Stomatologie Aspects of Cutaneous Diseases B e h ç e t’s S y n d ro m e
ROBERT J. GORLIN, D.D.S., M.S. AND HEDDIE O. SEDANO, D.D.S., Dr. O.
delineated as an entity by Beh9et comprises 1) aphthous stomatitis, 2) genital ulcers, and 3) recurring uveitis (hypopyon). The syndrome was thought to be the result of an allergic reaction, a concept supported by its frequent association with erythema nodosum. Recently an autoimmune pathomechanism has been suggested, based upon the presence of autoan tibodies against oral mucosa and a rise in the titer of immunoglobulins in the lesions of the oral mucosa and in the blood of patients with the condition. Other findings bolstering the concept of autoimmune causa tion are the presence of a marked mononuclear infiltrate in the oral epithelium of affected patients, and the constant association of macrophages with degenerated epithelial cells in the oral mucosa adjacent to the areas of ulceration. B e lie fs syndrome usually occurs between the ages of 10 and 45 years, with a predilection for males in a proportion of 3-5:1. Most cases appear in the Middle East and in individuals of Jewish extraction. Few cases have been reported in the United States. Early manifes tations of the condition are malaise, weakness, mild fever, sore throat, tonsillitis, muscular pain, and stiffness of the joints, generally of the legs, all or any of which may appear up to 5 years prior to the main manifesta tions of the syndrome. Several types of ocular lesions such as conjunctivitis, keratitis, and retinitis, but most commonly chronic, recurrent uveitis with hypopyon, which may lead to severe diminution of vision up to blindness, have been reported in 80% of affected individuals. Initially, the
T he
syndrom e
Dr. Gorlin is Professor and C hairm an o f the Division o f Oral Pathology, School o f Dentistry, University o f M innesota, M inneapolis, Minnesota. Dr. Sedano is Associate Professor, Division o f Oral Pathology, School of Dentistry, University of Minnesota, Minneapolis, M inne sota.
patient experiences intense periorbital pain and photo phobia, usually starting in one eye, and eventually affecting both eyes. Recurrent conjunctivitis may precede the uveitis for several years. The genital lesions, which occur in 85% of patients, are large, recurrent aphthae, usually on the scrotum but also on the penis and inner thighs. When lesions develop on the vulva, they may lead to fenestration of the labia minora and severe scarring after healing. Involvement of the perineum may occur in both sexes. Oral ulcerations are the most constant feature of B e lie fs syndrome. The aphthae, which represent the initial manifestations in 60% of the cases, tend to appear in groups anywhere on the oral mucosa. They are of various sizes, markedly painful, well circumscribed by an erythematous halo, and covered by a yellowish-gray exudate. Similar lesions arise in the pharynx and esophagus and cause dysphagia. Another oral finding, considered characteristic for the syndrome, is the almost complete or total disappearance of the fungiform papil lae. Cutaneous manifestations are pustules, impetigo, folliculitis, furuncles and ulcers. Erythema nodosum, especially of the legs, occurs in 30% of the patients and is sometimes accompanied by increased body tempera ture. Erythema multiforme-like lesions may also be part of the cutaneous picture. Other developments may be recurrent thrombophle bitis of superficial veins of the legs (45%), arthralgia (35%), and epididymitis (5%). The central nervous system is affected in 20 to 50% of the patients. Meningi tis, spastic weakness, external ophthalmoplegia, slurred speech, dysphagia, and acute mental confusion or progressive dementia are the most frequent manifesta tions of involvement of the central nervous system. Sixty-five percent of patients with neural disease die within the first year after the appearance of signs or
J. Dermatol. Surg. Oncol 3:4 July/A ugust 1977
389
BE HÇE T'S S Y N D R O M E
FIGURE I. Severe conjunctivitis in a patient with Behqet’s syndrome.
