Recurrent Pericarditis and Dermatitis Herpetiformis

has its greater mass inferior to the thoracic inlet. I Seventyeight to 80 percent of these masses descend into the anterior mediastinum while the remainder descend into the posterior mediastinum with the middle mediastinal goiters rarely en<--ountered. J The most <--ommon symptoms associated with intrathoracic goiters are dyspnea, stridor, dysphagia, hoarseness, coughing, wheezing, and cervical mass. Interestingly, in addition to mechanical findings, the presented case also had neurologic and biochemical findings. The case presented here had a large intrathoracic goiter and most of the symptoms were due to compression on adjacent structures. Dyspnea and dysphagia were due to compression on trachea and esophagus, respectively. There was no tracheal deviation which is caused by bilateral conlpression. Horner's syndrome secondary to thyroid disease may be seen. It usually results from cervical sympathetic chain invasion by malignancies, but secondary to sympathetic chain <-'ompression with multinodular goiter has also been reported.· We think that our patient's neurologic symptoms were due to <--ompression and would be resolved by resection of the Inass. Superior vena cava syndrome with intrathoracic goiter has been reported. 5 It is seen with malignancy more frequently than with benign intrathoracic goiter. Positional dilated veins may simulate SVCS and should be differentiated. Hyperthyroidism with intrathoracic goiter has been reported in up to 20 percent of case reports,6 but it has been reported in recent series only occasionally. I It is interesting that our patient had all these combined findings. Our patient had clinical manifestations of hyperthyroidism, confirmed by thyroid function tests, probably due to using propylthiouracil before admission to the hospital. She had mild symptoms and laboratory findings. If the patients have not had any compression symptoms, radioactive iodine treatnlent may be used. As in our patient who has compression symptoms, operative treatment should be required. j

REFERENCES Katlic MR, \Vang CA, Grillo HC. Substernal goiter. Ann Thorac Surg 1985; 39:391-99 2 Hicks GL. The ilnpossihle intubation - \Vhat next. J Cardiovasc Surg 1986; 27:737-39 3 Frazer RG, Pare

JA~

eds. Diseases of the mediastinum. In:

Diagnosis of the chest. Philadelphia: WB Saunders, 1978: 17931870 4 Levin R, Newman SA, Login IS. Bilateral Horner's syndrome

secondary to multinodular goiter. Ann Intern Med 1986; 105:55051 5 Sy WM, Lao RS, Seo IS. Scintigraphic features of superior vena caval obstnlction due to substernal nontoxic goiter. Br J Radiol 1982; 55:301-03 6 Allo MD, 1C:nnpson N\~

Rationale for the operative management

of the mediastinum. Chest 1972; 62:297-302

1006

Recurrent Pericarditis and Dermatitis Herpetiformis* Evidence for Immune Complex Deposition in the Pericardium Rahmat Afrasiabi, M.D.; Paul A. Simp, M.D., F.C.C.R; S. Mark Albini, M.D.; Haskell M. Rosenbaum, M.D.; and Robert L. PiscateUi, M. D.

Recurrent pericarditis can be associated with many chronic illnesses. Dermatitis herpetiformis is a chronic papulovesicular eruption which is characterized by granular 19A deposits in the dermal papillary tips and associated with a gluten-sensitive enteropathy. We describe the first case of recurrent pericarditis in association with dermatitis herpetiformis. This supposition is supported by exclusion of other possible etiologies and pericardial biopsy which revealed the deposition of IgG, 19A and complement. (Chest 1990; 97:1006-07)

I ANA =antinuclear antibody I 60-year-old white woman with a 30-year history of dermatitis herpetiformis developed recurrent pericarditis and malabsorption. Dermatitis herpetiform is is a chronic papulovesicular eruption characterized by granular 19A deposits in the dermal papillary tips. It is often associated with a gluten-sensitive enteropathy and a high occurence of serum HLA-BB and HLA-Dw3. A biopsy of this patient's skin lesions revealed 19A deposits in the dermal layer of the skin and H LA typing was positive for HLA-BB. Open pericardial biopsy revealed the presence ofIgA, IgG and complement deposition in the pericardium. This is the first case of recurrent pericarditis in association with dermatitis herpetiformis and malabsorption suggesting a common immunologic denominator in a multisystem disorder. ~

1"1.

CASE REPORT

A 60-year-old white woman with dermatitis herpetiformis presented with sudden onset of pleuritic chest pain and a threecomponent friction nIb in association with an exacerbation of her skin lesions. The patient had presented with three similar episodes in the previous seven years. She denied symptoms of upper respiratory infection; however, she had a tuberculous infection 33 years earlier for which she received a 12-month course of isoniazid and para-aminosalicylic acid. She first presented to us eight years ago with cardiac irritability (ventricular fibrillation, resuscitated twice) and chest pain. She was evaluated at that time with cardiac catheterization and angiography which demonstrated cardiomyopathy but no evidence of coronary artery disease. Since that time, she has had multiple episodes of pericarditis. Because of her extreme ventricular irritability, an endomyocardial biopsy was not attempted. Results of an AN A screen and titer, LE preparation, ESR, rheumatoid arthritis screen, extractable nuclear antigens and anti- DNA binding capacity were all within normal limits or negative. Computerized axial tomography of the heart revealed normal pericardial thickness with no evidence of calcification. Open peri*From the Departments of Medicine and Pathology, St. Mary's Hospital, Waterbury, cr. Reprint requests: Dr. Piscatelli, Department of Medicine, St. Marys Hospital, Waterbury, CT 06706 Recurrent Pericarditis and Dermatitis Herpetiformis (Afrasiabi et al)

