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Adiposis dolorosa
ADIPOSIS DOLOROSA* CLYDE L. WILSON, M.D. BOSTON, MASS.
E
VERY physician, in the course of his practice, is consuIted by obese individuaIs compIaining of painfuI areas of fat. The question of what causes the pain and what shouId be done about it aIways arises. Because of this, an attempt has been made to Iook up some of the Iiterature on the subject and to offer an expIanation for the syndrome of symptoms known as adiposis doIorosa. Adiposis doIorosa is a syndrome of symptoms associated with fat metaboIism and was first described by Dercum in 1892 from a case in the wards of a PhiIadeIphia HospitaI. This was preceded by a case report by Dercum2 in 1888, and one by Henry in 1891. Dercum considered the disease a cIinica1 entity and named it adiposis doIorosa because of its most characteristic symptom-painfu1 fat. In his origina paper, Dercuml described 3 cases of the disease with the gross pathoIogica1 findings of 2 cases, both of which showed abnorma1 thyroid gIands. In one the thyroid was enIarged whiIe in the other it was indurated and smaIIer than normaI. Both gIands had caIcareous deposits in them. This Ied Dercum to beIieve that the disease was a cIinica1 entity on the basis of a “disthyroidia.” In Igo Dercum and McCarthy3 described a case of adiposis doIorosa with compIete autopsy findings, the chief pathoIogica1 Iesion being an “adenocarcinoma” of the pituitary body. The thyroid was normaI. FoIIowing this severa cases were described, many of which showed abnormaIities of the pituitary gIand.5~6~7~10 Gushing’” in Ig 12 first questioned the rationaIe of caIIing the disease a cIinica1 entity, stating that, in his opinion, many cases reported as adiposis doIorosa “are actuaIIg exampIes of disturbed metaboIism
secondary to disease of the ductIess gIands.” He presented a case having a11 the characteristic symptoms of Dercum’s disease incIuding increased sugar toIerance. His case improved on treatment with extract of the whoIe pituitary gIand. The pituitary body was found to be enIarged at operation. Citing this case as an exampIe, he pointed out the fact that the disease probabIy began as a disturbance of the pituitary body affecting the thyroid secondariIy. He expIained the nervous disorders on a hypopituitary basis suggesting that many neuroses may be associated with ductIess gIand disturbances. WinkeIman and EckeIJ in 1923 reviewed the Iiterature and found that of the 200 cases of adiposis doIorosa reported onIy 13 had necropsy reports. They added a case of their own. Of these 16 cases, 2 showed no changes in the ductIess gIands. Of the I I cases in which the pituitary body was examined, 8 showed changes in that gIand. The thyroid was abnorma1 in 12 cases, the changes being caIcareous deposits, atrophy or hyperpIasia. Nine cases showed atrophic, “ scIerotic ” or cystic sex gIands. In 3 cases the suprarenaIs were abnorma1 (uniIateraI hyperpIasia, adenoma) and the pancreas showed abnorma1 changes in 2 cases (scIerosis and fatty invasion). From this data they concIuded that the disease is reaIIy a poIygIanduIar disorder, causing the disturbed fat metaboIism. This view has been quite generaIIv accepted. Purves-Stewart’4 in his new book cIsssifies the disease among the tropho-neuroses, probably due to disturbed activity of the thyroid and the posterior Iobe of the pituitary body. Labbe and BouIin* reported a case of adiposis dolorosa almost paraIIe1 to that herein reported. They couId not attribute the psychic and
* From the Lahey Clinic, Roston, 485
class.
