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Page 1: Pediatric onset of behcet's syndrome with myositis: case report and literature review illustrating unusual features

418

BRIEF REPORT

PEDIATRIC ONSET OF BEHCET’S SYNDROME WITH MYOSITIS: CASE REPORT AND LITERATURE REVIEW ILLUSTRATING UNUSUAL FEATURES

BIANCA A. LANG, RONALD M. LAXER, PAUL THORNER, MARK GREENBERG, and EARL D. SILVERMAN

We report a case of Behget’s syndrome with myositis in a pediatric patient, emphasizing the impor- tance of muscle involvement in the differential diagnosis of calf pain and swelling in Behqet’s syndrome. A review of the English-language literature from 1965 to the present suggests that the clinical picture of Behqet’s syndrome in children differs from that in adults, in that there is a lower frequency of ocular disease, and unusual manifestations appear to be more common.

Behset’s syndrome is a multisystem disorder with a clinical spectrum that has greatly expanded since it was first described in 1937. The initial triad of symptoms included recurrent oral and genital ulcer- ations and relapsing iritis ( I ) . Additional well- recognized features now include cutaneous vasculitis, synovitis, meningoencephalitis, gastrointestinal ulcer- ation, thrombophlebitis, and other vascular occlusive

From the Departments of Pediatrics, Immunology, and Pathology, Divisions of Immunology/Rheumatology and Hematology/ Oncology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Dr. Lang is the recipient of the Fellowship from The Arthritis Society (Canada). Drs. Laxer and Silverman are Associ- ates of The Arthritis Society (Canada).

Bianca A. Lang, MD, FRCPC: Department of Pediatrics, Division of Immunology/Rheumatology ; Ronald M. Laxer, MDCM, FRCPC: Department of Pediatrics, Division of Immunology/ Rheumatology; Paul Thorner, MD, FKCPC: Department of Pathol- ogy; Mark Greenberg, MD, FRCPC: Department of Pediatrics, Division of Hematology/Oncology; Earl D. Silverman, MD, FRCPC: Departments of Pediatrics and Immunology, Division of Immunology/Rheumatology .

Address reprint requests to Ronald Laxer, MDCM, FRCPC, Division of Immunology/Rheumatology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario MSG 1x8, Canada.

Submitted for publication July 10, 1989; accepted in revised form November 6, 1989.

- ~ .-

lesions (2-8). Myositis is rarely associated with Beh- set’s syndrome, with only 5 reported cases in the English-language literature (9-13). We describe a pa- tient with Behqet’s syndrome with localized myositis of the gastrocnemius muscle, and we emphasize the importance of this condition in the differential diagno- sis of calf pain and swelling in Behqet’s syndrome. Our patient also had neutropenia, another unusual mani- festation of this syndrome.

Although Behset’s syndrome is rarely diag- nosed in children, it must be considered in the differ- ential diagnosis of a multisystemic inflammatory dis- order. Our case report, as well as a review of the literature of Behqet’s syndrome diagnosed in patients during childhood, suggest differences in the clinical picture of this syndrome in pediatric and adult age groups. In particular, the presence of ocular disease appears to be significantly less common in pediatric patients.

Case report. The patient, a 15-year-old female of Mediterranean descent, was first admitted to the Hospital for Sick Children, Toronto, at the age of 9, with acute pericarditis. During the preceding 2 years, the patient had experienced recurrent erythema multiforme-like rashes, splenomegaly, neutropenia, and a history of migratory arthralgias. No infectious or inflammatory cause for her pericarditis was found, and she was treated with prednisone and aspirin; a peri- cardiocentesis was also performed. Two months later, she was readmitted for examination of recurrent pain- ful mouth ulcers, hepatosplenomegaly, and neutrope- nia. Again, all examinations gave negative results. Her neutropenia was treated with prednisone, which was tapered over 3% years; there was a recurrence of

Arthritis and Rheumatism, Vol. 33, No. 3 (March 1990)

Page 2: Pediatric onset of behcet's syndrome with myositis: case report and literature review illustrating unusual features

BRIEF REPORTS 419

Figure 1. Biopsy of the left gastrocnemius muscle. a, Myositis with an inflammatory infiltrate in the interstitial and penvascular regions, composed mainly of lymphocytes. One vessel (arrowhead) shows vasculitis, with necrosis of the wall and transmural inflammation. b, Focal necrosis of myocytes, associated with an infiltrate of lymphocytes and macrophages. (Original magnification X 400.)

neutropenia and mouth ulcers as the daily prednisone dosage was reduced.

