ALLERGY GRAND ROUNDS
Factitious allergic emergencies: Anaphylaxis and laryngeal “edema” Roy Patterson,
M.D., and Michael
Schatz,
M.D. Chicngo,
111.
DR. PATTERSON:Systemic IgE-mediated reactions or diseases that mimic them constitute some of the most acute of medical emergencies. The medical management of such cases consists of immediate measures to maintain life and prevent progression of symptoms and subsequent measures to prevent a recurrence by attempting to discover the inducing agent or mechanism. TWO of the most serious reactions of this type in man in terms of their rapidity of onset and potentia,l to cause death include systemic allergic reactions and laryngeal edema. The latter may occur as the primary medical problem or as a component of the systemic allergic reaction. The systemic allergic reaction is the human counterpart of experimental anaphylaxis in animals. The term anaphylaxis has been restricted by some to the induced, experimental animal model. However, the similarity of the immunologic, cellular, and pharmacologic mechanisms in syst’emic allergic reactions in man and anaphylaxis in animals appears to justify the use of the term a?taphylaxis in the description of the human event. The current discussion will include a review of two cases of acute recurrent human reactions. Both cases constituted considerable problems in emergency management, multiple hospitalizations, and diagnostic evaluation. One was a case of recurrent IgE-mediated anaphylaxis that was self-induced by antigen exposure. The second was a case of recurrent laryngeal stridor that was produced consciously or unconsciously by a patient in the absence of organic disease of the larynx. DR. SCHATZ: The first case is that of a ZO-year-old unmarried white female student nurse who was referred to the allergy service because of an episode of an acute allergic reaction requiring hospitalization for several days. This reaction had been severe. Symptoms had consisted of urticaria, rhinitis, angioedema, asthma, and hypotension. When seen as an outpatient, her medical record showed that she had been seen in consultation two years previously. At that time she had given a history of a severe generalized reaction following the ingestion of walnuts From the Section of Allergy and Immunology, Department of Medicine, Northwestern Universitv Medical School. McGaw Medical Center. Supported by United States Public Health Service Grant No. AI-11403 and the Ernest S. Razley Grant. Received for publication Oct. IO, 1974. Reprint requests to: Roy Patterson, M.D., Professor and Chairman, Department of Medicine, Northwestern University Medical School, 303 E. Chicago Ave., Chicago, Ill. 60611. Vol.
56, No. 3, pp. 15.3-159
Factitious
VOLUME 56 NUMBER 2
TAELE I. Laboratory
allergic
emergencies
153
findings
A. Case 1: Anophyloxis
Normal complete blood count, differential count, sedimentation rate; SMA-12 chemistries, electrolvtrs Urinalysis: Tntermittent pyuria up to 30 white cells per high-power field small bowel, and intravenous Normal chest, upper gastrointestinal, lower gastrointestinal, pyelogram x-rays intermediate strength purified protein derivative; febrile Kegative urine and blood cultures; agglutinins; heterophile; hepatitis-associated antigen Segativc or normal antinuclear antibody, lupus erythematosus preps; total serum complement, C3, C4; immunoglobulins; antistreptolysin-0 titer, C-reactive protein, rheumatoid factor Immediate skin t,ests : Positive 4+ prick: ragweed, dust 3+ prick: dematiacieae mix, cat dander, peas, soybeans Negative intradermal : trees, grass, dog, feathers B. Case 2: Stridor Normal Normal Normal Normal Normal
complete blood count, differential count; S&IA-B, serum electrolytes chest, upper gastrointestinal, oral cholecystogram x-rays electroencephalogram total serum complement, C3, C4, Cl inhibitor; immunoglobulins insoiratorv and exniratorv uulmonarv functions
; urinalysis
and had been advised to avoid these with great caution. During the course of the second allergy evaluation consisting of history and physical examination, the patient developed an acute reaction with conjunctivitis, nasal congestion, rhinorrhea, unilateral facial angioedema, and bronchospasm. Incomplete clearing of symptoms with 0.3 C.C.of epinephrine and 50 mg. of diphenhydramine resulted in hospitalization with recovery in 2 days. The patient denied ingestion of any food or drug for at least 12 hours prior to the onset of these symptoms. During the next 4 weeks, 2 more mild reactions occurred (rhinitis, conjunctivitis, bronchospasm) with no history of nut ingestion. One of these was observed by us to include unilateral conjunctivitis and periorbital edema. One week later the patient returned for skin tests, the results of which are shown in Table I, A. Thirty minutes after the prick tests1 a reaction developed including rhinitis, unilateral eon junctivitis and periorbital edema, urticaria, and bronchospasm. She was treated with epinephrine, antihistamines, and prednisone with improvement and was observed for several hours but not hospitalized. The patient was then instructed in the self-administration of epinephrine, advised to keep a dietary history, and a rice and lamb diet was initiated.2 The possibility of factitious disease was discussed among the medical staff at this time. On a later ambulatory clinic visit another similar acute reaction occurred. After appropriate therapy, the patient was removed to an observation area and her belongings examined. The patient’s purse contained a plastic bag with cracked Brazil nuts and filberts. IJrgent psychiatric consultation and confrontation of the patient with the evidence of the cra.cked nuts led to the admission of nut ingestion on that occasion. She denied previous induction of anaphylaxis by purposeful nut ingestion, stating that the last and only episode of anaphylaxis she induced was precipitated by a breakup with her fianc6. She implied it was a type of suicide gesture. She was referred for psychiatric care at this time, although she was quite resistant to this.