486
American Journal of Surgery
WiIson-Adiposis
nervous disorders to any one thing which couId at the same time cause obesity. Newburgh12 can see no reason why the disease cannot be expIained on the basis of energy intake. He points out that the painfu1 areas of fat may be made to disappear on reguIation of the diet aIone and feeIs that it is unnecessary to seek some special metabohc activity to explain the distribution of the fat. Whether the disease may be expIained upon the basis of disturbed metaboIism secondary to changes in the ductIess gIands, or on the basis of energy intake aIone, is not entireIy settIed. It seems more reasonabIe that both factors come into pIay and that the disease is reaIIy a syndrome of symptoms in obese peopIe. Adiposis doIorosa is more common in the femaIe than in the male in the proportion of five to one. It is more common after the menopause, between the ages of forty-five and sixty years, aIthough it may occur at any time between the ages of eIeven and seventy-eight years. Dercum’s 3 patients were fifty-one, sixty-four and sixty years oId, respectiveIy, a11 three being femaIes. Various etioIogica1 factors were described by the earIy writers. These incIuded syphiIis, tubercuIosis, aIcohoIism, acute infectious diseases and operations upon the sex organs. It is we11 known that there is a famiIia1 tendency toward obesity as we11 as toward nervous disorders. Both may occur in the same famiIy. SeveraI authors have reported exampIes of a definite famiIia1 tendency of adiposis doIorosa. Dercum’s disease has been cIassified into three groups according to the distribution of the painfu1 fat. These are (a) the noduIar type, the Ieast common, (b) the circumsciibed diffuse type, the most common, and (c) the generaILed diffuse type, the next most common. The parts most commonIy affected, in the order of their occurrence, are the trunk, shouIders, arms, thighs, forearms and Iegs. The face and hands are rareIy affected. In some cases the painfu1 fat
Dolorosa
MARCH, 1933
is sharpIy localized, resembIing painfu1 Iipomata. Deep furrows may separate the Iumps of fat and the skin is uneven aprons of fat over them. Sometimes reach to the knees. NearIy a11 of the patients are overweight. The disease is sIow and insidious in its onset. There deveIops an excessive deposit of fat diffuseIy over the trunk, and extremities, or in irreguIar deposits of fatty tumors which vary in size from I to 4 inches in diameter, painfu1 to the touch, and somewhat eIevated above the surrounding fat. The overIying skin may be norma in appearance, sIightIy reddened, or more often congested, and rareIy with distended veins. The coIor of the tumors may be due to vascuIar changes. These skin changes finaIIy disappear Ieaving indurated areas of fat tissue. UsuaIIy there are recurrences of the skin changes and pain unti1 there is a fairIy distinct noduIar tumor of considerabIe resistance and consistencv. The fat has been described as feeling OIike a “bag of worms ” or a “ caked breast.” The pain in the fatty masses may vary from tenderness to pressure to vioIent attacks of spontaneous pain which have been described “as though a dog were tearing the flesh from the body.” There is usuaIIy generaIized asthenia varying from fatigue to extreme prostration. The psychic phenomena in&de instabiIity, meIanchoIia, epiIepsy, impairment of memory, hysteria, menta1 confusion and true dementia. In addition the foIIowing conditions have been described as symptoms associated with adiposis doIorosa : (I) parasthesias and areas of anesthesia, (2) diminished tendon reff exes, (3) vasomotor phenomena such as flushing and cyanosis of the skin, tendency to bruise easiIy, purpura and spontaneous hemorrhages from the nose, stomach and uterus, (4) trophic change indicated by the sIow heaIing of uIcerations, (5) myxedema, (6) secondary anemia, (7) arthropathies. The differentia1 diagnosis is concerned with distinguishing this from other forms
NEW SERIES VOL. XIX,
No.
3
Wilson-Adiposis
DoIorosa
American
Journal
of Surgery
-187
of diseases associated with aItered fat metabohsm. In adiposis cerebraIis (FroehIich’s syndrome) there is a moderate deposition of fat, the formation of the body is feminine in the maIe as we11 as in the femaIe, the genitak are ilI deveIoped, and in the maIes the sex characteristics are aItered especiaIIy in the scanty growth of hair on the pubes, in the axiIIae, and on the face. In the femaIe the aItered sex characteristics are seen mostIy in the smaI1 size of the breasts
The treatment resoIves itseIf into the treatment of obesity or Iipomatosis and the associated conditions. SurgicaI remova of the painfu1 fat areas wherever possibIe is indicated. Restriction of diet is of distinct benefit as is the administration of thyroid or pituitary extract. If syphiIis is present it, of course, shouId be treated. SaIicyIates are often of benefit in the treatment of the pain. The foIIowing is the report of a case of the noduIar type of adiposis doIorosa:
and genitaIs. AdenoIipomatosisL5 is an unusua1 condition characterized by either genera1 or Iarge tumor-Iike masses of fat widespread over the body. HistoIogicaIIy this fat shows Iymphoid infiItration. In this disease there is asthenia, sometimes to emaciation, hypochondria, enlargement of the spIeen, and the bIood shows a decrease in the number of smaI1 mononucIear Ieucocytes. Lipodgstrophia is a rare form of obesity progressiva15 in its fuI1 form. It begins in chiIdhood and affects girIs aImost excIusiveIy. In this disease there is an emaciation of of the body with the upper portion aImost compIete disappearance of the subcutaneous fat, and an obesity of the Iower portion of the body. The genera1 heaIth may be good but there are often psychic phenomena. Lipomatosis of the pseudohypertrophic form of muscuIar dysthe dystrophic muscIe trophy I5 shows groups to be enIarged by marked infiItration of the fat between the muscIe bundIes. There may aIso be unusua1 in the mammary and fatty deposits inguina1 regions. The prognosis of Dercum’s disease as to Iife is good, but as to cure is bad. Dercum and McCarthy’s case ran a course of thirteen years. The tenderness aIong the course of the nerves usuaIIy subsides as the disease advances. The fatty masses become Iarger and more generaIized, and the asthenia and mentaI disorders progress. UIt.imateIy the patient intercurrent disease or dies of some complication.