At age 13, the patient first reported the occur- rence of genital ulcers. One year later, she presented with 2 large labial ulcers, which were debrided and treated locally.

During the next 16 months, the patient was admitted on 4 occasions for examination of calf pain and swelling. These episodes were characterized by the development of unilateral or bilateral calf swelling, redness, and pain. There was no preceding trauma, and there were no associated systemic problems, except for an increased frequency of mouth ulcers. At the time of the fourth episode, the patient presented with a 4-day history of severe left calf pain and a cutaneous ulcer on her right lower leg.

On physical examination, she appeared well,

but was febrile (38.2"C). The left calf was tender, warm, slightly erythematous, and swollen, with a circumference 3 cm greater than the right calf. There was a positive Homans' sign. A cutaneous ulcer, 2 cm in diameter, was observed above the right medial malleolus. There was a small area of ulceration on the right ear pinna, 1 aphthous ulcer on the buccal mu- cosa, and mild splenomegaly. The remainder of the examination, including ophthalmologic and neurologic examinations, gave normal results. Radiologic evalu- ation included a Doppler flow study of the left calf, which showed normal venous flow, and an ultrasound, which showed increased echoes suggestive of myosi- tis. Findings on venograms performed during previous episodes of calf swelling were negative.

Laboratory investigations showed neutropenia (560/mm3), anemia (hemoglobin 96 gdliter), normal

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420 BRIEF REPORTS

Figure 2. Skin biopsy of the right leg ulcer, showing marked vasculitis in the deep dermis, with necrosis of the vessel wall and extensive transmural and perivascular inflammation. The infiltrate consists mainly of lymphocytes. Arrows indicate the edge of the vessel wall (original magnification x 400).

platelet count (272,000/mm3), elevated erythrocyte sedimentation rate (120 mm/hour), and increased se- rum immunoglobulin levels (IgG 30.2 gndliter [normal 6151, IgA 7.84 gndliter [normal 0.35-2.41, and IgM 4.5 gm/liter [normal 0.4-2.51). Her creatine phosphokinase (CPK) level was initially elevated at 503 unitdliter (normal <180), but it returned to normal 3 days later. The partial thromboplastin time was slightly elevated at 45 seconds (normal 2540). Normal or negative results were found for the following tests: liver func- tion, renal function, serum complement, antinuclear antibody, rheumatoid factor, lupus erythematosus cell preparation, serologic studies for viruses and fungi, blood cultures, prothrombin time, and fibrinogen and coagulation factor assays. Findings on previous exam- inations had included normal results on bone marrow

aspiration and in vitro bone marrow culture, and normal white blood cell count survival curve and Rebuck skin window. Direct Coombs’ test and a test for antigranulocytic antibodies gave negative results.

A biopsy of the left gastrocnemius muscle showed a multifocal inflammatory infiltrate consisting of lymphocytes and macrophages within the perimy- sial and endomysial connective tissue (Figure 1 A), associated with necrosis of muscle fibers (Figure IB). A similar infiltrate was seen in a perivascular distribu- tion. Some vessels showed changes of vasculitis, with transmural inflammation and karyorrhectic debris. Im- munofluorescence studies revealed positive staining of vessels for C3. A biopsy of the right leg ulcer also showed evidence of vasculitis, with necrosis of vessels and prominent transmural and perivascular lympho- cytic infiltrates (Figure 2). Bacterial and fungal stains and cultures of muscle and skin biopsy specimens produced negative results.

Treatment with broad-spectrum antibiotics was instituted and, as had occurred during previous epi- sodes, the patient’s calf symptoms resolved over sev- eral days. Colchicine treatment was started; however, with this therapy, new areas of ulceration developed on the right thigh and at the muscle biopsy site. These failed to heal with local treatment and intravenous antibiotics, and repeat biopsies showed vasculitis. Daily treatment with prednisone resulted in rapid resolution of the ulcers.

