| Abstract: | This article reports 310 cases of BeheeCs syn-drome seen from 1958-1981 in the DermatologyDepartment, now the Institute of Dermatology,Shanghai First Medical College. The syndromeis considered to be a multi-systemic disease af-fecting 4 chief areas: the oral cavity (99.070), skin(96.8To), external genitalia (73.6To) and eyes(39.4To). In this series, joints are involved in 189{60.9%) among which 15'7 (83.1%) have knee jointinvolvement. Therefore, the joint may also beincluded in the chief affected areas. Other sys-tems such as the cardiovascular, gastrointestinaland neurologic systems or other organs are lesscommonly affected. In the 310, a group of 26 inwhich the 3-4 chief areas are simultaneously orsuccessively affected within 3 months accom-panied by more severe general condition, is con-sidered the acute type while the relst, with areasaffected in 6 months t0 21 years with mildersymptoms, are chronic type. Both types run achronic course with a tendency to ameliorateafter 14 years and chiefly involves simultaneousor successive attacks on 3-4 chief areas, repeatedrecurrence, and chronic course. Characteristicsof erytheinatous halo around the nodules, folli-culitis-like lesions, atrophy of tongue tip fungi-form papillae and positive needle puncture phe-nomenon are helpful in diagnosing the disease. Laboratory findings reveal increased ESR,mucoprotein and ceruloplasmin in the majoritywith some positive cryoglobulin determinations.Immunologic examinations show decreased cel-lular immunity. Hemorrheologic determinationand microcirculation of nail fold and tongue tipshow increased microcirculatory blood viscosity.Histopathologic examination of nodular skin le-sions show that the disease process is locatedmainly in the connective tissue of interlobularsepta of subcutaneous tissue and primary vas-culitis of arterioles, venules and capillaries ofthe middle and lower dermis with abscess-likeformation. The etiology remains obscure. |
