eelIs,” and the diagnosis of Hand“Former Resident Sts., Camp Hill, Pa.
in Oral
and
Maxillofacial
Surgery.
Present
address:
35th
and
Market
29
30 Hayduk
03, O.M. & O.P. January, 1967
Xchiiller-Christian disease is based on t,he demonstration of these “foam cells,“ which differentiate it from eosinophilic granuloma. HISTORICAL DATA
In 1893 Hands presented a case of skull defects, exophthalmos, and polpuria, (diabetes insipidus) in a 3-year-old child. Because of the soft, yellow, caseous material in the skull defects, he thought the disease to be a manifestation of tuberculosis. Schiiller,n in 1915, and Christian, in 1919, presented simi1a.r cases which they thought resulted from pituitary dysfunction. In 1928 Rowland” coordinated the clinical findings of the three investigators with his own histopathologic observations and coined the term Hand-Schiiller-Christian disease. CLINICAL PICTURE
Classically, but seldom observed, Hand-Schiiller-Christian disease exhibits a triad of defects in membranous bones, exophthalmos, and diabetes insipidus. The patient may also exhibit anemia, malnutrition, and jaundice, while the spleen, liver, kidneys, and lymph nodes may be enlarged. The cxophthalmos is caused by the deposition of foam cells in the fatty bursa back of the eye, and the diabetes insipidus is most likely due to pit.uitary infiltration with the same foam cells. It is not necessary for all the symptoms of the triad to be present in order t)o make a positive diagnosis of Hand-Schiiller-Christian disease. Conversely, .Abbassy and associates1o reported an atypical case in which the first signs were sudden blindness and increased intracranial tension, with skull lesions appearing later; diabetes insipidus and exophthalmos finally appeared, but not until 21/2 years later. The literature is filled with cases presenting only one or two portions of the triad. More commonly bone lesions occur with other less significant features (sore mouth, loose teeth, swollen jaws, gingival necrosis, and soft-tissue swellings over osseouslesions) .lj 2l 5l ’ Involvement of the facial bones, frequently associated with swelling and tenderness, often causes facial asymmetry. Disseminated skin manifestations include papular rashes, seborrhea of t,he scalp, and petechial eruptions. The lipid substances in the blood are not significantly altered, and the cholesterol level invariably lies within normal limits.‘> 2 The blood picture is also relatively normal, although anemia is common in the advanced stages. Diagnosis by sternal marrow aspiration is seldom successful.I X-RAY FINDINGS
Radiographically, the bony lesions are entirely destructive. The bone adjacent to the areas of destruction is relatively normal or, in some cases,has some radiolucency which falls short of actual destruction.3 The individual lesions are quite irregular in shape but grossly may appear round or ovoid. When the calvaria have been extensively involved, these have been referred to as geo-
Volume 23 Number 1
Hand-Schiiller-Christian
disease
31
graphic skull lesions. On a proportionate basis, the jaw lesions tend to be smaller than the skull lesions, The bone about the apices of the molar teeth seems to have some predilection for the disease process. Also, where an unerupted tooth is present, a tooth follicle has been known to be invaded, and often this may mimic a dentigerous cyst. The lesion is always osteolytic, and it is characteristic that it never produces new bone. A review of 600 cases at the Mayo Clinic revealed that many lesions of Hand-Schiiller-Christian disease were erroneously diagnosed as Vincent’s infection, aphthous or herpetic stomatitis, and various other oral conditions. Likewise, severe periodontoclasia or multiple bony lesions should be looked upon with great suspicion, especially in children. The following case report concerns a male child with Hand-Schiiller-Christian disease with typical bone and skull defects and accompanying diabetes insipidus. The case is unique, however, in that almost the entire mandibular architecture was lost to the disease process in a period of approximately 9 months. The case, advanced as it may be, has been held in check by one of the new antineoplastic drugs. CASE REPORT A thin 3-year-old white boy, was admitted to the John Sealy Hospital on Nov. 30, 1962, with a chief complaint of “drinking too much water. ” His mother stated that about 6 months prior to admission the child fell and hit his left temporal region. A physician prescribed medication, and the lump seemingly resolved although the area remained tender. About 3 weeks prior to admission the child began to drink excessive amounts of water (four 8 ounce glasses per hour, if permitted). ‘The mother also noted increased nocturia and anorexia. On Nov. 6, 1962, he was taken to a local physician because of sore teeth and a rash which appeared on the arms and legs. This was diagnosed as a %ose infection,” and the patient was dismissed. A week later the child was taken to another physician, who recommended hospitalization and a bone biopsy. Physical
