31-Year-Old Withdrawn Woman With Hand Discoloration

HISTORY A 31-year-old woman is seen by her physician because of mottled skin on the dorsa of the hands, low blood pressure, and a confusing array of abnormal physical findings and laboratory test results. She is evasive when queried about heavy menses. Will not disclose her sexual history. PHYSICAL EXAMINATION At first encounter, patient is withdrawn but conversationally capable; appearance as shown. Heart rate, 62 beats per minute and regular. Blood pressure (BP) in right arm, seated, 88/46 mm Hg. Orthostatic pulse and BP measurements not performed. Temperature, 35.5°C (95.9°F). LABORATORY TESTS Hematocrit, 29%. Mean corpuscular volume, 92 fL. White blood cell count, 3800/μL; platelet count, 136,000/μL. Serum creatinine, 0.6 mg/dL.   What's Your Diagnosis? Answer on next page , ANSWER: ANOREXIA NERVOSA As soon as this patient disrobed (Figure 1), her emaciation, which had been fairly effectively cloaked by her loose-fitting clothing, became painfully obvious. What was intentionally omitted from the history given above is that this woman was residing on a psychiatric unit because of known anorexia nervosa, and the internist who was consulted was asked about her “purpuric” hands. Anorexia and bulimia remain extremely common in our culture that is obsessed with thinness—over and above legitimate health concerns about obesity. Television, print advertisements, and the Internet bombard the public with the message that obesity is unsightly and offensive and represents both weakness of will and moral turpitude. The corollary is, “You can never be too rich or too thin,” a statement that is wildly misleading and that causes particular pain to those who see the effects of malnutrition—whether in the aged (in whom underweight is the leading nutritional disorder); in victims of famine in other countries; and in persons with anorexia. Although the health hazards of obesity constitute an enormous public health problem in the United States, the damage from the opposite problem—voluntary malnutrition—carries a higher case mortality per affected person. CAVEAT ABOUT CLOTHING AND HABITUS My involvement in the case illustrated began with a call, during my chief medical residency 22 years ago, from the inpatient psychiatric unit of my hospital, requesting that I see an anorectic patient who was ill. These photographs emphasize how well the patient hid her abnormalities. Resourceful secrecy is common and destructive in anorectic patients. Of course, once the patient’s body is seen, as in this case, the secret is revealed forever unless the patient continues to find new clinicians who do not communicate with the previous ones. This fact highlights 2 points—one obvious yet often disregarded because of the pressures of time, expediency, or a false sense of modesty—the other less well recognized: 1.  The patient must be undressed for any meaningful examination other than of the ears, mouth, and eyes. This might seem an unnecessary instruction, but research—often unpublished because no one wants to admit to the problem—shows that half of cardiac auscultations in some teaching hospitals are performed through clothing! Clearly, such practices introduce artifacts and mask findings. Television segments—whether they be news, medical dramas, or advertisements—often show auscultation that preserves modesty by being performed through clothing. This phenomenon serves to change societal expectations, and a method that distorts findings eventually becomes legitimized. Although it takes time and occasionally causes embarrassment, there is no alternative to exposing the field, as is vividly underscored by this case. 2.  Do not be misled by habitus: some persons with anorexia and bulimia may have near normal weight. (However, their metabolism will be grossly deranged if they balance binges with purges.) If an accurate history is forthcoming from either the patient or a parallel source, you need not hesitate to make the diagnosis—even in someone who is overweight. ORAL SIGNS Two oral signs of anorexia warrant mention: •Selective erosion of the posterior aspect of the dentition from repeated exposure to regurgitated stomach acid1 (Figure 2). This occurs only if self-induced vomiting is part of the complex. The dental literature refers to this sign as perimolysis. A differential effect on teeth (enamel) as compared with dental fillings is attributable to the relative acid solubility of the 2 materials. A similar finding can be produced in gastroesophageal reflux,2  so unless one is an expert in oral and dental diagnosis, it is probably wise to avoid diagnosing anorexia nervosa on the basis of enamel erosions alone. •Parotid enlargement.3  The differential diagnosis of this finding is considerable, but you can dismiss several of the other causes when the patient is a high school