摘要:

Histopathologic and immunofluorescence findings of facial annular erythema on a 3-month-old female child, as well as serological detection of anti-SS-A (Ro) and anti-SS-B (La) antibodies, led to the diagnosis of neonatal lupus erythematosus (LE), while no sign of abnormality in the conducting system of the heart was found. During the pregnancy of the present child her mother, with positive anti-SS-A and anti-SS-B antibodies, had a history of Sweet’s syndrome. She was treated with corticosteroid, resulting in a gradual diminution of the existing complete atrioventricular block of the fetus. This history may implicate a potential therapeutic effect for the congenital heart block associated with neonatal LE of corticosteroid given to the pregnant mothe

ISSN:1018-8665    

DOI:10.1159/000246940

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 54-year-old woman presented a lesion on the dorsum of the right hand. Routine laboratory tests were normal or negative. Histological examination revealed mucin deposits in the reticular dermis. The lesion cleared within 1 year without any treatment but biopsy. This case does not fit the diagnostic criteria of any known cutaneous mucinoses.

ISSN:1018-8665    

DOI:10.1159/000246796

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We report a case of bullous systemic lupus erythematosus (SLE). The patient suddenly presented with a widespread blistering eruption on the trunk and neck during the course of treatment of SLE. A skin biopsy specimen showed subepidermal blisters, and direct immunofluorescence (IF) revealed deposition of IgA, IgG and IgM at the dermoepidermal junction. Indirect IF showed linear IgG deposition along the base of 1 M NaCl-split skin. Rapid resolution of the blisters occurred following treatment with dapsone 50 mg daily.

ISSN:1018-8665    

DOI:10.1159/000246941

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Patients with pemphigus are occasionally associated with neoplasms. We report a case of pemphigus with carcinoid. To our knowledge, this is the first case of coexistence of pemphigus and carcinoid.

ISSN:1018-8665    

DOI:10.1159/000246942

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Two factors, its classification and its immunogenetics, may have contributed to an incomplete understanding of the pathogenic mechanism in the disease group commonly termed ‘cicatricial pemphigoid’. We have previously demonstrated that pure ocular cicatricial pemphigoid (OCP) is a unique clinical and immunopathological entity. In an effort to better define the immunogenetic characteristics for patients with pure OCP, we performed HLA typing for the class II MHC gene HLA-DQB 1*0301 allele, by amplifying genomic DNA extracted from patients with polymerase chain reaction, followed by hybridization with a sequence-specific oligonucleotide probe on dot-blotted and Southern-blotted amplified DNA samples. Ninety percent (9/10) of patients with pure OCP had the DQB1*0301 allele, in contrast to 52% (17/33) of normal individuals who had the DQB 1*0301 allele. The high frequency of the HLA-DQB 1*0301 allele in patients with pure OCP may point to a distinct immunogenetic pattern and possibly a distinct pathomechanism for the dise

ISSN:1018-8665    

DOI:10.1159/000246943

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246799

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246800

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Serum from 58 patients with linear IgA disease was tested by indirect immunofluorescence on the novel substrate cylindroma, which produces an abundance of basement membrane components. The use of split skin and of cylindroma has identified 2 target antigens. The majority of patients have a target antigen associated with epidermal cells and similar in distribution on cylindroma to hemidesmosome proteins. A minority have a dermal antigen with the distribution of collagen VII on cylindroma. These data support the hypothesis that linear IgA disease is a heterogeneous disease with regard to the target antigens.

ISSN:1018-8665    

DOI:10.1159/000246944

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246802

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Linear IgA disease (LAD) has circulating IgA auto-antibodies which have been found to be associated with epidermal- and dermal-derived antigens. The localization and molecular weight(s) of the IgA target antigen(s) implicated in LAD and their relationship to the target antigens for the IgA antibodies in bullous pemphigoid (BP) and cicatricial pemphigoid (CP) are unknown. Sera from patients with all 3 diseases who had circulating IgA antibodies were studied by immunoblotting with both epidermal and dermal extracts. Our findings indicate that there is heterogeneity in the localization and molecular weights of the target antigen(s), with multiple bands demonstrated with some sera. Three principal target antigens were identified with molecular weights of 110–120, 160–180 and one approximately 285 kD. These antigens were common to all 3 diseases and were found in 25 patients. These results suggest that the target antigens for IgA basement membrane zone antibodies in LAD, BP and CP are multiple, complex in composition and may differ from the conventional BP antig

ISSN:1018-8665    

DOI:10.1159/000246945

出版商:S. Karger AG    

年代:1994

数据来源: Karger