Table 1-Resulta of Immunologic Studies Test

Units

Result

Reference Range

Immunoelectrophoresis IgG 600 mwdl (750-1780) (180-480) IgA 153 IgM 110 (55-170) 1-356 (470-1300) IgG subclass 2-186 (115-750) (20-130) 3-19 4-9.7 (2-165) (640-1350) Total652 Negative Antireticulin AB C 3-4Il Serum complement mwdl (65-130) C.--y (20-40) A-Locus: 3, 28 HLA typing B-Locus: 8,60 C-Locus: CW3, CW7

cardial biopsy showed no evidence of granuloma, acute or chronic inflammation, amyloid or other infiltrative disease usually associated with cardiomyopathy. However, immunohistochemical studies on the pericardial tissue revealed IgG, 19A and complement deposition on the mesodermal surface and deeper areas of the pericardium. Depositions of granular deposits of 19A at the dermal-epidermal junction and faint continuous fine granular deposits of IgG at the same junctions were noted on the skin biopsy ofher active dermatitis herpetiformis lesions. Analysis of pericardial tissue from seven routine autopsy cases revealed no such deposition except in one case where the patient had IgG and 19A without complement deposition and suffered from AIDS. A more extensive immunologic investigation including HLA typing, immunoelectrophoresis, IgG subclasses, antireticulin antibody, and serum complements were undertaken (Table 1). Results indicated that she had HLA-B8 and low levels of IgG. and IgG3' The complement components C3 and C4 in the serum were markedly depressed (Table 1). Because ofabdominal pain, she underwent upper gastrointestinal endoscopy, cr scanning of the abdomen and endoscopic pancreaticoduodenography. These were normal although the pancreas was described as enlarged. Malabsorption testing showed an abnormal d-xylose test and increased fecal fat (Table 2). Biopsy of the third portion of the duodenum revealed mild chronic inRammation with minimal Rattening of the villi. Treatment consisted of oral steroids, dapsone and a gluten-free diet. Coincident with this therapy, the pericarditis, abdominal pain and dermatitis herpetiformis have become quiescent. DISCUSSION

Table 2-Reaulta ofMalab.orption Studies

D-xylose, urine D-xylose, serum Fecal lipids, (quantitative)

Result 2.2 22.8

13124 hr

Units g mwdl gI24 hours

*One hour normal subjects after a 25 g dose.

REFERENCES Laine LA, Holt KM. Recurrent pericarditis and ("eliac disease. JAMA 1984; 252:3168

This patient represents the first case with clear cut evidence of the immunologic linkage between dermatitis herpetiformis and recurrent pericarditis. Recurrent pericarditis in association with adult celiac disease has been reported.1,2 Dermatitis herpetiformis and adult celiac dis-

Test

ease exhibit several common features supporting the concept that these diseases share common immunopathologic Illechanisms. These include clinical remission in respollse to a gluten-free diet, HLA-BB association, and similar ~astroin­ testinal abnormality in 60 to 75 percent of patients with dermatitis herpetiformis. 3 The absence of any evidence of collagen vascular disease such as SLE, viral, bacterial, fungal or myobacterial infections, and infiltrative and granulomatous disease in our patient, as well as the presence of immune complexes in the pericardium and skin support an altered immunity and immunologic basis for this association. The circulatin~ immune complexes containing 90 KD gly(,'oprotein, and a component of skin and intestinal mucosa, have het~1l demonstrated in patients with dermatitis herpetiformis. • Such complexes have been shown to have cytotoxic and inUllUnogenic potential resulting in antigluten and anti-OO KD glycoprotein antibodies. S Whether there is a cross reactivity between gluten, 90 KD glycoprotein antigens and pericardial tissue predisposing to recurrent pericarditis and immune (,'Omplex deposition is not known. However, clinical remission of recurrent pericarditis in patients with celiac a true relationship disease on gluten-free diet su~est between these two entities. I Defective Fe receptor function of tissue macropha~es in patients with dermatitis herpetiformis has been shown which may predispose these patients to higher levels of circulatin~ immune complexes which can then be deposited in tissues causing tissue damage. 6 In summary, we believe that recurrent pericarditis can occur in patients with dermatitis herpetiformis on the basis of immune complex deposition with affinity for pericardial antigens. Whether there is a cross reactivity between ~Iuten, 90 KD glycoprotein and pericardial anti~ens predisposin~ these patients to immune complex-mediated pericardial tissue damage is a postulate to be elucidated by further studies. However, the interestin~ combination of these findings suggests the multisystem involvement of skin, pericardium, small intestine, and pancreas by a common immune mechanism.

Reference Range (3.9) (29.0-72.0)* (0-7)

2 Faizallah R, Costello FC, LEE FI, \VaJker R. Adult eeliac disease and recurrent pericarditis. Di~

Dis Sei 1982; 27:593-97

3 Katz 51. Dermatitis herpetifi>rmis. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg 1M, Austen KF, eds.

in

Dermat()I()~

~eneral

medicine, vol!. New York: Mc.:Graw-Hill, 1987:593-97 4 Maury CPJ, Teppo A-M. Demonstration of tissue 90 KD ~Iyco­

protein as antigen in circulatin~

I~G

immune (.'()(nplexes in

dermatitis herpetiformis and (.'(>eliac disease. Lancet 1984; 1:89294 5 Eterman K~ Feltkamp TEW Antibodies to ~luten in ~astrointestinal

and reticulin

diseases. elin Exp Immunol1978; 31:92-99

6 Lawley TJ, Hall R~

Fauci AS, Katz SI, Hambur~er

MI, Frank

MM. Defective Fe-receptor functions associated with HLA-B8I DRw3 haplotype: studies in patients with dermatitis herpetiformis and normal subjects. N En~1

J Med 1981; 304:185-92

CHEST I 97 I 4 I APRIL, 1990

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