Mrs. G. P., an American housewife, of fortythree, came to the Lahey CIinic compIaining of painfu1 tumors on the arms, and abdomen, and pains in the Iegs. Five years ago the patient first noticed a soft tumor beneath the Ieft breast. This was removed surgicaIIy and was found to be a fat tumor. Two years ago a similar tumor appeared upon the Ieft forearm. It grew to about the size of a walnut and then spread out becoming Iess prominent. At the same time simiIar smaIIer tumors of a like nature deveIoped nearby. About three weeks before coming to the CIinic the patient noticed a soft sweIIing in the right biceps region, on the right forearm, and on both sides of the chest. AI1 of these areas were painfu1. At times there were sharp pains running through the extremities, and through the areas of the tumors. There were aIso painfu1 areas in the thighs. The patient was quite worried about financial matters, and regarding the heaIth of herseIf and husband. She had gained 20 pounds in the past fifteen years. She was afraid that she was becoming insane. She had had a myomectomy in 191 I and a postoperative hernia repair in I 9 I 2. An expIoratory Iaparotomy had been done foIIowing an attack of “hysteria” in 1913. TonsiIIectomy was done in 1930. About’] two years previousIy she suddenIy became paraIyzed in her Iegs whiIe on the beach. She had the wiI1 to move the Iegs but couId not. A physician was calIed and after examining her toId the patient that he couId find nothing wrong with her and advised rest. These orders were carried out and after about two weeks the patient was abIe to resume her norma activities. She had been married fifteen years, with one miscarriage. Her mother died in an insane asylum at fifty-three years of age. Two great-grandfathers became insane and committed suicide
488
American Journal of Surgery
Wilson-Adiposis
by cutting their throats. Her mother’s four uncIes became insane and committed suicide. The patient’s brother is high strung and nervous. The patient’s father is large in size and of a stabIe temperament. The patient was an obese female, weighing about 183 pounds. The examination was essentiaIIy negative except for a carious right Iower premoIar tooth. There was slight diffuse tenderness to pressure over the entire abdomen. The abdomina1 waII was thick and in the right Iower quadrant, in the fatty tissue, there was a soft painfu1 nodule about 2 cm. in diameter, barely paIpabIe. On the volar surface ofthe Ieft forearm there was a soft painfu1 noduIe about 2 cm. in diameter just dista1 to the cubita fossa on the radia1 side. It was sIightIy tender, movable, aud resembIed fat tissue. SimiIar, though smaIIer, areas were present just dista1 to this one, and on the voIar surface of both wrists. The hands, feet and face did not show evidence of excessive fat. The reflexes were normaI. A biopsy of the nodule on the forearm was done and the pathoIogica1 report was Iipoma. This case is an exampIe of the noduIar type of adiposis dolorosa. AI1 of the cardina1 signs and symptoms were present: obesity, pain and menta1 manifestations in a middIe-aged woman. There is a distinct background for her nervous temperament in her famiIy history. Her worries over impending insanity and over financia1 matters heIped to accentuate her neurosis. The pain is the most diffIcuIt to
DoIorosa expIain. Pain in the abdomina1 waI1 of obese persons mav be due to stretching of the nerves that suppIy”it. No expIanation is offered for the pain in the fatty tumors of the forearm. SUMMARY
That adiposis doIorosa is not a cIinica1 entity is shown by the fact that there have been no findings consistent in a11 of the cases reported. It seems more reasonabIe to assume that the condition is one of either simpIe obesity or Iipomatosis associated with neurosis or neurasthenia, and that the pathoIogica1 conditions that have been found in these cases that have come to autopsy were incidenta1. Whether obesity is to be expIained upon the basis of energy intake alone or energy intake PIUS endocrine gIand disturbances is not entirely settIed. The preponderance of evidence points towards the Iatter. This is borne out by the disease adiposis cerebraIis (Froehlich’s syndrome) which is a disease of obesity associated with consistent pathoIogica1 findings in the pituitary gland. On the other hand, improvement in cases of adiposis doIorosa has been observed where the diet aIone has been reduced. It is weI1, therefore, to Iook upon adiposis doIorosa as a syndrome of Iipomatosis or generaIized or IocaIized obesity associated with neurasthenia or neurosis. The treatment is symptomatic. A case of the adiposis doIorosa syndrome is reported.