symptoms. The heart, the lungs, the gastrointestinal tract and other visceral systems may also be affected. Necropsy of some patients with the Behcet’s syndrome has shown extensive pulmonary and splenic fibrosis, atrophic esophagogastroenteritis, subchronic panophthalmitis, and disseminated atrophic or degener ative lesions in the brain. Histologic studies have revealed the primary ocular defect to be an obliterative vasculitis which eventuates in hemorrhagic infarcts, iridocyclitis, and retinal detachment. Behcet’s syndrome must be differentiated from erythema multiforme. Some authors regard erythema multiforme as a variant of the Behcet’s syndrome. We think not and that the ocular and oral lesions in the two entities are entirely different. In and around the mouth erythema multiforme is marked by superficial mucosal sloughing and hemorrhagic crusting of the lips, whereas Behcet’s syndrome presents intraoral aphthae only. Incomplete forms of the syndrome of Behcet occur in which not all three major components are observed. Reiter’s syndrome sometimes enters into differential diagnosis. Patients with Behcet’s syndrome show at times a characteristic erythematous reaction to an intracutaneous injection of saline solution. Moderate leukocytosis and slight elevation of the sedimentation rate also may attend episodes of the disease.
390
J. Dermatol. Surg. Oncol. 3:4 July/A ugust 1977
FIGURE 2. Aphthae on a lip and tongue o f a patient with Behget’s syndrome.
FIGURE 3. Deep ulceration on a lip with yellowish-gray exudate, elevated borders, and an ery thematous halo in a patient with Behget's syndrome.
REFER EN C ES E
Davies, J.D. Behyet’s syndrom e with haemoptysis and pulm o nary lesions. J. Pathol. 109:351-356. 1973. 2. Davis, E., and Melzer, E. A new sign in Behyet’s syndrome: scanty fungiform papillae in tongue. Arch. Intern. Med. 124:720721, 1969. 3. Monacelli, M„ and Nazzaro. P.. eds.. Behyet’s disease: a report of 28 cases. Arch. Ophthalm ol. 71:4-14. 1964. 4. Oshima, Y„ et al. Clinical studies on Behyet’s syndrome. Ann. Rheum. Dis. 22:36-45, 1963. 5. Saito, T„ et al. Auto-imm une m echanisms as a probable aetiol ogy o f Behyet’s syndrome: an electron microscopic study o f the oral mucosa. Virchows Arch. (Pathol. Anat.) (Abt. A.) 353:261272. 1971.
ROBERT J. GORLIN, D.D.S., M.S. AND HEDDIE O. SEDANO, D.D.S., Dr. O.
delineated as an entity by Beh9et comprises 1) aphthous stomatitis, 2) genital ulcers, and 3) recurring uveitis (hypopyon). The syndrome was thought to be the result of an allergic reaction, a concept supported by its frequent association with erythema nodosum. Recently an autoimmune pathomechanism has been suggested, based upon the presence of autoan tibodies against oral mucosa and a rise in the titer of immunoglobulins in the lesions of the oral mucosa and in the blood of patients with the condition. Other findings bolstering the concept of autoimmune causa tion are the presence of a marked mononuclear infiltrate in the oral epithelium of affected patients, and the constant association of macrophages with degenerated epithelial cells in the oral mucosa adjacent to the areas of ulceration. B e lie fs syndrome usually occurs between the ages of 10 and 45 years, with a predilection for males in a proportion of 3-5:1. Most cases appear in the Middle East and in individuals of Jewish extraction. Few cases have been reported in the United States. Early manifes tations of the condition are malaise, weakness, mild fever, sore throat, tonsillitis, muscular pain, and stiffness of the joints, generally of the legs, all or any of which may appear up to 5 years prior to the main manifesta tions of the syndrome. Several types of ocular lesions such as conjunctivitis, keratitis, and retinitis, but most commonly chronic, recurrent uveitis with hypopyon, which may lead to severe diminution of vision up to blindness, have been reported in 80% of affected individuals. Initially, the
T he
syndrom e
Dr. Gorlin is Professor and C hairm an o f the Division o f Oral Pathology, School o f Dentistry, University o f M innesota, M inneapolis, Minnesota. Dr. Sedano is Associate Professor, Division o f Oral Pathology, School of Dentistry, University of Minnesota, Minneapolis, M inne sota.