154
Patterson
and
TABLE II. Recognized
J. ALLERGY CLIN. IMMUNOL. AUGUST 1975
Schatz
causes of anaphylaxis
and laryngeal
stridor
A. Anaphylmir3
1. 2. 3. 4. 5.
Drugs (antibiotics, analgesics, Hetrrologous sera Pollen extracts Venoms (insect, snake) Foods
B. Acute,
recurrent
laryngeal
rtridor’a
dyes, iodinated
contrast
media, hormones,
vaccines,
dextran)
8
IJetraction of the tongue Acute inflammatory disease (nonspecific laryngitis, epiglotitis, diphtheria, measles, whooping cough) Laryngeal spasm (hypocalcemia, multiple sclerosis, tetanus, tabes) Laryngeal edema (allergic, hereditary) Foreign body aspiration Retained secretions Ball-valve tumor of the larynx or trachea
laryngotracheohronchitis,
Shortly after this episode, she began to report a fever up to lOlo F. unassociated with other significant symptomatology. She reported that urinalyses performed by her consistently contained 3+ protein although urinalyses done in three different laboratories failed to confirm this. She was admitted to the hospital after 7 weeks of fever for evaluation of the fever of unknown origin. Rectal temperatures under observation revealed a fever of 100° to lOlo with a normal diurnal variation. Laboratory workup included in Table I, A did not reveal the cause of the fever, although bone marrow, liver biopsy, and laparotomy were not done. Over the next two months the fever was reported gradually to have disappeared. DR. PATTERSON: As this problem was evaluated medically, we were seriously concerned about this patient’s prognosis since the recurrent anaphylactic reactions were severe, unpredictable, and unexplained. All possible causes of anaphylaxis( Table II, R) were considered to see if they could be incriminated as causes in this case. There were no leads as far as apparent or hidden causes of anaphylaxis. During the course of the medical observation and retrospectively there were several clues that pointed to the possibility of factitious anaphylaxis: (1) There was no evident explanation for the recurrent anaphylaxis. Usually the cause of anaphylaxis is readily apparent because of the immediate-type onset after an agent is injected or ingested (Table II, A). The cases of anaphylaxis we have seen tlue to ingested antigens have been readily apparent.” This, of course, does not imply that we believe all recurrent episodes of this type are factitious if there is no readily apparent agent. (2) The patient had a history of nut anaphylaxis providing a mechanism for factitious disease induction. (3) The patient appeared to have an inappropriate attitude toward her illness. In spite of the obvious concern of the physicians involved and the instruction to use epinephrine and seek emergency medical care at the onset of symptoms, she expressed minimal concern about the illness or the prognosis. (4) An unusual pattern of attacks appeared. Three episodes occurred while the patient was visiting the outpatient
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155
allcrgp service. Although the attacks were not limited to these three, this appcared to be more t,han would be expected by chance during the total period of observation of 5 months. (5) Another major clue was that during three episodes observed by us, a u&nteral conjunctivitis and prriorbital edema occurred although the other clinical manifestations were generalized. A logical explanation appearctl to be that the patient was putting nut antigen in one eye. After confrontation, this was admitted by the patient although on only one occasion. (6) Finally, there was some evidence of unusual behavior. One example is when the patient announced during one hospitalization that she had not urinated for 21 hours and yet had only 200 ml. of urine from the bladder by catheterization. Another example of unusual behavior was the protcinuria that the pat,icnt cletected but we could not confirm. In addition, although the fcrcr was not proved to bc Sactitious, this remains a hrong possibility. It has been reported that there is a high incidence of multiple types of factitious disease in patients presenting with self-induced illnesses, and Peterstlorf and Bennett” state, “It is well, however, to remember that fever occurring in the presence of factitious disease is likely to bc the result of deliberate trirkery.” Although the patient admitted to only one self-exposure to nuts with the intent of inducing anaphylaxis, it is our opinion that several, and probably all, of the current episodes over the previous 5 months were self-induced. None have occurred since confrontation during a final evaluation period of 5 months. A second case of factitious disease has been described elsewhcrc in details and will bc reviewed here to demonstrate a second type of nonorganic symptomatology that was considered to be a medical emcrgcney bp a variety of physicians on various occasions. 1)~. SCHATZ: The second case is that of a 3%year-old married hut separated black woman who appeared in the emergency room in acute respiratory distress. The symptoms were tachypnca (45 breaths per minute) and marked inspiratory stridor. This was characterized by loud, high-pitched inspiratory sounds that wcrc localized to the lar,vnx by observation and auscultation with a stethoscope. A diagnosis of laryngeal obstruction was made, and immcdiatc therapy with cpincphrine, antihistamines, and intravenous hydrocortisone was instituted. The attack subsided and the patient was atlmittcd to the intensive care unit for observation. Examination of the vocal corals by indirect laryngoscopy showed no cvidcncc of obstruction. A detailed history was obtained with difficulty bccausc the patient appeared relatively incorllnillllicative. The following hist0r.v appeared reliable after repeated discussions. Several years of episodes o-f respiratory tlistress of the type requiring admission werr described. At least 15 emergcnep admissions to several hospitals were reported. Subsequently, 5 of these hospital admissions were confirmed by review of records from one of the hospitals listed by the patient. She described her attacks as occurring without any cridcnt precipitating factor and at any time of day. They never awakened her at night, however, or occurred while she was alone. There was no history of drug allergy or TgE-mcdiatcd respiratory disease. Because of her attaoks, she had stopped working 6 months previously and was never left alone by her mother or