REFERENCES
I. DERCUM, F. X. Three cases of hitherto unclassified
2.
3.
4.
5.
6.
resembhng in its grosser aspects, affection, obesity but associated with specia1 nervous symptoms-adiposis doIorosa. Am. J. M. SC., 102: 521, 1892. DERCUM, F. X. A subcutaneous connective tissue dystrophy of the anus and back associated with symptoms of myxedema. J. Nervous w Ment. Dis., 15: 695-697, 1888. DERCUM, F. X., and MCCARTHY, D. J. Autopsy in a case of adiposis dolorosa. Am. J. M. SC.. 124: 994. 1902. WINKELMAN, N. W., and ECKEL, J. L. Adiposis doIorosa (Dercum’s disease).:CIinicopathoIogicaI study. J. A. M. A., 85: rg35-1939, 1925. PRICE, G. E. Adiposis dolorosa: a cIinica1 and pathoIogicaI study, with the report of two cases with necropsy. Am. J. M. SC., 137: 705, agog. PRICE, G. E. and BIRD, J. T. Adiposis doIorosa: report of a case with increased sugar toIerance and epileptiform convulsions. J. A. M. A., 84: 247-248, 1925.
7. FOOTE, N. C., GOOD, R. W., and MENARQ M. C. Case of adiposis doIorosa with necropsy. Am. J. Patb., 2: 251-262, rgz6. 8. LABBE: M,, and BOULIN, R. Dercum’s disease, not a chnrcal entity. Bull. et mkm. Sot. mid. d. b6p. de earis, 5 I : 687695, 1927. g. BABOUNEIUX, L., AZERAD, E., and WIDIEZ, A. Adiposis doIorosa. Gaz. d. b$p., IOO: ro57-1059, ‘927. IO. WALDNOP, C. P. OriginaI cIinica1 interpretation of Dercum’s disease, (adiposis doiorosa). Endocrinology, 8: 54-60, 1924. I I. LAROCHE, G. Pathogenesis of Dercum’s disease. Ann. de mid., 23: 445-453, 1928. 12. NEWBURGH, L. H. Cause of obesity. J. A. M. A., 97: 16959, 193’. 13. CUSHING, H. C. The Pituitiary Body and its Disorders. PhiIa., Lippincott. 14. PURVES-STEWART, Sir J. The Diagnosis of Nervous Diseases. London, ArnoId, p. 430. 15. Cecil Medicine. Ed. 2, p. 627.
