1/52. IgA multiple myeloma presenting as an acquired bullous disorder.A 63-year-old man presented with an intensely pruritic vesiculo-bullous eruption on the limbs and was subsequently found to have an IgA kappa multiple myeloma. The eruption clinically and histologically was suggestive of linear IgA disease (LAD), dermatitis herpetiformis (DH), epidermolysis bullosa acquisita (EBA), or bullous lupus erythematosus (LE), with the skin biopsy revealing subepidermal bullae and dermal papillary micro-abscesses. However, direct immunofluorescence showed a unique pattern of diffuse dermal IgA staining. Although chemotherapy produced a dramatic resolution of the lesions, which paralleled the fall in serum IgA paraprotein level, the myeloma later became progressive and the resulting paraprotein increase was accompanied by recurrence of the eruption. We propose that this patient's rash was the presenting manifestation of his multiple myeloma, and was a consequence of transudation of IgA paraprotein into the dermis.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
2/52. Bullous systemic lupus erythematosus.A 19-year-old woman with a 6 month history of systemic lupus erythematosus (SLE) developed a widespread urticated, erythematous eruption associated with tense, fluid-filled blisters, erosions and crusting. biopsy showed subepidermal blistering with a prominent neutrophilic infiltrate. Direct immunofluorescence showed markedly positive granular IgG deposition with weak IgM, IgA and C3 at the dermoepidermal junction. No circulating antibodies were detected on indirect immunofluorescence. A diagnosis of bullous systemic erythematosus was made. Treatment with prednisone was ineffective. Subsequent treatment with dapsone led to rapid sustained remission of skin symptoms. Bullous SLE is a rare manifestation of SLE. We review the recent literature and discuss the distinctive features of this condition and contrast them with cutaneous SLE with blisters and the subepidermal blistering disorders.- - - - - - - - - - ranking = 6.2654487273436keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
3/52. Amicrobial pustular dermatosis in two patients with immunological abnormalities.We report two patients with severe amicrobial pustular dermatosis with immunological abnormalities: a 63-year-old woman with a 30-year-history of discoid lupus erythematosus and sicca syndrome, and a 35-year-old woman with high levels of gamma-globulinemia and positive antinuclear antibodies. Both patients presented with crusty and eroded erythematous plaques studded with aseptic pustules on the back, face, and scalp. Histological examination showed acanthosis, neutrophilic exocytosis to the epidermis, and neutrophilic and lymphocytic infiltration with nuclear dust in the dermis. These patients were diagnosed as having "amicrobial pustulosis associated with autoimmune diseases". The eruptions improved with combination treatment of oral prednisolone with cyclosporin A or diaminodiphenylsulphone. Although the pathogenesis remains unclear, amicrobial pustular dermatosis might be one of the cutaneous complications in autoimmune diseases.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
4/52. Bullous systemic lupus erythematosus with milia and calcinosis.Bullous systemic lupus erythematosus (SLE) is a rare skin manifestation of SLE. It shares many features with epidermolysis bullosa acquisita (EBA). We report on a patient with SLE who developed a vesiculobullous eruption followed by findings not typical in bullous SLE, namely milia, mild scarring, and calcinosis. We discuss the relationship between bullous SLE and EBA.- - - - - - - - - - ranking = 5.9527780510016keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
5/52. pyoderma gangrenosum in a patient with bullous systemic lupus erythematosus.We report a 55-year-old woman with bullous systemic lupus erythematosus, who later developed pyoderma gangrenosum (PG). dapsone was effective for the eruption of bullous bullous systemic lupus erythematosus but not for pyoderma gangrenosum. cyclosporine was effective for the skin lesions of pyoderma gangrenosum. This is the first reported case of PG associated with bullous systemic lupus erythematosus.- - - - - - - - - - ranking = 8.3338892714022keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
6/52. Vesiculobullous systemic lupus erythematosus: a case with circulating IgG and IgA autoantibodies to type VII collagen.We report a case of a 17-year-old Japanese boy with vesiculobullous systemic lupus erythematosus. He had a facial "butterfly" erythema and blistering eruption develop. Antinuclear, RNP, and Sm antibodies were positive. The American Rheumatism association criteria for a diagnosis of systemic lupus erythematosus were met. A skin biopsy specimen showed papillary microabscesses of neutrophils and subepidermal blisters and deposits of IgG, IgA, IgM, and C3 by direct immunofluorescence. Indirect immunofluorescence on salt-split skin showed linear IgG, IgA, and C3 deposits on the dermal side. IgG and IgA autoantibodies in his serum reacted with the 290-kd proteins of type VII collagen from human dermal extracts and the recombinant fusion proteins from the noncollagenous domain of type VII collagen by Western immunoblot. To our knowledge, this is the first report of vesiculobullous systemic lupus erythematosus with circulating IgG and IgA autoantibodies to type VII collagen.- - - - - - - - - - ranking = 8.3338892714022keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
7/52. Bullous SLE: response to methotrexate and relationship with disease activity.We report on a case of 40-year-old female with systemic lupus erythematosus (SLE) who developed a severe bullous eruption on sun-exposed areas. The bullous manifestation was associated with a flare of lupus serologies, whereas the previous manifestations of the disease were quiescent. Due to prior intolerance to many drugs, she was given oral methotrexate (10 mg/week). The drug administration was followed by a rapid and full resolution of cutaneous lesions. Five months later she developed a class III lupus nephritis. Our case is the first report of efficacy of methotrexate in bullous SLE and rises the question of the relationship between bullous eruption and SLE activity.- - - - - - - - - - ranking = 1.2646452504307keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
8/52. Bullous lupus erythematosus in a patient with pre-existing dermatomyositis.The group of autoimmune cutaneous connective tissue diseases (CCTD) includes systemic sclerosis, dermatomyositis (DM), lupus erythematosus (LE), and subtypes. Most CCTD patients can be assigned to one of these phenotypes or mixed forms, and transitions among the subsets exist. We present the case of a 26-year-old woman with bullous LE and a background of pre-existing DM. Observations of transitions between DM and LE are extremely rare. Nevertheless, this case demonstrates the close relationship between these two disorders in the context of autoimmune connective tissue disease.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
9/52. Bullous eruption: a manifestation of lupus erythematosus.Bullous lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus (SLE), often associated with autoimmunity to type VII collagen. Generally, patients with BSLE meet the criteria for SLE as defined by the American College of rheumatology. We present a case of a 17-year-old adolescent girl who presented with a vesiculobullous eruption without detectable type VII collagen antibodies and without full criteria for SLE. Differential staining was characteristic for lupus erythematosus (LE), suggesting her eruption is related to LE. We review the spectrum of bullous disease in patients with LE and discuss the pathogenesis and histology of these eruptions, as well as current therapeutic options.- - - - - - - - - - ranking = 7.1905556102003keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
10/52. Papulonodular dermal mucinosis in lupus erythematosus.We report two cases of lupus erythematosus (LE) in which a truncal papulonodular eruption predominated. Histologically the lesions were characterized by diffuse dermal mucin without the usual inflammatory or epidermal changes of LE. It is uncommon in LE for dermal mucin to be present in a sufficient quantity to produce a papulonodular eruption in the absence of typical epidermal changes.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
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