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LETTERS Behcet’s syndrome and palate perforation To the Editor: Behcet described the relapsing symptom triad of aphthous stomatitis, genital ulcerations, and iridocyclitis (1). This syndrome also produces the histopathologic changes of vasculitis in other areas, thus it is a systemic disease (2). Herein we report a patient with Behcet’s syndrome and an unusual complication. In April, 1975 a 26-year-old male had fever and oral ulcerations lasting 5 days. Four months later the same symptoms were observed and in April 1976, conjunctivitis and diplopia with paralysis of the right lateral rectus muscle were noted. In January 1977 the patient had a new flareup, losing 15 kg of body weight as a consequence of multiple and painful oral ulcers that discouraged eating. Results of the ophthalmologic examination were normal. Laboratory tests showed: leukopenia of 2,900/mm3 and 1,298 lymphocytes/mm3. Forty-six percent of the lymphocytes formed E rosettes (normal 66.37 f 1.89). EAC rosettes were within normal limits. Skin tests (PPD, coccidioidin, histoplasmin, SIC-SD) were negative, as were bacterial and fungal cultures; cryoglobulins, antiDNA antibodies (Farr), and antinuclear antibodies by indirect immunofluorescence were not found. Radiologic and scintiscan studies of sacroiliac joints and paranasal sinuses were normal. Biopsy from oral ulcers disclosed perivascular infiltration of lymphocytes and polymorphonuclear leukocytes; immunofluorescent staining failed to identify deposits of immunoglobulins. HLA-B5 was demonstrated by microlymphocytotoxicity. In May 1977 the patient had fever, headache, paresthesias in the legs and hands, ulcers of the penis foreskin, conjunctivitis, and regurgitation of food into the nasal cavity. An ulcer perforating the soft palate was observed. Prednisone was increased to 60 mg daily and 10 mg of chlorambucil were started. The diagnosis of Behcet’s syndrome was established by the presence of the three major criteria: oral ulcers, genital ulcers, and ocular lesions, plus other systemic manifestations, all of which have been reported in this condition. Mucocutaneous and colonic ulcerations in general are shallow and not larger than 1 to 1.5 cm; no report is known describing perforation. The complication Arthritis and Rheumatism, Vol. 22, No. 3 (March 1979)
of soft palate perforation is important in the differential diagnosis, since this has been described in systemic lupus erythematosus, Wegener’s granulomatosis, lethal midline granuloma, mycotic infections, and neoplasia. All these diseases were ruled out in our case by clinical and laboratory parameters. CARLOS LAVALLE, M.D. JESUSGUDINO,M.D. SERGIOR. REINOSO, M.D. JORGEALCOCER,M.D. ANTONIO FRAGA,M.D. Servicio de Reumatologia, Hospital General, Centro Medico La Raza IMSS A venida Vallejo y Jacarandas Mexico, I S , DF
REFERENCES 1. Behcet H: Uber rezidivierende, aphthose, durch einen Virus verursachte Geschwure am Mund, am Auge und anden Genitalien. Derm Wschr 105:1152-1157, 1937 2. ODuffy JD, Canney JA, Deodhar S: Behcet’s disease. Ann Intern Med 7556 1-570, 1971 3. Mason RM, Barnes CG: Behcet’s syndrome with arthritis. Ann Rheum Dis 28:95-103, 1969
Cost of nerve conduction studies in carpal tunnel syndrome To the Editor: Dr. Joseph Rogoff s letter (1) is indicative of the thinking that has caused health care costs to soar in this country. He objects to the fact that Drs. Ahmed and Braun treated 4 of their 5 patients with carpal tunnel syndrome and polymyalgia rheumatica with corticosteroid injections into the carpal tunnel without bothering to obtain nerve conduction studies. All of the patients responded, although 1 or 2 needed later repeat injections (2). Dr. Rogoff evidently feels that any ambulatory patient with carpal tunnel symptoms should be dispatched from the office without treatment and scheduled for nerve conduction studies at a cost of $75 or more. When these results are obtained, the patient should return to the rheumatologist’s office to pay another office visit fee and possibly have an injection into