E
VERY physician, in the course of his practice, is consuIted by obese individuaIs compIaining of painfuI areas of fat. The question of what causes the pain and what shouId be done about it aIways arises. Because of this, an attempt has been made to Iook up some of the Iiterature on the subject and to offer an expIanation for the syndrome of symptoms known as adiposis doIorosa. Adiposis doIorosa is a syndrome of symptoms associated with fat metaboIism and was first described by Dercum in 1892 from a case in the wards of a PhiIadeIphia HospitaI. This was preceded by a case report by Dercum2 in 1888, and one by Henry in 1891. Dercum considered the disease a cIinica1 entity and named it adiposis doIorosa because of its most characteristic symptom-painfu1 fat. In his origina paper, Dercuml described 3 cases of the disease with the gross pathoIogica1 findings of 2 cases, both of which showed abnorma1 thyroid gIands. In one the thyroid was enIarged whiIe in the other it was indurated and smaIIer than normaI. Both gIands had caIcareous deposits in them. This Ied Dercum to beIieve that the disease was a cIinica1 entity on the basis of a “disthyroidia.” In Igo Dercum and McCarthy3 described a case of adiposis doIorosa with compIete autopsy findings, the chief pathoIogica1 Iesion being an “adenocarcinoma” of the pituitary body. The thyroid was normaI. FoIIowing this severa cases were described, many of which showed abnormaIities of the pituitary gIand.5~6~7~10 Gushing’” in Ig 12 first questioned the rationaIe of caIIing the disease a cIinica1 entity, stating that, in his opinion, many cases reported as adiposis doIorosa “are actuaIIg exampIes of disturbed metaboIism
secondary to disease of the ductIess gIands.” He presented a case having a11 the characteristic symptoms of Dercum’s disease incIuding increased sugar toIerance. His case improved on treatment with extract of the whoIe pituitary gIand. The pituitary body was found to be enIarged at operation. Citing this case as an exampIe, he pointed out the fact that the disease probabIy began as a disturbance of the pituitary body affecting the thyroid secondariIy. He expIained the nervous disorders on a hypopituitary basis suggesting that many neuroses may be associated with ductIess gIand disturbances. WinkeIman and EckeIJ in 1923 reviewed the Iiterature and found that of the 200 cases of adiposis doIorosa reported onIy 13 had necropsy reports. They added a case of their own. Of these 16 cases, 2 showed no changes in the ductIess gIands. Of the I I cases in which the pituitary body was examined, 8 showed changes in that gIand. The thyroid was abnorma1 in 12 cases, the changes being caIcareous deposits, atrophy or hyperpIasia. Nine cases showed atrophic, “ scIerotic ” or cystic sex gIands. In 3 cases the suprarenaIs were abnorma1 (uniIateraI hyperpIasia, adenoma) and the pancreas showed abnorma1 changes in 2 cases (scIerosis and fatty invasion). From this data they concIuded that the disease is reaIIy a poIygIanduIar disorder, causing the disturbed fat metaboIism. This view has been quite generaIIv accepted. Purves-Stewart’4 in his new book cIsssifies the disease among the tropho-neuroses, probably due to disturbed activity of the thyroid and the posterior Iobe of the pituitary body. Labbe and BouIin* reported a case of adiposis dolorosa almost paraIIe1 to that herein reported. They couId not attribute the psychic and
* From the Lahey Clinic, Roston, 485
class.
486
American Journal of Surgery
WiIson-Adiposis
nervous disorders to any one thing which couId at the same time cause obesity. Newburgh12 can see no reason why the disease cannot be expIained on the basis of energy intake. He points out that the painfu1 areas of fat may be made to disappear on reguIation of the diet aIone and feeIs that it is unnecessary to seek some special metabohc activity to explain the distribution of the fat. Whether the disease may be expIained upon the basis of disturbed metaboIism secondary to changes in the ductIess gIands, or on the basis of energy intake aIone, is not entireIy settIed. It seems more reasonabIe that both factors come into pIay and that the disease is reaIIy a syndrome of symptoms in obese peopIe. Adiposis doIorosa is more common in the femaIe than in the male in the proportion of five to one. It is more common after the menopause, between the ages of forty-five and sixty years, aIthough it may occur at any time between the ages of eIeven and seventy-eight years. Dercum’s 3 patients were fifty-one, sixty-four and sixty years oId, respectiveIy, a11 three being femaIes. Various etioIogica1 factors were described by the earIy writers. These incIuded syphiIis, tubercuIosis, aIcohoIism, acute infectious diseases and operations upon the sex organs. It is we11 known that there is a famiIia1 tendency toward obesity as we11 as toward nervous disorders. Both may occur in the same famiIy. SeveraI authors have reported exampIes of a definite famiIia1 tendency of adiposis doIorosa. Dercum’s disease has been cIassified into three groups according to the distribution of the painfu1 fat. These are (a) the noduIar type, the Ieast common, (b) the circumsciibed diffuse type, the most common, and (c) the generaILed diffuse type, the next most common. The parts most commonIy affected, in the order of their occurrence, are the trunk, shouIders, arms, thighs, forearms and Iegs. The face and hands are rareIy affected. In some cases the painfu1 fat
