Behcets disease Behcets disease Diagnosis criteria 1 North

Behcet’s disease

Behcet’s disease ---Diagnosis criteria (1) • North American criteria of O’Duffy and associates: (1) Aphthous stomatitis (2) Aphthous genital ulceration (3) Uveitis (4) Cutaneous “pustular” vasculitis (5) Synovitis (6) Meningoencephalitis • Diagnosis: (1) + 2 of the above six • Incomplete form: (1) + 1 of the above six • Exclusion: inflammatory bowel disease, SLE, Reiter’s disease, and herpetic infections

Behcet’s disease ---Diagnosis criteria (2) • International criteria for Behcet’s disease (ISG): (1) Oral: minor aphthae, major aphthae or herpetiform ulcer, recurrent at least 3 times in 12 month (2) Genital: recurrent genital aphthae or scarring, esp. scrotum in man (3) Eye: anterior uveitis, posterior uveitis (retinal vasculitis) (4) Skin: EN-like lesion or papulopustular (5) Pathergy: neutrophilic vascular Rx or LCV by 24 or 48 hr after needle insertion • Diagnosis: (1) + 2 of above four

Behcet’s disease ---Clinical features • 108 P’t • Oral ulcer(107) Ocular (70~85%) Genital ulcers(90) Arthritis (50%) Uveitis(42) Neurologic (< 25%) Arthritis(30) Vascular Retinal vasculitis(20) Renal Vascular lesions(20) GI tract Meningoencephalitis(18) Pathergy equivalent(10) Positive pathergy test(6)

Behcet’s disease ---Cutaneous features • 108 P’t • Papulopustular(48) Erythema nodosum(40) Pseudofolliculitis(8) Acneform nodules(8) Pyoderma gangrenosum-like/ulcerative(3) Nondescript(1) • Vesicle, pustules, Sweet’s syn, pathergy, superficial migratory thrombophelebitis, infiltrative erythema, acral purpuric papulo-nodule

Therapeutic ladder for Complex aphthosis and Behcet’s disease (1) Complex aphthosis/ mucocutaneous disease ---Topical or intralesional steroid ---Colchicine: 0. 6 mg bid * 2 wks to tid, decrease 50% recurrence ---Dapsone: 50 mg or 100 mg ---Combination of above (2) Severe mucocutaneous disease ---Thalidomide: 200 mg bid *5 D --> 100 mg bid * 15~60 D, no effect on eye ---Low dose MTX(7. 5 ~20 mg): watch liver cirrhosis and renal prob. ---Prednisone ---IFN - alfa-2 a: decrease aphthae, pustular vasculitis and arthritis (3) Severe ocular and systemic disease ---Prednisone ---Azathioprine ---Cyclophosphamide ---Chlorambucil ---Cyclosporine: decrease eye s/s

Multiple lipomas (1) Multiple symmetric lipomatosis (2) Familial multiple lipomatosis (3) Dercum’s disease ( adiposis dolorosa) (4) Encephalocraniocutaneous lipomatosis overlapping with Proteus syndrome (5) Bannayan-Riley-Ruvalcaba syndrome overlapping with Cowden’s syndrome (6) Bannayan-Zonana syndrome (7) MEN-type 1 (8) Frohlich’s syndrome (9) Gardner’s syndrome

Synonyms • Familial multiple lipomatosis (FML) =Familial multiple lipomas =Multiple circumscribed lipomas =Hereditary multiple lipomas =Multiple subcutaneous lipomas =Symmetric lipomatosis =Discrete lipomatosis • Multiple symmetric lipomatosis (MSL) =Benign symmetric lipomatosis =Launois-Bensaude adenolipomatosis =Madelung’s disease =Symmetric lipomatosis =Multiple symmetric lipomatosis

FML MSL middle age Onset age 20~30 y/o Sex M: F= 2: 1 Heredity not hereditary, some AD, always hereditary familial Distribution Forearms, thighs neck, Neck, shoulder, head, shoulder(-) M: F= 4: 1 Tumor morphology discrete, movable fibrous capsule prox. upper limbdiffuse, may invade deep tissue Associated ondition not known alcoholism(90%)