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the carpal tunnel. This type of expensive and useless diagnostic overkill when rapid simple treatment is available represents one of the major problems in American medicine, especially “academic” medicine. Carpal tunnel syndrome can usually be diagnosed accurately on clinical examination. Injection treatment is simple and frequently effective. Perhaps an argument can be made for electrodiagnostic studies prior to surgery or if there is some diagnostic question. Otherwise they are unnecessary and a waste of money. LONNIEB. HANAUER, MD, FACP 116 Millburn Ave. Millburn, NJ 07041
nerve diseases (and carpal tunnel syndrome is one) might consider doing the conduction studies himself. The technique should be available to these physicians and could be carried out in the office at the time of the original visit. Indeed, electrodiagnosis is a clinical, not a lab, procedure and is as much a part of the clinical evaluation as the reflex hammer, the pin, or the manual muscle test. There is another obvious reason for electrodiagnosis in this particular case. Drs. Ahmed and Braun describe the corticosteroid treatment for carpal tunnel syndrome as a “curative” treatment. However, a scientific article should include clear definition of the condition. Without electrodiagnosis, neither the reality of the condition nor its cure can really be accepted.
REFERENCES 1. Rogoff JB:
Electrical studies in carpal tunnel syndrome. Arthritis Rheum 21:865, 1978 2. Ahmed T, Braun AI: Carpal tunnel syndrome with polymyalgia rheumatica. Arthritis Rheum 2 1:22 1-223; 1978
JOSEPHB. ROGOFF,MD Director of Rehabilitation Medicine Professor, New York Medical College
Gold and the immune system Reply To the Editor: Dr. Hanauer’s letter appears to indicate that he has little experience with electrodiagnosis evaluation. I might use his same words-how many patients received expensive corticosteroid injections ($100.00) unnecessarily on the basis of an incorrect diagnosis of carpal tunnel syndrome? In many cases, in my experience, where the clinical diagnosis of carpal tunnel syndrome is evident, the electrical findings are so severe that corticosteroid injections are inadequate and surgery should be undertaken to prevent denervation atrophy of the denervated muscles. Where the carpal tunnel syndrome is so slight as to be “cured” by the injection, a trial of sleeping with a dorsiflexion splint may also effect a cure,” without the possibly unnecessary and expensive injection. The involvement may also not be carpal tunnel syndrome but something else. The real question is that of demonstrating the reality of the carpal tunnel syndrome, which, in my experience, might turn out to be peripheral neuropathy, cervical outlet syndrome, cervical root compression, or some other condition. Electrodiagnosis is extremely useful for defining the exact involvement. As for sending the patient for the electrodiagnosis, I think any physician caring for peripheral ‘C
To the Editor: The recent publication by Lorber et a1 (1) was strongly suggestive that chrysotherapy has a direct effect on the immune system and that this effect was immunosuppressive in nature. Although the authors are correct in stating that the mechanism of action of gold compounds in rheumatoid arthritis is unknown, there are a number of articles that indicate an effect on the immune system. Whether the effect is direct or indirect is unknown. Both Scheiffarth et a1 (2) and I (3) were able to show that gold had a direct effect on the immune system as assayed by plaque-forming cells. These studies, which used exogenous antigens, showed that the immune system was affected in both IgM and IgG antibody production. Although both of these short term animal experiments measured immunoenhancement, unpublished results from this laboratory have shown that high doses of gold are immunosuppressive when given over a longer time. In all probability, the mechanism by which gold causes an immunosuppressive effect is a dose-dependent extension of the mechanism that causes immunoenhancement. This biphasic response is not unknown in nature. It would be tempting to postulate that gold salts assert their beneficial effect on patients by destruction of B cells which produce rheumatoid factor and/or other deleterious antibodies. However, Gerber et a1 (4) have shown that gold preparations delay the develop-
