Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.

BACKGROUND: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. CASE REPORT: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin's physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block. CONCLUSION: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants.

Remission of congenital complete heart block without anti-Ro/La antibodies: A case report.

Anti-Ro/La negative congenital heart block (CHB) is uncommon. We report one such case of CHB, with no associated structural heart disease or maternal autoantibodies. The heart block reverted to sinus rhythm spontaneously at two weeks of age, and the patient remains in sinus rhythm at a one year followup. Whether patients with antibody negative complete heart block have a different clinical course is conjectural.

Annular Erythema Associated with Anti-Ro/La Antibody Occurring in Plantar Area / 대한피부과학회지

Recurrent annular erythema associated with anti-Ro/La antibody is a diagnostic term for annular erythemas that usually occurs in the face and the upper extremities of patients with positive anti-Ro/La antibodies. They have been reported in patients with Sjogren's syndrome, lupus erythematosus, or Sjogren's syndrome/systemic lupus erythematosus syndrome. Recently, there have been cases without any underlying autoimmune diseases. We, hereby, report an annular erythema, associated with anti-Ro/La antibody occurring in both soles, which is an unusual location for this disease.

Recurrent Annular Erythema in the Patients with Sj gren's Syndrome and/or Lupus Erythematosus-like Features / 대한피부과학회지

BACKGROUND: Recurrent annular erythema associated with anti-Ro (SSA) or anti-La (SSB) antibodies has been recognized as a unique cutaneous manifestation of Sj gren's syndrome or Sj gren's syndrome/SLE overlap syndrome in Orientals. OBJECTIVE: The purpose of this study was to evaluate the clinical, histological and serological aspects of recurrent annular erythema in the korean patients with Sj gren's syndrome and/or lupus erythematosus-like features. METHODS: This clinical investigation was carried out on 6 outpatients with this disorder who had visited Department of Dermatology, Seoul National University Hospital, from January 1996 to December 2003. RESULTS: These patients (four females, two males; range of age, 24-60 years, median 45 years) had relatively well demarcated annular erythematous plaques mainly on trunk and extremities. Two-thirds of the patients were anti-Ro positive and one-third was anti-La positive. Biopsy specimens revealed a moderate degree of perivascular and periappendageal lymphocytic infiltration throughout dermis. Other features to suggest LE were not demonstrated. Four patients were treated with oral hydroxychloroquine with good response. CONCLUSION: Although all 6 patients showed annular erythema associated with anti-Ro or anti-La antibodies, only two could be diagnosed as Sj gren's syndrome/SLE overlap syndrome. The other four presented with LE-like features such as photosensitivity, nephropathy and arthralgia.

Two Cases of Annular Erythema Associated with Anti-Ro/La Autoantibodies / 대한피부과학회지

Recent studies have demonstrated that annular erythema(AE) associated with anti-Ro(SSA) and/or La(SSB) autoantibodies as a unique cutaneous manifestation of Sj gren syndrome(SS) or SS/systemic lupus erythematosus(SLE) overlap syndrome in Orientals. We report two cases of annular erythemas who had anti-Ro/La antibodies in a setting of SS/SLE overlap syndrome and in a case of anti-La antibody alone with no underlying autoimmune disease, respectively. It appears that the AE may not be a rarely encountering cutaneous lesions among Korean/Oriental people.

Clinical constellation of annular erythema associated with anti-Ro/La autoantibodies

Annular erythema (AE) associated with anti-Ro (SSA) and/or La (SSB) autoantibody in patients with Sjogren syndrome (SS) or with SS/systemic lupus erythematosus overlap syndrome (SS/SLE), has recently been described in Orientals, and it may be a counterpart of annular skin lesion of the subacute cutaneous LE seen mostly in Caucasians. The author examined five Korean AE patients in respect to dinical diversity. In this small-sample study, subtle differences appeared between individual cases regarding the serologic features and the diagnoses of the disease. Among the five cases, four had circulating anti-Ro and anti-La antibodies, and one had only anti-La. Regarding the diagnosis, one was SS/SLE, two were primary SS, and the remaining two were only "AE associated with anti-Ro/La antibody". There seem to be a wide clinical spectrum in the disease expression of AE associated with anti-Ro/La autoantibody than previously thought.