Discussion. The diagnosis of Behget’s syndrome is frequently difficult to establish because of the lack of pathognomonic symptoms, signs, or laboratory find- ings. There have been several attempts to create formal diagnostic criteria; however, none have been universally accepted. Mason and Barnes (7) formu- lated 4 major criteria (buccal ulcers, genital ulcers, eye lesions, and skin lesions) and 6 minor criteria (gastro- intestinal lesions, thrombophlebitis, cardiovascular le- sions, arthritis, central nervous system lesions, or a positive family history), and proposed that 3 major, or 2 major and 2 minor, criteria were needed for the diagnosis.

O’Duffy and Goldstein recognized many of these same criteria and suggested that for diagnosis of complete Behget’s syndrome, an individual must exhibit any 2 of the following conditions: genital ulceration, uveitis, cutaneous vasculitis, synovitis, or meningoencephalitis in the presence of aphthous sto- matitis; 1 criterion coexisting with stomatitis consti- tuted an incomplete form of the disease (8). Our patient had recurrent aphthous stomatitis, genital ul-

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BRIEF REPORTS 42 1

Table 1. Clinical manifestations of Behcet’s syndrome in 37 patients 516 years of age*

Clinical manifestations Age at

Patient Reference onset Skin Thrornbo- no. no. (years) Sex Oral Genital Ocular? lesions: Joints GI§ phlebitis CNST Other

+ - + + + - - Hematuria - - - -

6 F + 1 23 23 2

2 F + - + + + 3 23 - + + + 23 9 M + 4

5 23 3 M + + - + + +

+ <1 F + + - + - - - -

- - - - - - -

6 23 I 1 M + - - + - + - + Neutropenia, thromboc ytopenia

- - - + + + - + + + -

4 F + + 11 F + + - - -

7 29 29 8

9 29 0.5 M + + + + + + - - - 10 29 10 M + + - + + + - - Nasal septum

perforation + - + + 11 30 8 M +

12 30 30 15 F + + - 13

14 15 7 F + + + 15 15 11 F +

32 14 F + - - + + + 16 17 32 2 F +

31 6 F + 18 19 31 12 F + + + +

33 11 F + 34 <I6 M + + + + - + 20

21 22 35 9 M + + + + - 23 27 7 F + + - + - - - - Splenomegaly,

pulmonary infiltrates

24 36 2 F + + + + - + - + - 25 28 16 M + + - + + + f + Splenomegaly,

26 37 16 M + - - + - - + - Pulmonary artery

27 5 16 F + + + + + + + 29 41 12 M + + - + + - - 28

30 38 12 M + - - + - - + + Nephrotic syndrome, amyloidosis

31 39 4 F + + - + - - - - Arnyloidosis 32 40 15 M + + +

7 M + + - + - - - - Pharyngeal myositis 33 13 34 42 8 M + + - + + 35 18 10 M + 36 43 11 M + - + + + - - + Intracranial arterial

37 Present 9 F + + - + + - - - Myositis,

- - - - - - - - - 1 1 M + - + +

- - - - - - - - - - - -

+ - - + - - - Myalgias - - - - - + + + - + +

+ - - - - - - - - - - - -

+ - - - + - + Fevers - - -

- - - -

Budd-Chiari syndrome

aneurysm - -

24 14 M + + + + + - - + Neutropenia - -

+ + + - - -

- - - - - - - + + - + -

aneurysm

report neutropenia, splenomegaly , pericarditis

* Thirty-five patients met criteria for complete Behcet’s syndrome as proposed by Mason and Barnes (7) and by O’Duffy and Goldstein (8). Patients 13 and 18 met O’Duffy and Goldstein’s criteria for incomplete Behcet’s syndrome (8). GI = gastrointestinal; CNS = central nervous system. t Patients 14, 17, 19, 24, 28, 32, and 36 had uveitis; patients 3, 9, 12, and 22 had recurrent conjunctivitis. t. Erythema nodosum, cutaneous vasculitis, pathergy, pyoderma, or pustules. 8 Colitis or ulceration of the GI tract. Patients 1. 4, and 6 had abdominal pain only. T Meningoencephalitis, seizures, memory loss, cranial nerve palsy, quadriparesis, or papilledema.

