Correspondence
Medical Mystery — The Answer Revealed
N Engl J Med 1998; 338:266-268January 22, 1998
- Article
To the Editor:
In the December 4 Image in Clinical Medicine my guess is that the twin on the right (Figure 1Figure 1
A Medical Mystery.)1 has Addison's disease. But that seems too obvious to be correct.1 ReferencesJohn K. Emy, M.D.
New York University Medical Center, New York, NY 100161
Frothingham R . A medical mystery. N Engl J Med 1997;337:1666-1666
Full Text | Web of Science | Medline
To the Editor:
“Do not fear me that I am bronzed, that the sun has tanned me. . . . Swarthy I am and beautiful, O daughters of Jerusalem.”1
Beautiful she is, and bronzed, too, but not by the sun. The patient is the twin on the right, who appears older than her sister and gaunt in comparison — sequelae of chronic adrenocortical deficiency (Addison's disease). But what of etiology?
Were Dr. Frothingham an endocrinologist, we might perhaps guess that the patient's adrenocortical deficiency is idiopathic. But the doctor's publication record suggests that he is a mycobacteriologist. We believe, therefore, that the patient has tuberculosis, with adrenal involvement and secondary adrenal insufficiency.
1 ReferencesRobert L. Deresiewicz, M.D.
Kevin M. Dushay, M.D.
Harvard Medical School, Boston, MA 02115To the Editor:
Does the blame lie deep within
That one twin looks dark and thin?
For sure there is no telling
Why her adrenal glands are failing.
Most likely, says the detective,
Autoimmunity made them defective.
Dietmar Schiller, M.D.
Elisabethinen Hospital, A-4010 Linz, AustriaTo the Editor:
How naughty to ask your readers to make a diagnosis from just a picture, but what a relief that you have not lost the sense of fun that used to enlighten medical life before everyone became so deadly serious.
With regards from a devoted reader since my resident days more than 55 years ago.
Richard Bayliss, M.D.
61 Onslow Sq., London SW7 3LS, United KingdomAuthor/Editor Response
Dr. Frothingham and the editor reply:
To the Editor: The patient on the right (an old friend of mine) has adrenal insufficiency, probably caused by autoimmune adrenocortical disease. She had fatigue for at least one year. During the preceding three months she had nausea, vomiting, and dizziness and lost 13 kg. A medical evaluation revealed only asymptomatic cholelithiasis, and she was scheduled for elective cholecystectomy. After seeing her in church and noting her hyperpigmentation, I suspected that she had adrenal insufficiency and brought her to the hospital. Her blood pressure and pulse were 85/40 mm Hg and 84 per minute, respectively, while she was sitting and 78/28 mm Hg and 124 per minute while she was standing. The remainder of the examination was normal except for the hyperpigmentation. The results of laboratory studies were as follows: serum sodium, 130 mmol per liter; potassium, 4.4 mmol per liter; cortisol, 1.0 μg per deciliter (normal, 5–28 μg per deciliter) at base line and 0.7 μg per deciliter one hour after cosyntropin injection. All the patient's symptoms responded promptly to combined glucocorticoid and mineralocorticoid replacement therapy, and her pigmentation gradually returned to normal.
Both women have had recent physical examinations and laboratory studies. Both have normal thyroid function, but their mother has hypothyroidism. Both have normal serum glucose concentrations now, but the twin on the left had gestational diabetes during the last two of her seven pregnancies.
Both women have had stable facial weakness since birth. The twin on the left is left-handed and has right facial weakness. She has a slightly asymmetric smile and cannot wink her right eye. The twin on the right is right-handed and has left facial weakness to the same degree.
Richard Frothingham, M.D.
Durham Veterans Affairs Medical Center, Durham, NC 27705-3875Author/Editor Response
In this “Medical Mystery,” we invited readers to tell us which twin was the patient and to propose her diagnosis. We received 1347 responses. A few interesting replies are given here. The tally: Addison's disease in the woman on the right, 832 votes; right facial weakness in the woman on the left, 53 votes; both Addison's disease in the woman on the right and facial weakness in the woman on the left (the best choice), 20 votes; incorrect diagnoses, 442 votes. A number of people offered the possibility of a polyglandular autoimmune disorder. Only further follow-up can determine whether this diagnosis is correct.
Jerome P. Kassirer, M.D.
