ABSTRACT
A newborn presented with erythematous lesion over face, which appeared soon after birth. Diagnosis of neonatal lupus erythematosus (NLE) was confirmed by positive anti-Ro SSA antibody and skin biopsy. But anti-La SSB antibody was negative. Her hepatic transaminases were high. But no cardiac manifestations were noted.
Subject(s)
Antibodies, Antinuclear/immunology , Diagnosis, Differential , Face , Female , Humans , Infant, Newborn , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/immunology , Prognosis , Ribonucleoproteins/immunologyABSTRACT
Se presentan, analizan y comparan con otras series los hallazgos dermatológicos, clínicos, histopatológicos e inmunológicos de 17 casos de lupus eritematoso cutáneo subagudo (LECSA) con anticuerpos (Ac) anti-Ro positivos.Las manifestaciones dermatológicas de nuestros pacientes fueron lesiones papuloescamosas policíclicas, más frecuentes que las psoriasiformes. Se localizaban preferentemente en miembros superiores, dorso y zonas de exposición sola. Presentaban un borde eritematovesiculoso bien delimitado. Otras lesiones adoptaron la forma de reloj de arena. Estas manifestaciones fueron recurrentes con períodos de actividad y calma. También fueron relevantes la fotosensibilidad, la hipopigmentación y, en algunos casos, discreta atrofia. Histopatológicamente lo más destacable fue la degeneración vacuolar de la capa basal de queratinocitos y la ausencia de hiperqueratosis folicular, diferenciándose del lupus eritematoso discoide crónico (LEDC). Las restantes lesiones se parecían a las del lupus eritematosos sistémico (LES), pero con menor intensidad. Hubo poco engrosamiento de la menbrana basal PAS positiva, y el infiltrado linfocitario subpapilar y perivascular fue escaso. En algunos casos se demostró edema dérmico alcian blue positivo, poniendo de manifiesto la presencia de mucina. A diferencia de lo relatado por otros autores, encontramos discreta atrofia en 4 pacientes. Lo más significativo de los exámenes inmunológicos fue la presencia del Ac anti-Ro en el 100% de los enfermos y el anti-La solo en el 17,6%. Fue criterio de inclusión para esta serie tener anti-Ro positivo con anti-ADN y anti-Sm negativos. El FAN fue positivo en el 70%. Con respecto a las manifestaciones clínicas generales, se observaron artritis/artralgias en el 100% de los casos. Las lesiones fueron simétricas, no erosivas ni deformantes. Un solo enfermo tuvo pleuresia y glomerulonefritis crónica difusa...
Clinical, histopathologic, and immunological findings in 17 patients with subacute cutaneous lupus erythematosus (SCLE) and Roantibodies are described. Skin manifestations consisted of recurrent, polycyclical, circumscribed papulosquamous, psoriasiform, or hourglass-like lesions with vesiculoerythematous borders, mainly on the upper limbs, back, and sun-exposed areas. Photosensitivity, hypopigmentation, and occasionally mild atrophy were also noted. Histopathologic features included vacuolar degenerative changes involving keratinocyte basement membrane. Unlike chronic discoid lupus erythematosus lesions, follicular hyperkeratosis was absent. Although other characteristics were similar to those seen in systemic lupus erythematosus patients, specimens from SCLE showed less basement membrane thickening and inflammatory cell infiltrates. A few samples revealed positive alcian blue staining for dermal mucin. Only four patients had moderateatrophy. Since only Ro-positive, and DNA- and Sm-negative patients were assessed, immunological studies showed Ro antibodies in 100%of cases and La antibodies in 17.6%. FAN measurements using rat liver and Hep-2 cells were positive in 70% of patients. SCLE was associated with symmetrical, non-erosive, and non-deforming arthritis/arthralgia in all patients, vasculitis in three (17.8%), Raynauds syndrome in two (11.8%), pleuritis and chronic glomerulonephritis in one, and panniculitis in one. Ro antibody screening tests were reviewed.
Subject(s)
Female , Adult , Middle Aged , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/pathology , Autoantibodies/blood , Skin/pathologyABSTRACT
BACKGROUND: Antibodies to Bo antigen are present in most patients with subacute cutaneous lupus erythematosus and in about 50% of Korean patients with systemic lupus erythematosus (LE). However, the pattern of in vivo epidermal deposits of anti Ro antibodies has not been widely recognized. OBJECTIVE: The purpose of thjs study was to define characteristic findings of direct and indirect immunofluorescence(IF) in patients with high-titer anti-Ro or anti-Ro/La positive LE. METHODS: Lupus band test (riirect IF with normal appearing forearm skin specimens) and indi- rect IF with normal skin substr ates were performed with 3 patients of systemic LE who have high titers( >1: 640) of anti-Ro or anti-Ro/La antibodies but have no antibodies against other nuclear antigens such as nDNA/Sm/nRNP/Scl-70. RESULTS: An identical pattern of immune deposits was observed in the epidermis in all 3 pa- tients through direct and indirect, IF examinations. The characteristic pattern recognized was "fine speckling" of IgG (or IgG/IgM) mainly at the nuclei on the basal keratinocytes or keratinocytes throught the epidermis. In the immunoblot assay performed with one patient, IgG anti-Ro/La anti- bodies were identified to recognize the 52/42kD antigens (probably, the Ro/La antigens) in the cultured keratinocyte extracts. CONCLUSION: Most direct IF studies in patients with systemic LE(lupus band test) have shown granular depositions of immunoglabulins and complement components along the dermoepidermal junction, however, the staining patterns as observed in this study may have been overlooked. The recognizable fine speckled patteen of immune deposits at the epidermal keratinocytes could he taken into account as a positivi. finding in the broad category of "lupus band", seen with the normal appearing skin in patient s with systemic LE, especially, who have high titers of anti-Ro/ La antibodies.
Subject(s)
Humans , Antibodies , Antigens, Nuclear , Complement System Proteins , Epidermis , Forearm , Immunoglobulin G , Keratinocytes , Lupus Erythematosus, Cutaneous , Lupus Erythematosus, Systemic , SkinABSTRACT
In Systemic lupus erythematosus several correlations with anti-Ro antibodies have been noted. They are the increased incidence of photos nsitive skin disease, rheumatoid factor positivity, Sjogren's syndrorme, and a greatly increased incidence of the DR 3 hapIotype. In this study we examined the prevalence of anti-Ro anibodies(not concerned for anti-La, Sm, RNP) by double immunodiffusion method among thirty two Korean patients with systemic lupus erythematosus who had positive results on routine fluorescent antinuclear antibody test. We also have seen skin manifestations on these patients, and examined if there is any difference of the incidence for each skin sign between anti-Ro positive and negative subgroups. From the data obtained, the proportion of anti-Ro positives among these patients with systemic lupus erythematosus was 53% Regarding to the difference of the incidence of cutaneous rnanifestations between the two subgroups, the photosensitivity reaction was recongnized as a uniqu symptorn rnore prevalent (p<0. 05) in anti-Ro positive subgroup with the frequency of 65%.