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422 BRIEF REPORTS

Table 2. studies of adult patients

Comparison of the clinical features of BehGet’s syndrome in 37 pediatric patients and in 3

Adult studies

Pediatric Oshima, 1963 Shimizu, 1971 Chajek, 1977 patients, (ref. 46; n = (ref. 2; n = (ref. 40, n =

Feature no. (%) 85). Ti 3 3 9 , Yo 41), Ti

Oral ulcers Genital ulcers Ocular lesions Skin Joints Gastrointestinal Thrombophlebitis Central nervous system

37 (100) 28 (75) 11 (30) 31 (84) 20 (54) 15 (40) 6 (16)

12 (32)

98 64 79 85 64 59 19 29

99 69 90 84 52 52 10 10

98 88 76 88 29

37 29

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ceration, cutaneous vasculitis, and arthritis, thus ful- filling both sets of proposed criteria. The major, and many of the minor, features of Behqet’s syndrome have been well described in the literature (1-8); how- ever, our patient’s case illustrates several unusual aspects of this disease.

Myositis, rarely described in Behget’s syndrome, was a prominent feature of our patient’s illness, leading to hospitalization for calf pain and swelling on 4 occa- sions. Because of the increased incidence of both super- ficial and deep thrombophlebitis in Behget’s syndrome, a deep vein thrombosis was considered the most likely cause of these symptoms; however, the results of venograms and Doppler studies were repeatedly nor- mal. Cellulitis was also considered, although there was no area of skin ulceration on the affected leg predis- posing to infection. Myositis, suggested by ultrasound findings of increased echogenicity, scintigraphic stud- ies showing increased soft tissue uptake, and an ele- vated CPK level, was confirmed by muscle biopsy. The histopathologic findings, as well as negative re- sults of cultures and organism stains, pointed to a noninfectious myositis.

There are 5 case reports of myositis associated with Behget’s syndrome in the English-language liter- ature. In 2 of these patients, the clinical presentation was one of bilateral calf pain, as was seen in our patient. Both of these patients had necrotizing myosi- tis on biopsy (9,lO). A third patient presented with pain and swelling of the upper thigh, and although his CPK value was normal, a biopsy of the quadriceps femoris muscle showed an inflammatory myositis (1 1). In 2 of these 3 patients, symptoms resolved without treatment. Arkin et a1 (12) reported a patient with Behget’s syndrome who had high CPK levels and right arm pain, which was diagnosed as thrombophlebitis.

The patient died of central nervous system involve- ment and at autopsy, was found to have diffuse necro- sis and muscle inflammation of the right forearm, in addition to thrombophlebitis. There was also an inflammatory process affecting both quadriceps muscles.

In our patient, as well as in all 4 patients mentioned above, the histologic features present at the time of muscle biopsy were characterized by a chronic inflammatory cell infiltrate and significant muscle fiber degeneration. In 2 patients, perivascular infiltration was noted, and in 1 of these patients, an associated vasculitis, as seen in our patient, was reported. Dys- phagia resulting from localized myositis involving the pharyngeal musculature, confirmed by biopsy, has been described in a 14-year-old boy with Behget’s syndrome (13). Symptoms resolved with prednisone treatment.

In addition to myositis, clinical evidence of muscle involvement reported in Behqet’s syndrome has included myalgias (14-16) and muscle weakness (17,18). A peripheral neuropathy was the presenting clinical feature of 1 patient whose muscle biopsy showed evidence of vasculitis (19). Subclinical muscle involvement has also been described in Behget’s syn- drome (20,21). Afifi et al studied proximal muscle tissue from 7 patients, 2 of whom had mild muscle weakness, and upon electron microscopic examina- tion, found significant abnormalities of the muscle fibers in all patients (21). Hamza et al described 22 patients with Behqet’s syndrome who underwent mus- cle biopsy, and found that 2 of the patients had perivas- cular infiltration and 5 had perivascular fibrosis (22).