examination
Upon admission to the hospital, the patient was given a complete physical examination. The vital signs were within normal limits, and the examination findings were unremarkable except for a 3 by 3 cm. soft area over the left eye, which was not tender. X-ray
examination
A skull series revealed destructive changes consistent with a diagnosis of HandSchiiller-Christian disease (Fig. 1). The chest film disclosed a bony lesion in the seventh rib, and films of the pelvis also revealed two small lesions. The long-bone films were unremarkable. A bone-marrow aspiration demonstrated eosinophilia and an increase in plasma cells and reticulum cells which was compatible with a diagnosis of histiocytosis. The complete blood count was unremarkable. The cholesterol was found to be 265 mg. per cent. Electrolytes were within normal limits, as were the urinalysis, blood urea nitrogen, calcium, and phosphorus. The VDRL determination was also negative. A bone biopsy of the rib lesion was consistent with a diagnosis of Hand-Schiiller-Christian disease. Once the diagnosis of Hand-Schiiller-Christian disease with secondary diabetes insipidus was made, the patient was started on a regimen of intramuscular Pitres&n and radiation therapy to the skull, rib, and pelvic lesions. He was given 869 r (266 kv.) to the left temporal,
Fig. CxkHive right
1. Lateral oblique mentgclnograni of mandii,le during deSkUCtiVe ChangeS in ~'itrnus and I~oriy of mandible.
petrous
ridge,
parents were instructed Second
hospital
first, hospital
and pelvic regions, Tllf: patient was s;ulWquently on regulating the dosage of Pitressin.
admi*nion
tlisc*hargetl,
showing
ant1 the
admission
The patient returned to the outpatient clinic on Oct. 1, 1963 (9 months later). The mother reported that the child, now 4 years old, was becoming more difficult to manage and “appears on a downhill course.” He had not gained n-right since being discharged from the ]~ospita], and a soft spot was noted over the left clavicle, (This spot had appeared 3 weeks earlier.) The mother reported an increase in the patient’s water intake since January, 1963, and stated 8.1~0that a ‘(fern” teeth had been lost in t,he last 6 months. The patient was admitted to John Aealy Hospital on Oct. 1, 1963. Physical examination revealed a malnourished, pale, weak child with dry skin, seborrhea of the scalp, a* asymmetrical face with left frontal prominence, loose mandibular incisor teeth, fetid breath, and a small mass over the left clavicle. Repeat s-ray films showed that the skull lesions had somewhat increased in size (Fig. 2) and that the entire mandible was absent except for a small horseshoe-like ring, 0,.5 cm. in diameter, representing the remnants of the mandible (Fig. 3). The mandibular teeth mere no longer embedded in bone but were freely movable upon digital gingivae and the malocpressure, thus accounting for the red, tender, swollen, hyperplastic elusion. Oral hygiene was nonexistent. On Oct. 14, 1963, the patient developed gingival pain, at which time all the mandibular t,ceth were noted to have Class II1 mobility. The patient was immediately placed upon a full liquid diet, but this increased neither his appetite nor his weight. On Oct. 1’7, 1963, he reportedly “lost a few more mandibular teeth.” He was returned to a normal soft diet, but his weight remained between 25 and 26 pounds. Further x-ray therapy of the bony lesions was discouraged, and on Oct. 4, 1963, VeIban (vinblastine sulfate, Lilly), an antineoplastio drug derived from periwinkle, was started. He was given 4 mg. once weekly, provided t,he white blood count remained above 4,000 per cubic millimeter (total dose, 48 mg. or 4 mg. per kilogram of body weight). Oral examination on Jan. 20, 1964, revealed that several teeth were almost exfoliated and in danger of being aspirated. On Feb. 4, 1964, the patient was taken to the operating room, where the remaining mandibular teeth and the loose maxillary molars were removed under general anesthesia. Gauze sponges were applied for hem&&s, and the patient was Bleeding was minimal, and healing returned to the postanesthetic room in good condition.