156
Patterson
and
J. ALLERGY CLIN. IMMUNOL. AUGUST 1975
Schatz
TABLE III. Characteristics
of Munchausen’s
syndrome9
1. Feigned severe illness of a dramatic and emergency nature 2. Evidence of disease surreptitiously produced by self-mutilation 3. Evidence of many previous hospital procedures, particularly burr holes 4. Pathologic lying 5. Aggressive. unrulv behavior 6. Dzcarture from the hospital against medical advice 7. Background of many hospitalizations and extensive travel 8. Absence of any readily discernible ulterior motive
laparotomy
scars and cranial
grandmother because of their concern over her attacks. No other pertinent information was obtained by history. The laboratory analyses and examinations that were conducted are listed in Table I, B. DR. PATTERSON: The differential diagnosis of upper airway obstruction considered in the workup of this patient is listed in Table II, B.‘, 8 No definitive evidence for any of these etiologies was found. During the hospital stay, a second attack of the laryngeal stridor occurred. She was treated with epinephrine, aminophylline, and hydrocortisone with subsidence of the attack, and indirect laryngoscopy by an otorhinolaryngologist was negative. Bronchoscopy, performed to exclude laryngeal pathology or a ball valve tumor, was negative. After further hospital observation without additional attacks, she was discharged with instructions to return to the emergency service at the onset of symptoms. The absence of explanation for her attacks and the examination demonstrating a normal upper airway had suggested the possibility of nonorganic laryngeal stridor. The allergy service requested immediate consultation if the patient appeared in the emergency room with an attack of laryngeal stridor. A few days later the patient did appear with the same symptoms as on prior occasions. The high-pitched, crowing stridorous inspirations could be heard down the corridor from the emergency room. A rapid assessment of the patient was made and the following were noted: (1) The patient was lying comfortably in a semirecumbent position. (2) All vital signs were normal. (3) There was no cyanosis. (4) Immediate examination of her vocal cords by indirect laryngoscopy duri?zg the stridorous respirations demonstrated that they were normal. At this time we were convinced that the patient was consciously or unconsciously producing the stridorous respirations herself. The patient coughed periodically, apparently from the irritation induced by the stridorous respirations. After observing her cough, we attempted to use this as a method of distraction to demonstrate that normal inspirations could occur. The patient was encouraged vigorously to cough on several occasions. Her induced coughing episodes distracted her so that she took a normal breath immediately before and after the cough, and this was repeated on several occasions. We considered this the definitive proof of nonorganic stridor. The attack subsided when she was told that manipulation of her neck would induce a remission of her respiratory distress. The patient was referred to the psychiatry service. She was informed that her attacks of stridor were recognized by the physicians as being produced by
Factitious
VOLUME 56 NUMBER 2
TABLE IV. Hysterical Characteristic Age
sex stntus Personality Intellinence Psychopathology SuggrsCbility Delrree of morbidity
versus )
factitious
allergic
emergencies
157
i!iness’0 Factitious
Hysteria
Wide distribution Mainly female but not to samo extent as factitious Common in both married and single Often inadequate and with a ready tendency to dissociation Broad distribution but freauentlv found in the dull range ’ ” Symptoms commonly directed to immediate tangible gain Common though often ill-sustained Phvsicial suffering or threat to life not common-
Majority 15-25 Mainly female Much more common in single More effectiva Usually
than hysteria
average or ahovc
Not usually directed to immediate tangihle gain Rare Gross disfigurement or even thwat to life common
her, and psychiatric care was instituted. The patient has had no subsequent attacks. She has not admitted that she had consciously produced these attacks; hence we have not been absolutely certain that these attacks were actually factitious laryngeal stridor rather than conversion hysteria. DR. SCHATZ: Nonpsychotic feigned or self-induced diseases include Munchausen’s syndrome, malingering, conversion hysteria, and factitious illnesses. These syndromes have two main features in common. First, they result in a considerable degree of medical involvement and economic cost because of the hospitalizations, emergency room visits, and clinic visits. Second, definitive treatment of these illnesses requires the recognition of the nature of the problem and the initiation of appropriate psychiatric care. Although the preceding syndromes are similar, they often may be differentiated on clinical grounds. The characteristics of Munchausen’s syndrome are shown in Table III.g Although the dramatic presentations and multiple hospitalizations of our patients arc reminiscent of Munchausen’s syndrome (especially Case 2, inspiring the eponym “Munchausens stridoP), clearly the other features of the syndrome were not present. Malingering was originally defined to apply to military service personnel and involves the conscious simulation of disease in order to escape arduous, dangerous, or unpleasant duty, or to obtain financial compensation.1° Although our patient with anaphylaxis was consciously aware of producing her own illness and our patient with stridor may have been, we do not believe the resulting secondary gain was tangible enough in either instance to constitute malingering. Hysterical conversion reactions involve motivation at an entirely unconscious level. “A diagnosis of hysteria implies dissociation of a kind that limits the patient’s awareness of the mechanism of symptom dcvelopmcnt.“l” As mentioned before, our patient with stridor may have been an example of this type of patient. To be differentiated from the foregoing is a complex syndrome that has been called deliberate disabilitylO and that we would choose to categorize as factitious illness. This syndrome has been reported as dermatitis factitia,ll factitious fever,”