patient experiences intense periorbital pain and photo phobia, usually starting in one eye, and eventually affecting both eyes. Recurrent conjunctivitis may precede the uveitis for several years. The genital lesions, which occur in 85% of patients, are large, recurrent aphthae, usually on the scrotum but also on the penis and inner thighs. When lesions develop on the vulva, they may lead to fenestration of the labia minora and severe scarring after healing. Involvement of the perineum may occur in both sexes. Oral ulcerations are the most constant feature of B e lie fs syndrome. The aphthae, which represent the initial manifestations in 60% of the cases, tend to appear in groups anywhere on the oral mucosa. They are of various sizes, markedly painful, well circumscribed by an erythematous halo, and covered by a yellowish-gray exudate. Similar lesions arise in the pharynx and esophagus and cause dysphagia. Another oral finding, considered characteristic for the syndrome, is the almost complete or total disappearance of the fungiform papil lae. Cutaneous manifestations are pustules, impetigo, folliculitis, furuncles and ulcers. Erythema nodosum, especially of the legs, occurs in 30% of the patients and is sometimes accompanied by increased body tempera ture. Erythema multiforme-like lesions may also be part of the cutaneous picture. Other developments may be recurrent thrombophle bitis of superficial veins of the legs (45%), arthralgia (35%), and epididymitis (5%). The central nervous system is affected in 20 to 50% of the patients. Meningi tis, spastic weakness, external ophthalmoplegia, slurred speech, dysphagia, and acute mental confusion or progressive dementia are the most frequent manifesta tions of involvement of the central nervous system. Sixty-five percent of patients with neural disease die within the first year after the appearance of signs or
J. Dermatol. Surg. Oncol 3:4 July/A ugust 1977
389
BE HÇE T'S S Y N D R O M E
FIGURE I. Severe conjunctivitis in a patient with Behqet’s syndrome.
symptoms. The heart, the lungs, the gastrointestinal tract and other visceral systems may also be affected. Necropsy of some patients with the Behcet’s syndrome has shown extensive pulmonary and splenic fibrosis, atrophic esophagogastroenteritis, subchronic panophthalmitis, and disseminated atrophic or degener ative lesions in the brain. Histologic studies have revealed the primary ocular defect to be an obliterative vasculitis which eventuates in hemorrhagic infarcts, iridocyclitis, and retinal detachment. Behcet’s syndrome must be differentiated from erythema multiforme. Some authors regard erythema multiforme as a variant of the Behcet’s syndrome. We think not and that the ocular and oral lesions in the two entities are entirely different. In and around the mouth erythema multiforme is marked by superficial mucosal sloughing and hemorrhagic crusting of the lips, whereas Behcet’s syndrome presents intraoral aphthae only. Incomplete forms of the syndrome of Behcet occur in which not all three major components are observed. Reiter’s syndrome sometimes enters into differential diagnosis. Patients with Behcet’s syndrome show at times a characteristic erythematous reaction to an intracutaneous injection of saline solution. Moderate leukocytosis and slight elevation of the sedimentation rate also may attend episodes of the disease.
390
J. Dermatol. Surg. Oncol. 3:4 July/A ugust 1977
FIGURE 2. Aphthae on a lip and tongue o f a patient with Behget’s syndrome.
FIGURE 3. Deep ulceration on a lip with yellowish-gray exudate, elevated borders, and an ery thematous halo in a patient with Behget's syndrome.
REFER EN C ES E
Davies, J.D. Behyet’s syndrom e with haemoptysis and pulm o nary lesions. J. Pathol. 109:351-356. 1973. 2. Davis, E., and Melzer, E. A new sign in Behyet’s syndrome: scanty fungiform papillae in tongue. Arch. Intern. Med. 124:720721, 1969. 3. Monacelli, M„ and Nazzaro. P.. eds.. Behyet’s disease: a report of 28 cases. Arch. Ophthalm ol. 71:4-14. 1964. 4. Oshima, Y„ et al. Clinical studies on Behyet’s syndrome. Ann. Rheum. Dis. 22:36-45, 1963. 5. Saito, T„ et al. Auto-imm une m echanisms as a probable aetiol ogy o f Behyet’s syndrome: an electron microscopic study o f the oral mucosa. Virchows Arch. (Pathol. Anat.) (Abt. A.) 353:261272. 1971.