girl, a college student of either sex, or a young adult. Such young persons carry the greatest risk of anorexia nervosa, bulimia, and binge eating disorder.4-6 HAND AND ORTHOSTATIC SIGNS  Persons who stimulate vomiting by touching the pharynx and uvula may repeatedly abrade the dorsum of the hand they thrust into the throat. The sign consists of a highly characteristic, although not pathognomonic, set of indistinct tooth marks, shallow abrasions, and calluses.7  Although our patient had unexplained skin mottling on the hands, this feature was not present. Autonomic instability can be part of the complex of self-starvation.8  So can a variety of cardiac disorders that might predispose to tachycardia or that might require diuretic therapy that in turn could lead to orthostatic hypotension.9  In addition, patients with anorexia are often markedly volume-depleted—either because of a simple reduction in liquid intake or because laxative and/or diuretic abuse is part of their disorder. How does one decide which it is? Anorexia-related intravascular volume depletion from inadequate intake can itself cause orthostatic hypotension; however, the lack of a tachycardic response would not be expected unless vascular reflexes were impaired—and in this patient, the heart rate is suspiciously slow given the degree of hypotension. A persistently and inappropriately slow heart rate for degree of hypotesion suggests an eating disorder, particularly in association with such other characteristic features as hypothermia and unexplained leukopenia.10 ADDITIONAL MANIFESTATIONS Unexplained rectal prolapse, particularly in the highest risk population—namely, young women—can suggest anorexia nervosa.11  The study cited describes at least 4 physiologic strains on tissues supporting the rectal mucosa in persons with bulimia—a very high percentage of whom abuse laxatives and diuretics and also induce vomiting. These patients are constipated and exercise excessively. The vomiting itself transiently elevates intra-abdominal pressure, with the resulting tendency to “intussuscept” outward any poorly tethered portion of the rectal wall and mucosa.11 In one case that is bizarre even by the extraordinary standards of the bulimia literature, a woman habitually donated her blood, which she described as “filthy”— that is, polluted by eating and bingeing. She speculated that she could evaporate if only she were able to donate enough blood.12 These signs, along with features in the history and in screening laboratory studies,10  provide additional means to facilitate early recognition and diagnosis of anorexia nervosa despite the patient’s concealment strategies. The physical signs gain importance because the victim’s clever and effective disguise of the problem seems designed to help him or her avoid the assistance  that is so desperately needed. If this situation sounds familiar, it may be because the concealment of the disorder, the refusal to accept the diagnosis when it is made and explicated, and the intensely charged psychology of the encounter between physician and patient are familiar phenomena from alcoholism. Anorexia can be considered an even more difficult condition because the cultural gulf between patient and examiner often seems even wider than in the case of the alcoholic—with ready exploitation of such elements as “You can’t understand because you are not of my generation” and “You are a not a young girl—you have no idea.” COMPETING DIAGNOSTIC POSSIBILITIES Anorexia nervosa is often mistaken for something else, in part because it is painful for the clinician to observe such a devastating condition. Addisonism in its extreme form can produce a degree of cachexia similar to that seen in photographs of concentration camp survivors. (The deceased persons in such photographs are, if possible, even thinner and more upsetting than the survivor—both because of their thinness and because they have lost their lives.) So can panhypopituitarism—even though the hypothyroid component would tend to reduce caloric need. The concurrence of hypotension, bradycardia, and hypothermia will inevitably lead to consideration of Addisonism and “secondary” or pituitary Addisonism, so that vigilance is required to distinguish the two. Although this woman is younger than most persons with carcinomatosis, one sees a similar degree of catabolic damage in some persons during end-stage malignant disease. The lack of an apparent primary tumor site on physical examination, and of lymphadenopathy, reduces the likelihood of cancer, but of course does not rule it out completely. For example, small-cell carcinoma of the lung can lead to profound wasting with only a small primary and inapparent systemic metastases. The classic differential diagnosis of wasting and “Slim disease” in Africa and elsewhere includes tuberculosis and HIV infection.13  This