158
Patterson
and Schatz
J. ALLERGY CLIN. IMMUNOL. AUGUST 1975
occult factitial tl~yrotoxicosis~‘2 and factitial liypoglycemia.13 In these patients, the motivation is conscious but the ulterior motive not at all obvious. It seems to bc a syndrome involving young, single, female individuals”, lo, I13I3 with emotional irr~maturit~l”~ l2 but adequate personality structure2 and at least average intelligence.” These patients have been noted to often have a medical association”, lo, l” lack of and possess certain hysterical characteristicsll, I2 (“denial, inappropriate concern, overcooperativeness in the search for organic etiology, and uncoopcrativencss when the quest turned to psychiatric causes““). Based on these features, our patient with anaphylaxis would appear to fit this category quite prcciscly and as such represents to our knowledge the first patient with factitious anaphylaxis to be reported. Although it may be at times difficult to differentiate hysterical conversion reactions from factitious diseasc10 (as it is in our patient with stridor), Table IV’” shows certain differentiating features. Based on this table, our patient with stridor would appear to have many characteristics common to patients with hystcrical symptoms. Although formal intelligence or personality testing was performed in neither of our patients, it appeared to us that our patient with stridor was significantly less intelligent and had a less effective personality structure than did our patient with anaphylaxis. Although the secondary gain in our patient with stridor was not totally obvious, it did appear that her illness climinated the possibility of working and allowed her to be tot,ally dependent on her family and never to be alone. The tangible gain in our patient with anaphplaxis was much less clear. As described in the case report, our patient with strider certainly was suggestible in terms of the cesation of her acute attack. Finally, the two cases described here arc different from each other in terms of risk to the patient. The nut-induced anaphylaxis could have resulted in death if an cxces~ dose was taken or possibly if appropriate therapy had not been given. In contrast, because the laryngeal stridor was not laryngeal edema, it did not constitute any risk to the paCent. There is a paucity of information regarding the psychopathology in patients with factitious disease because these patients are in general quite resistant to exploratory psychotherapy. I1 Subconscious conflicts and defense mechanisms in these patients may revolve around feeling of inadequacy,l’s I2 seeking the sick role,ll dependency needs,12 self-punishment,ll, I2 introverted rage,‘* or limited repression. lo One study of such patients reported that they are motivated by their fear, guilt, or introverted rage to repeat complex behavior, which at least temporarily provides relief of anxiety. l5 It remains speculative what motives and early experiences shape this peculiar personality structure, although early impaired parental relationships may be important.14 Although confrontation (in the case of factitious illness) or insight psychotherapy (in the case of hysterical conversion reactions) do not always (perhaps even usually) provide favorable results,l”, I1 our patients have not to our knowledge had recurrences of their illnesses since the nature of their problem was diagnosed and presented to them. W’e cannot determine, however, whether this represents a “cure” or only a temporary remission.
Factitious
VOLUME 56 NUMBER 2
allergic
emergencies
159
REFERENCES 1 Booth, B, M., III : Diagnosis of immediate allergy, in Patterson, R., editor: Allergic tlis~,ases-iiiaguosis and management, Philadelphia, 1972, J. 13. Lippincott Company, p. 63. 2 Golhert. T. 11.: Food allergy and immunologic diseases of the gastrointestinal tract, irt Pattc&n, R., editor: Alle$ic diseases-diagnosis and manage&&t, Philadelphia, 1572, J. B. Lippincott Company, p. 362. 3 Kelly, mechanisms and treatment, J. F., and Patterson, R. : Anaphylaxis-course, .T. A. M. A. 227: 1431.1436, 1974. T. M., Patt,erson, R., and Pruzansky, J. J.: Systemic allergic reactions to ingested 4 Golbwt, antigcw, J. ALLERGY 44: 96.107, 1969. 5 Petersdorf, R. G., and Bennett, I. L.: Factitious fever, Ann. Intern. Med. 46: 1039-1062, 1957 Patterson, R., Schatz, M., and Horton, M.: Munchausen’s strider: Ronorganic laryngeal obstruction, Clin. Allergy 4: 309-312, 1974. Iiallantyne, J., and Groves, J., editors: Scott-Browns diseases of the ear, nose, and throat, London, 1971, Butterworth and Co., Ltd. Harvey, A. M., Johns, R. J., Owens, A. H., and Ross, R. S.: The principles and practice of nwdicine, New York, 1972, Appleton-Century-Crofts. 9 Ireland, P., Sapira, J. D., and Templeton, 13.: Munchausen’s syndrome, Am. J. Med. 43: 579-592,
1967.
Br. Med. 10 Haivkings, .T. R., J&es, K. S., Sim, M., and Tibbetts, R. W.: Deliberate disability, J. 1: 361-367, 1956. factitia, South. Med. J. 66: 1279-1285, M. H., and Abrams, H. S.: Dermatitis 11 Hallender, 1973.
thyrotoxicosis, 12 Rose, E., Sanders, T. P., Webb, W. L., and Hines, R. C.: Occult factitial Ann. Intern. Med. 71: 309-315, 1969. 13 Service, F. J., and Palumbo, P. J.: Factitial hypoglycemia, Arch. Intern. Med. 134: 336340, 1974. P. L., and Service, F. J.: Self-induced hypoglycemia: A review 14 Moore, G. L., McBurney, of psychiatric aspects and report of three cases, Psychiatry Med. 4: 301-311, 1973. Its relationship to 15 Cramer, B., Gershberg, M. R.., and Stern, M.: Munchausen syndrome: malingering, hysteria and the physician-patient relationship, Arch. Gen. Psychiatry 24: 573-578,
1971.
