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Speckled particulate epidermal nuclear immuno globulin g deposition in normal skin correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen






Archives of Dermatology 114(5): 705-710

Speckled particulate epidermal nuclear immuno globulin g deposition in normal skin correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen

Clinical and laboratory findings were correlated from 46 patients with Ig[immunoglobulin]G localization in epidermal nuclei in a speckled (particulate) pattern on direct immunofluorescence of normal skin. Cutaneous manifestations included lupus erythematosus (LE), swollen hands or sclerodactyly, alopecia, vasculitis and dyspigmentation. Systemic manifestations included arthritis or arthralgia, Raynaud's phenomenon, serositis, vascular headaches, mild renal disease, myositis and sicca syndrome. High titer (mean = 1:142, 800) serum antibody to extractable nuclear antigen (ENA) was found in 81%. Eighty-six percent had antibody to an RNase-sensitive antigenic component of ENA (ribonucleoprotein or RNP); 14% had antibody to an RNase-resistant ENA termed Sm. Deposition of IgG in a speckled pattern in epidermal nuclei is an immunopathologic marker for a subset of connective tissue disease characterized by antibody to ENA. Those with Sm specificity had systemic LE (SLE). Those with RNP specificity had Raynaud's phenomenon usually associated with overlapping features of SLE, scleroderma and/or dermatomyositis.


Accession: 006459292



Related references

Prystowsky, S.D.; Tuffanelli, D.L., 1978: Speckled (particulate) epidermal nuclear IgG deposition in normal skin. Correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen. Clinical and laboratory findings were correlated from 46 patients with IgG localization in epidermal nuclei in a speckled (particulate) pattern on direct immunofluorescence of normal skin. Cutaneous manifestations included lupus erythematosus (LE)...

Bentley Phillips C.B.; Geake T.M.S., 1980: Mixed connective tissue disease characterized by speckled epidermal nuclear immuno globulin g deposition in normal skin. Four African female patients are described who presented with the features of systemic sclerosis. Overlapping features of lupus erythematosus or dermatomyositis were present in 3 cases but were not prominent. Direct immunofluorescence of uninvolve...

Bentley-Phillips, C.B.; Geake, T.M., 1980: Mixed connective tissue disease characterized by speckled epidermal nuclear IgG deposition in normal skin. Four African female patients are described, who presented with the features of systemic sclerosis. Overlapping features of lupus erythematosus or dermatomyositis were present in three cases but were not prominent. Direct immunofluorescence of unin...

Prystowsky S.D.; Tuffanelli D.L., 1977: Epidermal nuclear immuno globulin g deposition correlation of clinical features and laboratory findings in 47 patients. Clinical Research 25(2): 100A

Prystowsky S.D.; Tuffanelli D.L., 1976: Epidermal nuclear immuno globulin g deposition correlation of clinical features and laboratory findings in 32 patients. Journal of Investigative Dermatology 67(4): 562

Andersen I.; Andersen P.; Elling P.; Graudal H., 1983: Epidermal nuclear immuno globulin deposits in some connective tissue diseases correlation with extractable nuclear antigen antibodies. In vivo nuclear deposits of IgG were demonstrated by direct immunofluorescence in epidermal cells of normal skin from 6 patients with serum antibodies to an RNase-sensitive extractable nuclear antigen (ENA). Addition of complement to the skin sect...

Gilliam, J.N.; Prystowsky, S.D., 1977: Mixed connective tissue disease syndrome cutaneous manifestations of patients with epidermal nuclear staining and high titer serum antibody to rnase sensitive extractable nuclear antigen. Fifteen patients with epidermal nuclear staining on direct immunofluorescence of normal skin and high titer serum antibody to RNase sensitive extractable nuclear antigen (ENA) had diffuse nonscarring and focal alopecia, abnormal pigmentation, swol...

Seres T.; Kavai M.; Sonkoly I.; Bodolay E.; Zsindely A.; Vegh E.; Szegedi G., 1985: Quantitation of anti extractable nuclear antigen antibodies by elisa technique in patients with systemic lupus erythematosus and extractable nuclear antigen mixed connective tissue disease. The extractable nuclear antigens (ENA) and anti-ENA antibodies are diagnostic and pathogenetic factors in mixed connective tissue disease (MCTD) and systemic lupus erythematosus (SLE). A rapid micro-ELISA technique was introduced to detect anti-nR...

Claudy A.L.; Salandre J.; Claudy D., 1981: Epidermal nuclear immuno globulin g deposition in normal skin characterization by anti ribo nucleo protein immuno globulin g fab fragments. Epidermal nuclear IgG deposition in clinically normal skin may occur in patients with scleroderma or scleroderma-like features. To evaluate the mechanisms of the fixation, Fab fragments of anti-RNP [ribonucleoprotein] IgG antibodies, obtained afte...

Garcia D.L.T.rre I.; Espinoza Campos E., 1982: Speckled epidermal nuclear staining elution of immuno globulin g and demonstration of anti nuclear antibody activity of this fraction in standard substrates. Arthritis & Rheumatism 25(4 SUPPL): S108