Dolorosa
MARCH, 1933
is sharpIy localized, resembIing painfu1 Iipomata. Deep furrows may separate the Iumps of fat and the skin is uneven aprons of fat over them. Sometimes reach to the knees. NearIy a11 of the patients are overweight. The disease is sIow and insidious in its onset. There deveIops an excessive deposit of fat diffuseIy over the trunk, and extremities, or in irreguIar deposits of fatty tumors which vary in size from I to 4 inches in diameter, painfu1 to the touch, and somewhat eIevated above the surrounding fat. The overIying skin may be norma in appearance, sIightIy reddened, or more often congested, and rareIy with distended veins. The coIor of the tumors may be due to vascuIar changes. These skin changes finaIIy disappear Ieaving indurated areas of fat tissue. UsuaIIy there are recurrences of the skin changes and pain unti1 there is a fairIy distinct noduIar tumor of considerabIe resistance and consistencv. The fat has been described as feeling OIike a “bag of worms ” or a “ caked breast.” The pain in the fatty masses may vary from tenderness to pressure to vioIent attacks of spontaneous pain which have been described “as though a dog were tearing the flesh from the body.” There is usuaIIy generaIized asthenia varying from fatigue to extreme prostration. The psychic phenomena in&de instabiIity, meIanchoIia, epiIepsy, impairment of memory, hysteria, menta1 confusion and true dementia. In addition the foIIowing conditions have been described as symptoms associated with adiposis doIorosa : (I) parasthesias and areas of anesthesia, (2) diminished tendon reff exes, (3) vasomotor phenomena such as flushing and cyanosis of the skin, tendency to bruise easiIy, purpura and spontaneous hemorrhages from the nose, stomach and uterus, (4) trophic change indicated by the sIow heaIing of uIcerations, (5) myxedema, (6) secondary anemia, (7) arthropathies. The differentia1 diagnosis is concerned with distinguishing this from other forms
NEW SERIES VOL. XIX,
No.
3
Wilson-Adiposis
DoIorosa
American
Journal
of Surgery
-187
of diseases associated with aItered fat metabohsm. In adiposis cerebraIis (FroehIich’s syndrome) there is a moderate deposition of fat, the formation of the body is feminine in the maIe as we11 as in the femaIe, the genitak are ilI deveIoped, and in the maIes the sex characteristics are aItered especiaIIy in the scanty growth of hair on the pubes, in the axiIIae, and on the face. In the femaIe the aItered sex characteristics are seen mostIy in the smaI1 size of the breasts
The treatment resoIves itseIf into the treatment of obesity or Iipomatosis and the associated conditions. SurgicaI remova of the painfu1 fat areas wherever possibIe is indicated. Restriction of diet is of distinct benefit as is the administration of thyroid or pituitary extract. If syphiIis is present it, of course, shouId be treated. SaIicyIates are often of benefit in the treatment of the pain. The foIIowing is the report of a case of the noduIar type of adiposis doIorosa:
and genitaIs. AdenoIipomatosisL5 is an unusua1 condition characterized by either genera1 or Iarge tumor-Iike masses of fat widespread over the body. HistoIogicaIIy this fat shows Iymphoid infiItration. In this disease there is asthenia, sometimes to emaciation, hypochondria, enlargement of the spIeen, and the bIood shows a decrease in the number of smaI1 mononucIear Ieucocytes. Lipodgstrophia is a rare form of obesity progressiva15 in its fuI1 form. It begins in chiIdhood and affects girIs aImost excIusiveIy. In this disease there is an emaciation of of the body with the upper portion aImost compIete disappearance of the subcutaneous fat, and an obesity of the Iower portion of the body. The genera1 heaIth may be good but there are often psychic phenomena. Lipomatosis of the pseudohypertrophic form of muscuIar dysthe dystrophic muscIe trophy I5 shows groups to be enIarged by marked infiItration of the fat between the muscIe bundIes. There may aIso be unusua1 in the mammary and fatty deposits inguina1 regions. The prognosis of Dercum’s disease as to Iife is good, but as to cure is bad. Dercum and McCarthy’s case ran a course of thirteen years. The tenderness aIong the course of the nerves usuaIIy subsides as the disease advances. The fatty masses become Iarger and more generaIized, and the asthenia and mentaI disorders progress. UIt.imateIy the patient intercurrent disease or dies of some complication.