LETTERS Behcet’s syndrome and palate perforation To the Editor: Behcet described the relapsing symptom triad of aphthous stomatitis, genital ulcerations, and iridocyclitis (1). This syndrome also produces the histopathologic changes of vasculitis in other areas, thus it is a systemic disease (2). Herein we report a patient with Behcet’s syndrome and an unusual complication. In April, 1975 a 26-year-old male had fever and oral ulcerations lasting 5 days. Four months later the same symptoms were observed and in April 1976, conjunctivitis and diplopia with paralysis of the right lateral rectus muscle were noted. In January 1977 the patient had a new flareup, losing 15 kg of body weight as a consequence of multiple and painful oral ulcers that discouraged eating. Results of the ophthalmologic examination were normal. Laboratory tests showed: leukopenia of 2,900/mm3 and 1,298 lymphocytes/mm3. Forty-six percent of the lymphocytes formed E rosettes (normal 66.37 f 1.89). EAC rosettes were within normal limits. Skin tests (PPD, coccidioidin, histoplasmin, SIC-SD) were negative, as were bacterial and fungal cultures; cryoglobulins, antiDNA antibodies (Farr), and antinuclear antibodies by indirect immunofluorescence were not found. Radiologic and scintiscan studies of sacroiliac joints and paranasal sinuses were normal. Biopsy from oral ulcers disclosed perivascular infiltration of lymphocytes and polymorphonuclear leukocytes; immunofluorescent staining failed to identify deposits of immunoglobulins. HLA-B5 was demonstrated by microlymphocytotoxicity. In May 1977 the patient had fever, headache, paresthesias in the legs and hands, ulcers of the penis foreskin, conjunctivitis, and regurgitation of food into the nasal cavity. An ulcer perforating the soft palate was observed. Prednisone was increased to 60 mg daily and 10 mg of chlorambucil were started. The diagnosis of Behcet’s syndrome was established by the presence of the three major criteria: oral ulcers, genital ulcers, and ocular lesions, plus other systemic manifestations, all of which have been reported in this condition. Mucocutaneous and colonic ulcerations in general are shallow and not larger than 1 to 1.5 cm; no report is known describing perforation. The complication Arthritis and Rheumatism, Vol. 22, No. 3 (March 1979)
of soft palate perforation is important in the differential diagnosis, since this has been described in systemic lupus erythematosus, Wegener’s granulomatosis, lethal midline granuloma, mycotic infections, and neoplasia. All these diseases were ruled out in our case by clinical and laboratory parameters. CARLOS LAVALLE, M.D. JESUSGUDINO,M.D. SERGIOR. REINOSO, M.D. JORGEALCOCER,M.D. ANTONIO FRAGA,M.D. Servicio de Reumatologia, Hospital General, Centro Medico La Raza IMSS A venida Vallejo y Jacarandas Mexico, I S , DF
REFERENCES 1. Behcet H: Uber rezidivierende, aphthose, durch einen Virus verursachte Geschwure am Mund, am Auge und anden Genitalien. Derm Wschr 105:1152-1157, 1937 2. ODuffy JD, Canney JA, Deodhar S: Behcet’s disease. Ann Intern Med 7556 1-570, 1971 3. Mason RM, Barnes CG: Behcet’s syndrome with arthritis. Ann Rheum Dis 28:95-103, 1969
Cost of nerve conduction studies in carpal tunnel syndrome To the Editor: Dr. Joseph Rogoff s letter (1) is indicative of the thinking that has caused health care costs to soar in this country. He objects to the fact that Drs. Ahmed and Braun treated 4 of their 5 patients with carpal tunnel syndrome and polymyalgia rheumatica with corticosteroid injections into the carpal tunnel without bothering to obtain nerve conduction studies. All of the patients responded, although 1 or 2 needed later repeat injections (2). Dr. Rogoff evidently feels that any ambulatory patient with carpal tunnel symptoms should be dispatched from the office without treatment and scheduled for nerve conduction studies at a cost of $75 or more. When these results are obtained, the patient should return to the rheumatologist’s office to pay another office visit fee and possibly have an injection into
309
LETTERS