Factitious allergic emergencies: Anaphylaxis and laryngeal “edema” Roy Patterson,
M.D., and Michael
Schatz,
M.D. Chicngo,
111.
DR. PATTERSON:Systemic IgE-mediated reactions or diseases that mimic them constitute some of the most acute of medical emergencies. The medical management of such cases consists of immediate measures to maintain life and prevent progression of symptoms and subsequent measures to prevent a recurrence by attempting to discover the inducing agent or mechanism. TWO of the most serious reactions of this type in man in terms of their rapidity of onset and potentia,l to cause death include systemic allergic reactions and laryngeal edema. The latter may occur as the primary medical problem or as a component of the systemic allergic reaction. The systemic allergic reaction is the human counterpart of experimental anaphylaxis in animals. The term anaphylaxis has been restricted by some to the induced, experimental animal model. However, the similarity of the immunologic, cellular, and pharmacologic mechanisms in syst’emic allergic reactions in man and anaphylaxis in animals appears to justify the use of the term a?taphylaxis in the description of the human event. The current discussion will include a review of two cases of acute recurrent human reactions. Both cases constituted considerable problems in emergency management, multiple hospitalizations, and diagnostic evaluation. One was a case of recurrent IgE-mediated anaphylaxis that was self-induced by antigen exposure. The second was a case of recurrent laryngeal stridor that was produced consciously or unconsciously by a patient in the absence of organic disease of the larynx. DR. SCHATZ: The first case is that of a ZO-year-old unmarried white female student nurse who was referred to the allergy service because of an episode of an acute allergic reaction requiring hospitalization for several days. This reaction had been severe. Symptoms had consisted of urticaria, rhinitis, angioedema, asthma, and hypotension. When seen as an outpatient, her medical record showed that she had been seen in consultation two years previously. At that time she had given a history of a severe generalized reaction following the ingestion of walnuts From the Section of Allergy and Immunology, Department of Medicine, Northwestern Universitv Medical School. McGaw Medical Center. Supported by United States Public Health Service Grant No. AI-11403 and the Ernest S. Razley Grant. Received for publication Oct. IO, 1974. Reprint requests to: Roy Patterson, M.D., Professor and Chairman, Department of Medicine, Northwestern University Medical School, 303 E. Chicago Ave., Chicago, Ill. 60611. Vol.
56, No. 3, pp. 15.3-159
Factitious
VOLUME 56 NUMBER 2
TAELE I. Laboratory
allergic
emergencies
153
findings
A. Case 1: Anophyloxis
Normal complete blood count, differential count, sedimentation rate; SMA-12 chemistries, electrolvtrs Urinalysis: Tntermittent pyuria up to 30 white cells per high-power field small bowel, and intravenous Normal chest, upper gastrointestinal, lower gastrointestinal, pyelogram x-rays intermediate strength purified protein derivative; febrile Kegative urine and blood cultures; agglutinins; heterophile; hepatitis-associated antigen Segativc or normal antinuclear antibody, lupus erythematosus preps; total serum complement, C3, C4; immunoglobulins; antistreptolysin-0 titer, C-reactive protein, rheumatoid factor Immediate skin t,ests : Positive 4+ prick: ragweed, dust 3+ prick: dematiacieae mix, cat dander, peas, soybeans Negative intradermal : trees, grass, dog, feathers B. Case 2: Stridor Normal Normal Normal Normal Normal
complete blood count, differential count; S&IA-B, serum electrolytes chest, upper gastrointestinal, oral cholecystogram x-rays electroencephalogram total serum complement, C3, C4, Cl inhibitor; immunoglobulins insoiratorv and exniratorv uulmonarv functions
; urinalysis
and had been advised to avoid these with great caution. During the course of the second allergy evaluation consisting of history and physical examination, the patient developed an acute reaction with conjunctivitis, nasal congestion, rhinorrhea, unilateral facial angioedema, and bronchospasm. Incomplete clearing of symptoms with 0.3 C.C.of epinephrine and 50 mg. of diphenhydramine resulted in hospitalization with recovery in 2 days. The patient denied ingestion of any food or drug for at least 12 hours prior to the onset of these symptoms. During the next 4 weeks, 2 more mild reactions occurred (rhinitis, conjunctivitis, bronchospasm) with no history of nut ingestion. One of these was observed by us to include unilateral conjunctivitis and periorbital edema. One week later the patient returned for skin tests, the results of which are shown in Table I, A. Thirty minutes after the prick tests1 a reaction developed including rhinitis, unilateral eon junctivitis and periorbital edema, urticaria, and bronchospasm. She was treated with epinephrine, antihistamines, and prednisone with improvement and was observed for several hours but not hospitalized. The patient was then instructed in the self-administration of epinephrine, advised to keep a dietary history, and a rice and lamb diet was initiated.2 The possibility of factitious disease was discussed among the medical staff at this time. On a later ambulatory clinic visit another similar acute reaction occurred. After appropriate therapy, the patient was removed to an observation area and her belongings examined. The patient’s purse contained a plastic bag with cracked Brazil nuts and filberts. IJrgent psychiatric consultation and confrontation of the patient with the evidence of the cra.cked nuts led to the admission of nut ingestion on that occasion. She denied previous induction of anaphylaxis by purposeful nut ingestion, stating that the last and only episode of anaphylaxis she induced was precipitated by a breakup with her fianc6. She implied it was a type of suicide gesture. She was referred for psychiatric care at this time, although she was quite resistant to this.
