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PubMed1985

Progressive polysystemic immune-mediated disease in a dog.

A dog with polyarthritis, angular joint deformities, and a high serum antinucleolar antibody titer was treated over a period of 20 months. During the clinical course, leukopenia, thrombocytopenia, Coombs' positive hemolytic anemia, and a pemphigus-type skin disorder developed, all of which responded to immunosuppressive therapy. It is not known whether the polysystemic disease in this dog represents a pleomorphic manifestation of canine systemic lupus erythematosus or multiple autoimmune disorders occurring in the same animal.

Animals↗

PubMed1999

When to request immunofluorescence: practical hints.

This review highlights important considerations in obtaining good skin biopsy specimens to optimize results of direct immunofluorescence (IF) studies and also summarizes the various patterns of cutaneous IF deposition and their associated diagnoses. IF findings of immunobullous diseases, lupus erythematosus, vasculitis, lichen planus, and erythema multiforme are included. The uses of indirect IF studies are also reviewed including newer modifications that are valuable in helping to diagnose epidermolysis bullosa acquisita and paraneoplastic pemphigus.

Basement Membrane↗

PubMed1999

Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.

Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.

Aged↗

PubMed2007

Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases.

Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.

Adolescent↗

PubMed2002

[Oral antibiotics with antiinflammatory/ immunomodulatory effects in the treatment of various dermatoses].

A range of antibiotics has been shown to modify host functions apart from the antimicrobial potency. They may directly influence phagocyte and lymphocyte function, as well as chemotaxis. Effects on the generation and release of various cytokines involved in the inflammatory process have been studied both in vivo and in vitro. However, the molecular mechanisms have not been elucidated in detail. It is suggested that antibiotics can exert immunopharmacological effects on the basis of structure-activity relationships independent of their antibacterial activity. We present a collection of clinical studies and case reports dealing with the potential benefits of systemic antibiotics in the treatment of selected dermatoses which have primarily been classified as non-infectious. Generally accepted treatments such as dapsone in dermatitis herpetiformis and leukocytoclastic vasculitis or chloroquine in lupus erythematosus have not been considered. Based on existing clinical trials with a higher number of cases the following antibiotics have been shown to be effective: erythromycin in bullous pemphigoid and pityriasis rosea as well as tetracyclines in pemphigus vulgaris, bullous pemphigoid and pustulosis palmaris et plantaris. However, most reports have to be viewed critically due to the uncontrolled study design, particularly in dermatoses with a tendency to spontaneous resolution. Despite this lack of evidence-based data, it is important for the clinician to know about the potential use of antibiotics for various skin disorders as a primary treatment option or steroid-sparing therapeutic adjunct. In clinical practice the use of antibiotics depends on various factors including the severity of the disease, the patient's age, contraindications to conventional therapeutic regiments and economic considerations.

Adjuvants, Immunologic↗

PubMed1994

A novel immune-mediated subepidermal bullous dermatosis characterized by IgG autoantibodies to a lower lamina lucida component.

BACKGROUND: Immune-mediated subepidermal bullous dermatoses characterized by in vivo-bound linear IgG deposition at the cutaneous basement membrane zone include bullous pemphigoid, ocular cicatricial pemphigoid, anti-bullous pemphigoid antigen mucosal pemphigoid, anti-epiligrim mucosal pemphigoid, epidermolysis bullosa acquisita, and the bullous eruption of systemic lupus erythematosus. In this article, we describe a novel IgG-mediated bullous dermatosis. OBSERVATIONS: Clinically, a unique nonscarring dermatosis was characterized by the sudden onset of extensive bullae and erosions on mucous membrane and skin, resembling toxic epidermal necrolysis or pemphigus vulgaris. Histologically, the patient's skin lesion demonstrated neutrophilic papillary dermal infiltration and subepidermal blister formation, resembling dermatitis herpetiformis. Immunopathologically, there was linear IgG and C3 deposition at the skin basement membrane zone. The patient responded well to prednisone and azathioprine immunosuppression and has achieved a lasting remission without further therapy. Further immunologic investigations revealed that this unique dermatosis is distinct from all other known IgG-mediated subepidermal bullous dermatoses. CONCLUSIONS: This novel deep lamina lucida pemphigoid can be distinctly termed anti-p105 pemphigoid on the basis of antigenic specificity of the autoantibodies. Although this novel dermatosis resembles toxic epidermal necrolysis clinically, prudent use of diagnostic immunofluorescence studies can clearly delineate its immunologic nature. Prompt recognition of this unique dermatosis and timely initiation of appropriate immunosuppressive therapy could be life-saving for those patients suffering from this dermatosis.