Mrs. G. P., an American housewife, of fortythree, came to the Lahey CIinic compIaining of painfu1 tumors on the arms, and abdomen, and pains in the Iegs. Five years ago the patient first noticed a soft tumor beneath the Ieft breast. This was removed surgicaIIy and was found to be a fat tumor. Two years ago a similar tumor appeared upon the Ieft forearm. It grew to about the size of a walnut and then spread out becoming Iess prominent. At the same time simiIar smaIIer tumors of a like nature deveIoped nearby. About three weeks before coming to the CIinic the patient noticed a soft sweIIing in the right biceps region, on the right forearm, and on both sides of the chest. AI1 of these areas were painfu1. At times there were sharp pains running through the extremities, and through the areas of the tumors. There were aIso painfu1 areas in the thighs. The patient was quite worried about financial matters, and regarding the heaIth of herseIf and husband. She had gained 20 pounds in the past fifteen years. She was afraid that she was becoming insane. She had had a myomectomy in 191 I and a postoperative hernia repair in I 9 I 2. An expIoratory Iaparotomy had been done foIIowing an attack of “hysteria” in 1913. TonsiIIectomy was done in 1930. About’] two years previousIy she suddenIy became paraIyzed in her Iegs whiIe on the beach. She had the wiI1 to move the Iegs but couId not. A physician was calIed and after examining her toId the patient that he couId find nothing wrong with her and advised rest. These orders were carried out and after about two weeks the patient was abIe to resume her norma activities. She had been married fifteen years, with one miscarriage. Her mother died in an insane asylum at fifty-three years of age. Two great-grandfathers became insane and committed suicide
488
American Journal of Surgery
Wilson-Adiposis
by cutting their throats. Her mother’s four uncIes became insane and committed suicide. The patient’s brother is high strung and nervous. The patient’s father is large in size and of a stabIe temperament. The patient was an obese female, weighing about 183 pounds. The examination was essentiaIIy negative except for a carious right Iower premoIar tooth. There was slight diffuse tenderness to pressure over the entire abdomen. The abdomina1 waII was thick and in the right Iower quadrant, in the fatty tissue, there was a soft painfu1 nodule about 2 cm. in diameter, barely paIpabIe. On the volar surface ofthe Ieft forearm there was a soft painfu1 noduIe about 2 cm. in diameter just dista1 to the cubita fossa on the radia1 side. It was sIightIy tender, movable, aud resembIed fat tissue. SimiIar, though smaIIer, areas were present just dista1 to this one, and on the voIar surface of both wrists. The hands, feet and face did not show evidence of excessive fat. The reflexes were normaI. A biopsy of the nodule on the forearm was done and the pathoIogica1 report was Iipoma. This case is an exampIe of the noduIar type of adiposis dolorosa. AI1 of the cardina1 signs and symptoms were present: obesity, pain and menta1 manifestations in a middIe-aged woman. There is a distinct background for her nervous temperament in her famiIy history. Her worries over impending insanity and over financia1 matters heIped to accentuate her neurosis. The pain is the most diffIcuIt to
DoIorosa expIain. Pain in the abdomina1 waI1 of obese persons mav be due to stretching of the nerves that suppIy”it. No expIanation is offered for the pain in the fatty tumors of the forearm. SUMMARY
That adiposis doIorosa is not a cIinica1 entity is shown by the fact that there have been no findings consistent in a11 of the cases reported. It seems more reasonabIe to assume that the condition is one of either simpIe obesity or Iipomatosis associated with neurosis or neurasthenia, and that the pathoIogica1 conditions that have been found in these cases that have come to autopsy were incidenta1. Whether obesity is to be expIained upon the basis of energy intake alone or energy intake PIUS endocrine gIand disturbances is not entirely settIed. The preponderance of evidence points towards the Iatter. This is borne out by the disease adiposis cerebraIis (Froehlich’s syndrome) which is a disease of obesity associated with consistent pathoIogica1 findings in the pituitary gland. On the other hand, improvement in cases of adiposis doIorosa has been observed where the diet aIone has been reduced. It is weI1, therefore, to Iook upon adiposis doIorosa as a syndrome of Iipomatosis or generaIized or IocaIized obesity associated with neurasthenia or neurosis. The treatment is symptomatic. A case of the adiposis doIorosa syndrome is reported.
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