In addition to myositis, uncommon features of our patient’s illness included neutropenia, pericarditis, and splenomegaly. Each of these conditions has been previously described in Behqet’s syndrome (23-28).

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BRIEF REPORTS 423

Neutropenia has been reported in 2 patients, both of whom experienced disease onset before the age of 16 (23,24).

The occurrence of several unusual disease man- ifestations and the absence of ocular disease in our patient raised the question of whether BehGet’s syn- drome has a different clinical picture in the pediatric age group than in the adult population. Behqet’s syndrome is very uncommon in children, and there is little literature about this aspect of the disease. We therefore reviewed the medical literature from 1965 to the present for reports of pediatric cases of Behqet’s syndrome, and we found 37 cases including the present case (5,13,15,18,23,24,27-43) (Table 1 ) . A number of reviews have included a small number of children in their studies, but have not specified their clinical pictures (2,7,14,30,44-46). Therefore, these patients were not included in the present review. Of the studies found, 35 patients fulfilled criteria for complete BehGet’s syndrome (7,8), and 2 patients met O’Duffy and Goldstein’s criteria for incomplete Beh- Get’s syndrome (8). Three reported cases of BehGet’s syndrome in children were not included in Table I because they did not meet complete or incomplete criteria (47,413). However, because of the prolonged interval between onset of symptoms and the appear- ance of complete manifestations, it is likely that Beh- Get’s syndrome is actually underrecognized in chil- dren. Indeed, in the patient reported, the diagnosis of Behget’s syndrome was first considered 6 years after the onset of her first symptoms.

In the pediatric patients reviewed, the fre- quency of oral ulcers, genital ulcers, skin lesions, and joint symptoms was similar to the frequencies of these manifestations in the adults studied (2,40,46) (Table 2). However, eye disease was strikingly uncommon in the pediatric patients, occurring in only 11 of 37 patients (30%). Of these patients, only 7 of 37 (19%) had uveitis, and the remaining 4 had recurrent conjunctivi- tis. A low incidence of ocular disease was previously noted by Ammann et al (23), who described 6 children with Behset’s syndrome, only 1 of whom had eye involvement. Although another report suggested that there might be an increased frequency of eye disease in younger patients, the group studied included only adults with Behset’s syndrome (49).

Our literature review also indicated that despite the small number of pediatric cases, a number of unusual manifestations have been reported in children. These include neutropenia (23,24), splenomegaly (27,28), Budd-Chiari syndrome (28), renal involvement with

amyloidosis (38), recurrent pulmonary infiltrates (27), and the rupture of a pulmonary artery aneurysm (37).

Our 15-year-old patient with Behqet’s syn- drome has illustrated several important points about this illness. Myositis, although an uncommon manifes- tation of this disease, is an essential consideration in the differential diagnosis of painful swelling of an extremity. Because of the tendency for pathergy and poor wound healing in patients with Behqet’s syn- drome, the diagnosis of muscle involvement should be made using radiologic investigations, i.e., ultrasound or magnetic resonance imaging, rather than by muscle biopsy. In addition, other unusual clinical manifesta- tions of this multisystem disease, including neutrope- nia, may be seen in pediatric patients. We suggest that the clinical picture of Behqet’s syndrome in the pedi- atric age group differs from that seen in adults. Ocular involvement, a major criterion for the diagnosis of BehGet’s syndrome, appears to be significantly less common in the pediatric group, while unusual mani- festations appear to be more common.

Addendum. Since this manuscript was submitted for publication, 3 additional children with Behqet’s syndrome have been reported (Rakover Y, Adar H, Tal I , Lang Y , Kedar A: Behqet’s disease: longterm followup of 3 children and review of the literature. Pediatrics 83:98&992, 1989). All 3 of these patients had recurrent oral ulcers, skin manifes- tations, arthritis, and ocular involvement. Conjunctivitis with keratitis was noted in 1 patient, and uveitis occurred in the other 2 patients. Two of the patients had genital ulcers, and 2 had evidence of central nervous system involvement.

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