154
Patterson
and
TABLE II. Recognized
J. ALLERGY CLIN. IMMUNOL. AUGUST 1975
Schatz
causes of anaphylaxis
and laryngeal
stridor
A. Anaphylmir3
1. 2. 3. 4. 5.
Drugs (antibiotics, analgesics, Hetrrologous sera Pollen extracts Venoms (insect, snake) Foods
B. Acute,
recurrent
laryngeal
rtridor’a
dyes, iodinated
contrast
media, hormones,
vaccines,
dextran)
8
IJetraction of the tongue Acute inflammatory disease (nonspecific laryngitis, epiglotitis, diphtheria, measles, whooping cough) Laryngeal spasm (hypocalcemia, multiple sclerosis, tetanus, tabes) Laryngeal edema (allergic, hereditary) Foreign body aspiration Retained secretions Ball-valve tumor of the larynx or trachea
laryngotracheohronchitis,
Shortly after this episode, she began to report a fever up to lOlo F. unassociated with other significant symptomatology. She reported that urinalyses performed by her consistently contained 3+ protein although urinalyses done in three different laboratories failed to confirm this. She was admitted to the hospital after 7 weeks of fever for evaluation of the fever of unknown origin. Rectal temperatures under observation revealed a fever of 100° to lOlo with a normal diurnal variation. Laboratory workup included in Table I, A did not reveal the cause of the fever, although bone marrow, liver biopsy, and laparotomy were not done. Over the next two months the fever was reported gradually to have disappeared. DR. PATTERSON: As this problem was evaluated medically, we were seriously concerned about this patient’s prognosis since the recurrent anaphylactic reactions were severe, unpredictable, and unexplained. All possible causes of anaphylaxis( Table II, R) were considered to see if they could be incriminated as causes in this case. There were no leads as far as apparent or hidden causes of anaphylaxis. During the course of the medical observation and retrospectively there were several clues that pointed to the possibility of factitious anaphylaxis: (1) There was no evident explanation for the recurrent anaphylaxis. Usually the cause of anaphylaxis is readily apparent because of the immediate-type onset after an agent is injected or ingested (Table II, A). The cases of anaphylaxis we have seen tlue to ingested antigens have been readily apparent.” This, of course, does not imply that we believe all recurrent episodes of this type are factitious if there is no readily apparent agent. (2) The patient had a history of nut anaphylaxis providing a mechanism for factitious disease induction. (3) The patient appeared to have an inappropriate attitude toward her illness. In spite of the obvious concern of the physicians involved and the instruction to use epinephrine and seek emergency medical care at the onset of symptoms, she expressed minimal concern about the illness or the prognosis. (4) An unusual pattern of attacks appeared. Three episodes occurred while the patient was visiting the outpatient
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allergic
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allcrgp service. Although the attacks were not limited to these three, this appcared to be more t,han would be expected by chance during the total period of observation of 5 months. (5) Another major clue was that during three episodes observed by us, a u&nteral conjunctivitis and prriorbital edema occurred although the other clinical manifestations were generalized. A logical explanation appearctl to be that the patient was putting nut antigen in one eye. After confrontation, this was admitted by the patient although on only one occasion. (6) Finally, there was some evidence of unusual behavior. One example is when the patient announced during one hospitalization that she had not urinated for 21 hours and yet had only 200 ml. of urine from the bladder by catheterization. Another example of unusual behavior was the protcinuria that the pat,icnt cletected but we could not confirm. In addition, although the fcrcr was not proved to bc Sactitious, this remains a hrong possibility. It has been reported that there is a high incidence of multiple types of factitious disease in patients presenting with self-induced illnesses, and Peterstlorf and Bennett” state, “It is well, however, to remember that fever occurring in the presence of factitious disease is likely to bc the result of deliberate trirkery.” Although the patient admitted to only one self-exposure to nuts with the intent of inducing anaphylaxis, it is our opinion that several, and probably all, of the current episodes over the previous 5 months were self-induced. None have occurred since confrontation during a final evaluation period of 5 months. A second case of factitious disease has been described elsewhcrc in details and will bc reviewed here to demonstrate a second type of nonorganic symptomatology that was considered to be a medical emcrgcney bp a variety of physicians on various occasions. 1)~. SCHATZ: The second case is that of a 3%year-old married hut separated black woman who appeared in the emergency room in acute respiratory distress. The symptoms were tachypnca (45 breaths per minute) and marked inspiratory stridor. This was characterized by loud, high-pitched inspiratory sounds that wcrc localized to the lar,vnx by observation and auscultation with a stethoscope. A diagnosis of laryngeal obstruction was made, and immcdiatc therapy with cpincphrine, antihistamines, and intravenous hydrocortisone was instituted. The attack subsided and the patient was atlmittcd to the intensive care unit for observation. Examination of the vocal corals by indirect laryngoscopy showed no cvidcncc of obstruction. A detailed history was obtained with difficulty bccausc the patient appeared relatively incorllnillllicative. The following hist0r.v appeared reliable after repeated discussions. Several years of episodes o-f respiratory tlistress of the type requiring admission werr described. At least 15 emergcnep admissions to several hospitals were reported. Subsequently, 5 of these hospital admissions were confirmed by review of records from one of the hospitals listed by the patient. She described her attacks as occurring without any cridcnt precipitating factor and at any time of day. They never awakened her at night, however, or occurred while she was alone. There was no history of drug allergy or TgE-mcdiatcd respiratory disease. Because of her attaoks, she had stopped working 6 months previously and was never left alone by her mother or