Volume 23 Number 1
Hand-Schiiller-Christian
disease 33
E
Zllld
Figs. 8 and J. Posteroanterior and lateral skull views showing further mandibular lesions. Almost all of mandible is gone by second hospital
increase in alkull admission.
was rapid. Postoperatively, the boy’s appetite improved noticeably, although he no longer had mandibular teeth. Follow-up roentgenograms on June 1, 1964, revealed that the bony skull lesions had not but neither had they progressed. Of course, it must be rememb reg1 *essed significantly, disease often is quite variable and the total effect of Velbe m is thal ; Hand-Sehiiller-Christian still unknown. Of greater significance was the finding that the patient had gained weight (10 (which appear ‘S to Pou nds) for the first tune in 2 years and that his morale had improved benefit of the Velban therapy). he ttn additional on an outpatient basis. Six The patient was discharged in June, 1964, and followed
w
34
Hayduk
month follow-up roentgenograms revealed patient is still alive, more than 2 years disease was confirmed.
no remarkable bon)- changes. At this time after the diagnosis of Hand-Schiiller-Christian
the
DISCUSSION
It is hardly necessary to belabor further the point that Hand-SchiillerChristian disease, Letterer-Siwe disease, and eosinophilic granuloma are related clinically and pathologically. The relationship is now almost universally accepted.ll Often oral symptoms are the first hint of various systemic diseases,and when multiplicity or severity of lesions is noted, especially in children, the dental surgeon should becomeextremely suspicious. Upon checking the literature, one finds that the mortality rate for HandSchiiller-Christian disease ranges from 15 to 70 per cent, but this high percentage is based on those casesfrom the literature which are heavily weighted by patients with extensive disease involvement. Age, sex, extent, and localization are important influences upon the prognosis. Therefore, those patients in whom a number of organ systems are involved present a poorer prognosis than those with involvement of only one organ system. It is not true that the disease is completely progressive. However, medical care and research are necessary if any hope is to be realized. Laheyl’ reports that only about one third of the diagnosed casesactually terminate fatally. However, the true prognosis will not be known until more caseshave been followed and the cause of the diseasebecomesknown. SUMMARY
This article has presented the classification of Hand-Schiiller-Christian disease and discussed the use of a new antineoplastic drug (Velban) in its treatment. Also, the case of a 4-year-old boy with an extremely advanced mandibular lesion has been described. Appreciation Sealy Hospital,
is extended to Mack I. M&lain, for his interest and advice.
M.D., of the Radiology
Department,
John
REFERENCES
1. Nelson,
W. E.: Textbook
of Pediatrics,
ed. 7, Philadelphia,
1959, W. B. Saunders
Com-
2. $!‘%%e, R. W., Stuteville, 0. H., and Calandra, J. C.: Pathologic Physiology of Oral Disease, St. Louis, 1959, The C. V. Mosby Company. 3. Worth, H. M.: Principles and Practice of Oral Radiologic Interpretation, Chicago, 1963, Year Book Medical Publishers, Inc. 4. McCall, J. O., and Wald, S. S.: Clinical Dental Roentgenology, Philadelphia, 1958, W. B. Saunders Company. of Oral Pathology, Phila5. Shafer, W. G., Hine, M. K., and Levy, B. M.: A Textbook delphia, 1958, W. B. Saunders Company. of Oral Disease, St. Louis, 1955, The C. V. Mosby 6. Bernier, J. L.: The Management Company. 7. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company. 8. Blevins, C. B., Dahlin, D. C., Lovestedt, S. A., and Kennedy, R. L. J.: Oral and Dental Manifestations of Histiocytosis X, OVAL SURQ., ORAL MED. & ORAL PATE. 12: 473, 1959. of Eosinophilic Granuloma of Bone, Hand-Schiiller9. Peracchio, R. L.: A Description
Hand-Schiiller-Christian
disease 35
Christian Disease, and Letterer-Siwe Disease, ORAL SURG.,ORAL MED. & ORAL PATH. 11: 617, 1958. 10. Abbassy, A. Shafik, Massoud, Gamal-el-Dine, and Rida, Amin: Hand-Schiiller-Christian Disease, J. Pediat. 53: 233, 1958. 11. Lahey, M. I%: Prognosis in Reticuloendotheliosis in Children, J. Pediat. 60: 5, 1962.