Collagen vascular diseases.
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Plasmacytoid dendritic cell (P-DC) precursors in peripheral blood produce large amounts of interferon (IFN)-alpha/beta when triggered by viruses. However, when incubated with interleukin-3 and CD40 ligand, the same precursors differentiate into mature DCs that stimulate naïve CD4(+) T cells to produce Th2 cytokines. We recently reported that P-DCs accumulate in nasal mucosa of experimentally induced allergic rhinitis, supporting a role for this DC subset in Th2-dominated inflammation. Here we examined whether P-DCs accumulate in cutaneous lesions of lupus erythematosus (LE), a disorder associated with increased IFN-alpha/beta production. Our results showed that P-DCs were present in 14 out of 15 tissue specimens of cutaneous LE lesions, but not in normal skin. Importantly, the density of P-DCs in affected skin correlated well (r(s) = 0.79,P < 0.0005) with the high number of cells expressing the IFN-alpha/beta-inducible protein MxA, suggesting that P-DCs produce IFN-alpha/beta locally. Accumulation of P-DCs coincided also with the expression of L-selectin ligand peripheral lymph node addressin on dermal vascular endothelium, adding further support to the notion that these adhesion molecules are important in P-DC extravasation to peripheral tissue sites. Together, our findings suggested that P-DCs are an important source of IFN-alpha/beta in cutaneous LE lesions and may therefore be of pathogenic importance.
The spectrum of cutaneous lesions seen as a presenting feature or during the clinical course of systemic lupus erythematosus is described. Readers are acquainted with the histopathologic features, serologic associations, and pathogenesis when appropriate. Prognostic implications of individual cutaneous manifestations are also addressed.
Immunofluorescence, especially direct immunofluorescence of skin, has been widely used for diagnosis of connective tissue diseases. It is principally of value for LE and may be used also as a prognostic test for SLE. It is of relatively little value for diagnosis of other connective tissue diseases and appears not to predict the outcome of undifferentiated connective tissue disease. Cutaneous immunofluorescence is widely available and has an established place in the field of dermatopathology. As with other microscopic interpretations, substantial experience, along with an understanding of basic immunopathologic principles, is necessary for reliable results. Antibodies to certain ultrastructural components of the basement membrane zone are now available. These are being evaluated for further diagnostic applications to LE and other diseases that involve the epidermal basement membrane zone.
Cutaneous lupus erythematosus occurs in a spectrum from limited cutaneous disease to severe systemic disease. Chronic DLE generally follows a benign course, but rare transformation to systemic disease may occur. Subacute cutaneous LE is associated with a specific cutaneous lesion and mild to moderate systemic symptoms. Cutaneous disease should be managed by an experienced physician and can lead to control of the process.
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The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.
Lupus erythematosus (LE) is a multisystem disease. Genetic predisposition, altered immunity, hormones, drugs, viruses, and ultraviolet light all may play a role in etiology. A wide range of cutaneous lesions occur, and variants such as subacute cutaneous LE, complement-deficient LE, and neonatal LE have recently been emphasized. Management of the LE patient, including appropriate diagnostic studies and therapy relevant to the dermatologist, is discussed in the review.
Two patients who had lupus erythematosus and C1 esterase inhibitor deficiency are described. The data on eleven previously reported cases are reviewed and summarized with those from our own cases. Early complement component depletion secondary to C1 esterase inhibitor deficiency may predispose to the development of lupuslike syndromes by impairing the ability of the organism to handle foreign antigen. Further study of this unique group of patients may provide insight into the pathogenesis of immune complex disease.