the carpal tunnel. This type of expensive and useless diagnostic overkill when rapid simple treatment is available represents one of the major problems in American medicine, especially “academic” medicine. Carpal tunnel syndrome can usually be diagnosed accurately on clinical examination. Injection treatment is simple and frequently effective. Perhaps an argument can be made for electrodiagnostic studies prior to surgery or if there is some diagnostic question. Otherwise they are unnecessary and a waste of money. LONNIEB. HANAUER, MD, FACP 116 Millburn Ave. Millburn, NJ 07041
nerve diseases (and carpal tunnel syndrome is one) might consider doing the conduction studies himself. The technique should be available to these physicians and could be carried out in the office at the time of the original visit. Indeed, electrodiagnosis is a clinical, not a lab, procedure and is as much a part of the clinical evaluation as the reflex hammer, the pin, or the manual muscle test. There is another obvious reason for electrodiagnosis in this particular case. Drs. Ahmed and Braun describe the corticosteroid treatment for carpal tunnel syndrome as a “curative” treatment. However, a scientific article should include clear definition of the condition. Without electrodiagnosis, neither the reality of the condition nor its cure can really be accepted.
REFERENCES 1. Rogoff JB:
Electrical studies in carpal tunnel syndrome. Arthritis Rheum 21:865, 1978 2. Ahmed T, Braun AI: Carpal tunnel syndrome with polymyalgia rheumatica. Arthritis Rheum 2 1:22 1-223; 1978
JOSEPHB. ROGOFF,MD Director of Rehabilitation Medicine Professor, New York Medical College
Gold and the immune system Reply To the Editor: Dr. Hanauer’s letter appears to indicate that he has little experience with electrodiagnosis evaluation. I might use his same words-how many patients received expensive corticosteroid injections ($100.00) unnecessarily on the basis of an incorrect diagnosis of carpal tunnel syndrome? In many cases, in my experience, where the clinical diagnosis of carpal tunnel syndrome is evident, the electrical findings are so severe that corticosteroid injections are inadequate and surgery should be undertaken to prevent denervation atrophy of the denervated muscles. Where the carpal tunnel syndrome is so slight as to be “cured” by the injection, a trial of sleeping with a dorsiflexion splint may also effect a cure,” without the possibly unnecessary and expensive injection. The involvement may also not be carpal tunnel syndrome but something else. The real question is that of demonstrating the reality of the carpal tunnel syndrome, which, in my experience, might turn out to be peripheral neuropathy, cervical outlet syndrome, cervical root compression, or some other condition. Electrodiagnosis is extremely useful for defining the exact involvement. As for sending the patient for the electrodiagnosis, I think any physician caring for peripheral ‘C
To the Editor: The recent publication by Lorber et a1 (1) was strongly suggestive that chrysotherapy has a direct effect on the immune system and that this effect was immunosuppressive in nature. Although the authors are correct in stating that the mechanism of action of gold compounds in rheumatoid arthritis is unknown, there are a number of articles that indicate an effect on the immune system. Whether the effect is direct or indirect is unknown. Both Scheiffarth et a1 (2) and I (3) were able to show that gold had a direct effect on the immune system as assayed by plaque-forming cells. These studies, which used exogenous antigens, showed that the immune system was affected in both IgM and IgG antibody production. Although both of these short term animal experiments measured immunoenhancement, unpublished results from this laboratory have shown that high doses of gold are immunosuppressive when given over a longer time. In all probability, the mechanism by which gold causes an immunosuppressive effect is a dose-dependent extension of the mechanism that causes immunoenhancement. This biphasic response is not unknown in nature. It would be tempting to postulate that gold salts assert their beneficial effect on patients by destruction of B cells which produce rheumatoid factor and/or other deleterious antibodies. However, Gerber et a1 (4) have shown that gold preparations delay the develop-