patient had no coughing, so one would have to posit purely extrapulmonary or miliary disease if tuberculosis were present; however, she had no such condition. The negative results of a purified protein derivative (PPD) test are of little or no help, because the other findings constitute anergy, an expected immune dysfunction with this degree of wasting, regardless of the cause. It has more recently been recognized that several concomitants of defective response to infection are also common in anorexia. These include diminished febrile response in the presence of established bacterial infection and reduced local signs and symptoms at the site or sites of infection.14 I saw this patient before HIV infection had become widespread in the United States, and I believe she was not tested in view of her own preferences, lack of public health concern and, most important, a compelling and unrelated explanation for her wasting syndrome. CLINICAL FOLLOW-UP The patient’s husband reported that she moved to California and died of complications of anorexia several years after these photographs were made.  The lethality of anorexia nervosa became embedded in my thinking from a much earlier exposure when, as a pathologist in the 1980s, I performed an autopsy on an adolescent with anorexia who died of Pseudomonas aeruginosa  pneumonia. This occurred despite timely and intensive treatment and in the absence of any other medical problem that would predispose to a disorder much more characteristically seen in immunodebilitated persons after lengthy hospital exposure. The young girl who died of this pneumonia was not  leukopenic from her anorexia, by the way. WHAT IS NEWER Publications on anorexia proceed apace. New variants continue to be described: teenaged British girls of South Asian origin can have anorexia without the fatphobic verbalization that characterizes so many others with this condition.15  There is also a variant called orthorexia, wherein the concern to “eat right” and to “eat natural” becomes extended grotesquely until the diet is so constricted that anorexia eventuates.16 As befits the seriousness of the disorder and the refractoriness of the condition in so many cases, new studies on drug therapy appear regularly,17  and although the situation is grave, there are happy as well as tragic outcomes. Schneiderman H. Anorexia nervosa: a scourge often concealed by patients and revealed by physical examination. CONSULTANT. 2005;45:668-674. REFERENCES: 1. Peterson DS, Barkmeier WW. Oral signs of frequent vomiting in anorexia. Am Fam Physician. 1983;27:199-200. 2. Schroeder PL, Filler SJ, Ramirez B, et al. Dental erosion and acid reflux disease. Ann Intern Med. 1995;122:809-815. 3. Ahola SJ. Unexplained parotid enlargement: a clue to occult bulimia. Conn Med. 1982;46:185-186. 4. Fairburn CG, Cooper Z, Doll HA, et al. The natural course of bulimia nervosa and binge eating disorder in young women. Arch Gen Psychiatry. 2000;57:659-665. 5. Strober M, Freeman R, Morrell W. Atypical anorexia nervosa: separation from typical cases in course and outcome in a long-term prospective study. Int J Eat Disord. 1999;25:135-142. 6. Herzog W, Deter HC, Fiehn W, Petzold E. Medical findings and predictors of long-term physical outcome in anorexia nervosa: a prospective, 12-year follow-up study. Psychol Med. 1997;27:269-279. 7. Wynn DR, Martin MJ. A physical sign of bulimia. Mayo Clin Proc. 1984;59:722. 8. Rechlin T, Weis M, Ott C, et al. Alterations of autonomic cardiac control in anorexia nervosa. Biol Psych. 1998;43:358-363. 9. Olivares JL, Vazquez M, Fleta J, et al. Cardiac findings in adolescents with anorexia nervosa at diagnosis and after weight restoration. Eur J Pediatr. 2005 Mar 15. Epub ahead of print. 10. Miller KK, Grinspoon SK, Ciampa J, et al. Medical findings in outpatients with anorexia nervosa. Arch Intern Med. 2005;165:561-566. 11. Malik M, Stratton J, Sweeney WB. Rectal prolapse associated with bulimia nervosa: report of seven cases. Dis Colon Rectum. 1997;40:1382-1385. 12. Ghadirian AM. Bulimic purging through blood donation. Am J Psychiatry. 1997;154:435-436. 13. Schneiderman H. “Slim disease” and AIDS-related cachexia. Consultant. 1995;35:379-382. 14. Brown RF, Bartrop R, Beumont P, Birmingham CL. Bacterial infections in anorexia nervosa: Delayed recognition increases complications. Int J Eat Disord. 2005;37:261-265. 15. Tareen A, Hodes M, Rangel L. Non-fat-phobic anorexia nervosa in British South Asian adolescents. Int J Eat Disord. 2005;37:161-165. 16. Catalina Zamora ML, Bote Bonaechea B, Garcia Sanchez F, Rios Rial B. Orthorexia nervosa. A new eating behavior disorder? [in Spanish]. Actas Esp Psiquiatr. 2005;33:66-68. 17. Mondraty N, Birmingham CL, Touyz S, et al. Randomized controlled trial of olanzapine in the treatment of cognitions in anorexia nervosa. Australas Psychiatry. 2005;13:72-75.