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Schatz
TABLE III. Characteristics
of Munchausen’s
syndrome9
1. Feigned severe illness of a dramatic and emergency nature 2. Evidence of disease surreptitiously produced by self-mutilation 3. Evidence of many previous hospital procedures, particularly burr holes 4. Pathologic lying 5. Aggressive. unrulv behavior 6. Dzcarture from the hospital against medical advice 7. Background of many hospitalizations and extensive travel 8. Absence of any readily discernible ulterior motive
laparotomy
scars and cranial
grandmother because of their concern over her attacks. No other pertinent information was obtained by history. The laboratory analyses and examinations that were conducted are listed in Table I, B. DR. PATTERSON: The differential diagnosis of upper airway obstruction considered in the workup of this patient is listed in Table II, B.‘, 8 No definitive evidence for any of these etiologies was found. During the hospital stay, a second attack of the laryngeal stridor occurred. She was treated with epinephrine, aminophylline, and hydrocortisone with subsidence of the attack, and indirect laryngoscopy by an otorhinolaryngologist was negative. Bronchoscopy, performed to exclude laryngeal pathology or a ball valve tumor, was negative. After further hospital observation without additional attacks, she was discharged with instructions to return to the emergency service at the onset of symptoms. The absence of explanation for her attacks and the examination demonstrating a normal upper airway had suggested the possibility of nonorganic laryngeal stridor. The allergy service requested immediate consultation if the patient appeared in the emergency room with an attack of laryngeal stridor. A few days later the patient did appear with the same symptoms as on prior occasions. The high-pitched, crowing stridorous inspirations could be heard down the corridor from the emergency room. A rapid assessment of the patient was made and the following were noted: (1) The patient was lying comfortably in a semirecumbent position. (2) All vital signs were normal. (3) There was no cyanosis. (4) Immediate examination of her vocal cords by indirect laryngoscopy duri?zg the stridorous respirations demonstrated that they were normal. At this time we were convinced that the patient was consciously or unconsciously producing the stridorous respirations herself. The patient coughed periodically, apparently from the irritation induced by the stridorous respirations. After observing her cough, we attempted to use this as a method of distraction to demonstrate that normal inspirations could occur. The patient was encouraged vigorously to cough on several occasions. Her induced coughing episodes distracted her so that she took a normal breath immediately before and after the cough, and this was repeated on several occasions. We considered this the definitive proof of nonorganic stridor. The attack subsided when she was told that manipulation of her neck would induce a remission of her respiratory distress. The patient was referred to the psychiatry service. She was informed that her attacks of stridor were recognized by the physicians as being produced by
Factitious
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TABLE IV. Hysterical Characteristic Age
sex stntus Personality Intellinence Psychopathology SuggrsCbility Delrree of morbidity
versus )
factitious
allergic
emergencies
157
i!iness’0 Factitious
Hysteria
Wide distribution Mainly female but not to samo extent as factitious Common in both married and single Often inadequate and with a ready tendency to dissociation Broad distribution but freauentlv found in the dull range ’ ” Symptoms commonly directed to immediate tangible gain Common though often ill-sustained Phvsicial suffering or threat to life not common-
Majority 15-25 Mainly female Much more common in single More effectiva Usually
than hysteria
average or ahovc
Not usually directed to immediate tangihle gain Rare Gross disfigurement or even thwat to life common
her, and psychiatric care was instituted. The patient has had no subsequent attacks. She has not admitted that she had consciously produced these attacks; hence we have not been absolutely certain that these attacks were actually factitious laryngeal stridor rather than conversion hysteria. DR. SCHATZ: Nonpsychotic feigned or self-induced diseases include Munchausen’s syndrome, malingering, conversion hysteria, and factitious illnesses. These syndromes have two main features in common. First, they result in a considerable degree of medical involvement and economic cost because of the hospitalizations, emergency room visits, and clinic visits. Second, definitive treatment of these illnesses requires the recognition of the nature of the problem and the initiation of appropriate psychiatric care. Although the preceding syndromes are similar, they often may be differentiated on clinical grounds. The characteristics of Munchausen’s syndrome are shown in Table III.g Although the dramatic presentations and multiple hospitalizations of our patients arc reminiscent of Munchausen’s syndrome (especially Case 2, inspiring the eponym “Munchausens stridoP), clearly the other features of the syndrome were not present. Malingering was originally defined to apply to military service personnel and involves the conscious simulation of disease in order to escape arduous, dangerous, or unpleasant duty, or to obtain financial compensation.1° Although our patient with anaphylaxis was consciously aware of producing her own illness and our patient with stridor may have been, we do not believe the resulting secondary gain was tangible enough in either instance to constitute malingering. Hysterical conversion reactions involve motivation at an entirely unconscious level. “A diagnosis of hysteria implies dissociation of a kind that limits the patient’s awareness of the mechanism of symptom dcvelopmcnt.“l” As mentioned before, our patient with stridor may have been an example of this type of patient. To be differentiated from the foregoing is a complex syndrome that has been called deliberate disabilitylO and that we would choose to categorize as factitious illness. This syndrome has been reported as dermatitis factitia,ll factitious fever,”
