Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pemphigus vulgaris is an autoimmune bullous disorder characterized by autoantibodies directed against desmoglein 3. A group of 19 pemphigus vulgaris sera were characterized by immunoblotting, immunofluorescence, immunoprecipitation, and the passive transfer mouse model. The aim of these studies was to determine the specificity of the autoantibody response in these patients. All patients had clinical and histologic evidence of pemphigus vulgaris. Fogo selvagem sera (n = 8), bullous pemphigoid sera (n = 8), antinuclear antibodies positive sera from patients with lupus erythematosus (n = 2), and normal human sera (n = 8) were used as controls. All pemphigus vulgaris patients showed titers of IgG autoantibodies by indirect immunofluorescence > or = 1:60, predominantly of the IgG4 subclass and immunoprecipitated recombinant desmoglein 3 expressed in the baculovirus system. Patients with disease localized to the mucous membranes showed no reactivity with desmoglein 1 and only one had weak reactivity with mouse skin by indirect immunofluorescence (titer = 1:20). Sera of four of these mucosal patients were tested in the mouse model and three of four did not elicit skin or mucosal disease in the animals. In contrast, sera from all seven patients with disease involving the skin and mucous membranes (generalized disease) produced disease in neonatal mice. In one patient the disease evolved from pure mucosal involvement associated with anti-desmoglein 3 antibodies to a disorder involving mucosas and skin. This transition was associated with the appearance of anti-desmoglein 1 antibodies in the patient's serum. These studies indicate that the autoantibody response in pemphigus vulgaris is heterogeneous. Epitopes recognized by some pemphigus vulgaris sera are species specific and others may be mucosal specific.
 ...
PMID:Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. 932 96

Pemphigus erythematosus, also known as Senear-Usher syndrome, was originally described as a variant of pemphigus with features of lupus erythematosus but regarded today as a localized form of pemphigus foliaceus and considered an autoimmune bullous disease. The autoantigen is desmoglein 1, a desmosomal adhesion protein in keratinocytes. A 69-year-old man presented with a 3-month history of erosions and blisters on the cheeks, which then also appeared on the trunk. Clinical and histopathologic criteria as well as immunofluorescence studies lead to the diagnosis of pemphigus erythematosus with transition to pemphigus foliaceus.
 ...
PMID:[Pemphigus erythematosus]. 2252 97

Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. This result suggests that autoantibodies in pemphigus erythematosus are directed against different antigens and that these autoantibodies are produced by independent clones. Given these clinical and serological data, we suggest that pemphigus erythematosus behaves as a multiple autoimmune disease.
 ...
PMID:Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity? 2332 Jan 49

A 57-year-old male had been suffering from an itchy map-shaped symmetrical erosive erythema with a crust that was attached to his upper arm and buttock, and occasionally he suffered from spiking fever. Laboratory examinations showed neither anti-desmoglein 1/3 antibodies nor anti-BP 180 antibodies, and he fulfilled the criteria for a diagnosis of systemic lupus erythematosus (SLE). Histologically, there was eosinophilic necrosis of keratinocytes, liquefaction and degradation with severe lymphocyte infiltration into the epidermis and subepidermal blister formation, suggestive of a variant of SLE, bullous lupus erythematosus (BLE). One month after remission of BLE, peculiar annular hypopigmentation appeared on the peripheral borders. An immunohistochemical analysis showed a decrease in Melan A-positive melanocytes and concomitant pigment incontinentia, with dense infiltration of CD8(+) T cells and IL-17A(+) Th17 cells. An ultrastructural analysis revealed a decrease, but not a complete disappearance, of both melanocytes and melanosomes, and no impairment in melanosomal transfer. In this case report, we would like to introduce the development of annular depigmentation complicated with BLE, and discuss the effects of lupus condition on melanocyte damage based on immunohistological and electromicroscopic findings of those vitiliginous lesions.
...
PMID:A rare case of male bullous lupus erythematosus complicated with subsequent annular hypopigmentation. 2476 Nov 41

Pemphigus foliaceus (PF) is an autoimmune dermatologic disease that typically presents with painful, superficial blisters that evolve into scaling erosions in a seborrheic distribution. This case study intends to demonstrate that due to the relative scarcity of the disease and its distribution on the body, PF can easily be misdiagnosed. We present a 43-year-old African American male that presented to the dermatology clinic with an 18-month history of non-pruritic, violaceous, scaling patches and plaques most prominent on the malar cheeks, upper chest and upper back. He had been evaluated at an outside hospital with a high suspicion for cutaneous lupus erythematosus (CLE) and seborrheic dermatitis. However, repeated biopsies revealed non-specific spongiotic dermatitis, not consistent with CLE or seborrheic dermatitis. Over the subsequent months, he received treatment for both conditions without improvement in his symptoms. When he was referred to our dermatology clinic, repeat biopsies were obtained which demonstrated acantholysis and dyskeratosis in the granular layer, consistent with PF. Direct immunofluorescence revealed intercellular IgG staining most prominent in the epidermis, also consistent with PF. Finally, enzyme-linked immunosorbent assay for anti-desmoglein 1 returned positive, confirming the diagnosis. Upon review of the previous biopsies, focal areas of acantholysis and dyskeratosis were noted in the granular layer, which would have pointed away from a diagnosis of CLE or seborrheic dermatitis if PF was included in the clinical differential diagnosis. This case serves as a reminder that when there is a discrepancy in clinical-pathologic correlation, it is important to revisit the case and consider other pathologies.
 ...
PMID:Diagnosing Pemphigus Foliaceus: A Rare Blistering Disease Masquerading as a Common Dermatologic Disorder. 3021 75