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occult factitial tl~yrotoxicosis~‘2 and factitial liypoglycemia.13 In these patients, the motivation is conscious but the ulterior motive not at all obvious. It seems to bc a syndrome involving young, single, female individuals”, lo, I13I3 with emotional irr~maturit~l”~ l2 but adequate personality structure2 and at least average intelligence.” These patients have been noted to often have a medical association”, lo, l” lack of and possess certain hysterical characteristicsll, I2 (“denial, inappropriate concern, overcooperativeness in the search for organic etiology, and uncoopcrativencss when the quest turned to psychiatric causes““). Based on these features, our patient with anaphylaxis would appear to fit this category quite prcciscly and as such represents to our knowledge the first patient with factitious anaphylaxis to be reported. Although it may be at times difficult to differentiate hysterical conversion reactions from factitious diseasc10 (as it is in our patient with stridor), Table IV’” shows certain differentiating features. Based on this table, our patient with stridor would appear to have many characteristics common to patients with hystcrical symptoms. Although formal intelligence or personality testing was performed in neither of our patients, it appeared to us that our patient with stridor was significantly less intelligent and had a less effective personality structure than did our patient with anaphylaxis. Although the secondary gain in our patient with stridor was not totally obvious, it did appear that her illness climinated the possibility of working and allowed her to be tot,ally dependent on her family and never to be alone. The tangible gain in our patient with anaphplaxis was much less clear. As described in the case report, our patient with strider certainly was suggestible in terms of the cesation of her acute attack. Finally, the two cases described here arc different from each other in terms of risk to the patient. The nut-induced anaphylaxis could have resulted in death if an cxces~ dose was taken or possibly if appropriate therapy had not been given. In contrast, because the laryngeal stridor was not laryngeal edema, it did not constitute any risk to the paCent. There is a paucity of information regarding the psychopathology in patients with factitious disease because these patients are in general quite resistant to exploratory psychotherapy. I1 Subconscious conflicts and defense mechanisms in these patients may revolve around feeling of inadequacy,l’s I2 seeking the sick role,ll dependency needs,12 self-punishment,ll, I2 introverted rage,‘* or limited repression. lo One study of such patients reported that they are motivated by their fear, guilt, or introverted rage to repeat complex behavior, which at least temporarily provides relief of anxiety. l5 It remains speculative what motives and early experiences shape this peculiar personality structure, although early impaired parental relationships may be important.14 Although confrontation (in the case of factitious illness) or insight psychotherapy (in the case of hysterical conversion reactions) do not always (perhaps even usually) provide favorable results,l”, I1 our patients have not to our knowledge had recurrences of their illnesses since the nature of their problem was diagnosed and presented to them. W’e cannot determine, however, whether this represents a “cure” or only a temporary remission.
Factitious
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REFERENCES 1 Booth, B, M., III : Diagnosis of immediate allergy, in Patterson, R., editor: Allergic tlis~,ases-iiiaguosis and management, Philadelphia, 1972, J. 13. Lippincott Company, p. 63. 2 Golhert. T. 11.: Food allergy and immunologic diseases of the gastrointestinal tract, irt Pattc&n, R., editor: Alle$ic diseases-diagnosis and manage&&t, Philadelphia, 1572, J. B. Lippincott Company, p. 362. 3 Kelly, mechanisms and treatment, J. F., and Patterson, R. : Anaphylaxis-course, .T. A. M. A. 227: 1431.1436, 1974. T. M., Patt,erson, R., and Pruzansky, J. J.: Systemic allergic reactions to ingested 4 Golbwt, antigcw, J. ALLERGY 44: 96.107, 1969. 5 Petersdorf, R. G., and Bennett, I. L.: Factitious fever, Ann. Intern. Med. 46: 1039-1062, 1957 Patterson, R., Schatz, M., and Horton, M.: Munchausen’s strider: Ronorganic laryngeal obstruction, Clin. Allergy 4: 309-312, 1974. Iiallantyne, J., and Groves, J., editors: Scott-Browns diseases of the ear, nose, and throat, London, 1971, Butterworth and Co., Ltd. Harvey, A. M., Johns, R. J., Owens, A. H., and Ross, R. S.: The principles and practice of nwdicine, New York, 1972, Appleton-Century-Crofts. 9 Ireland, P., Sapira, J. D., and Templeton, 13.: Munchausen’s syndrome, Am. J. Med. 43: 579-592,
1967.
Br. Med. 10 Haivkings, .T. R., J&es, K. S., Sim, M., and Tibbetts, R. W.: Deliberate disability, J. 1: 361-367, 1956. factitia, South. Med. J. 66: 1279-1285, M. H., and Abrams, H. S.: Dermatitis 11 Hallender, 1973.
thyrotoxicosis, 12 Rose, E., Sanders, T. P., Webb, W. L., and Hines, R. C.: Occult factitial Ann. Intern. Med. 71: 309-315, 1969. 13 Service, F. J., and Palumbo, P. J.: Factitial hypoglycemia, Arch. Intern. Med. 134: 336340, 1974. P. L., and Service, F. J.: Self-induced hypoglycemia: A review 14 Moore, G. L., McBurney, of psychiatric aspects and report of three cases, Psychiatry Med. 4: 301-311, 1973. Its relationship to 15 Cramer, B., Gershberg, M. R.., and Stern, M.: Munchausen syndrome: malingering, hysteria and the physician-patient relationship, Arch. Gen. Psychiatry 24: 573-578,
1971.