Autoantibodies↗

PubMed1999

Pneumocystis carinii pneumonia in patients receiving immunosuppressive drugs for dermatological diseases.

In recent years, Pneumocystis carinii pneumonia (PCP) has been increasingly reported in patients without human immunodeficiency virus (HIV) infection. The increased occurrence of PCP in non-HIV-immunocompromised subjects has been attributed to several factors: use of stronger immunosuppressive regimens, higher awareness of PCP, advanced diagnostic technology and nosocomial spread of P. carinii. Appearance of PCP subsequent to the use of immunosuppressive drugs has been noticed in many inflammatory diseases such as rheumatoid arthritis, systemic lupus erythematosus and ulcerative colitis. Dermatologists frequently use immunomodulating agents, but the occurrence of PCP in patients receiving immunosuppressive drugs for skin diseases is largely unknown. We report four cases where PCP appeared following the use of immunosuppressive drugs primarily for cutaneous diseases, namely pemphigus, cutaneous necrotizing vasculitis (two cases) and Behçet's syndrome. These cases were identified in a computerized database study (1979-95) to evaluate the occurrence of PCP among immunocompromised hosts without HIV infection.

Behcet Syndrome↗

PubMed1998

[Extracorporeal photopheresis in therapy-refractory disseminated discoid lupus erythematosus].

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Discoid LE (DLE) is the most common form of cutaneous LE; the disseminated form of DLE is rare. We report an encouraging response to treatment with extracorporeal photopheresis (ECP) in a single patient with disseminated DLE who did not respond to conventional therapy. To the best of our knowledge this is the first successful use of ECP in the management of such a patient. Extracorporeal photopheresis is a therapeutic modality that has been under investigation for more than 12 years. Although originally developed for the treatment of cutaneous T-cell lymphoma, ECP has recently been used for the management of autoimmune diseases including systemic scleroderma, pemphigus vulgaris and SLE, as well as prevent organ rejection in patients with cardiac or kidney transplants and graft versus host disease after bone marrow transplantation.

Dose-Response Relationship, Drug↗

PubMedDate not supplied

Bullous systemic lupus erythematosus.

Blistering eruptions are rare cutaneous manifestations of lupus erythematosus (LE) that may be caused by different mechanisms. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic-, subacute-, and acute-cutaneous LE due to a severe vacuolar alteration of the dermoepidermal junction (DEJ), dermal edema, and lekocytoclastic vasculitis. An exaggerated example of such changes is rarely seen at the advancing edge of the annular plaques of subacute cutaneous LE with erythema-multiforme (EM)-like appearance, a condition formerly described as Rowell's syndrome. In a recently reported novel variant of LE-associated toxic epidermal necrolysis, dysregulated keratinocyte apoptosis has been proposed as an underlying mechanism. These vesiculobullous lesions are considered to be LE-specific. Blistering may also occur in LE in the context of a coexisting immunobullous disease. Pemphigus, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis, and linear IgA bullous dermatosis have been all reported in association with LE. Their differentiation relies upon characteristic clinical, histologic, and immunopathologic features (Table 1). These blistering eruptions are rather non-specific for LE.

Humans↗

PubMed1983

Specificity and inhibition of the epidermal cell detachment induced by pemphigus IgG in vitro.

IgG isolated from sera of patients with pemphigus vulgaris (PV) has been shown to induce cell detachment when added to primary epidermal cell cultures (PECC). We studied the specificity of this phenomenon. IgG fractions were purified from the sera of five patients with PV and control IgG fractions from the sera of normal donors and patients with bullous pemphigoid (BP), systemic lupus erythematosus (SLE), and anti-AB blood group sera (anti-AB). IgG fractions were added to PECC either at initial plating (0 hours), at media change (48 hours), or sequentially at both times, and cell detachment was quantitated at 72 and 96 hours. Significant cell detachment occurred only when PV IgG was added to the growth media sequentially at 0 and 48 hours (p = 0.001), and this effect was dose-dependent for either dose. Substitution of an unrelated IgG (BP, SLE, or anti-AB) at either time points reduced cell detachment to near control values. Furthermore, cell detachment was inhibited by the addition of the proteinase inhibitors alpha 2 macroglobulin (70% inhibition of detachment), aprotinin (63% inhibition), soybean and lima bean trypsin inhibitor (62 and 64%, respectively), and pepstatin (49%), but not by the inhibitors chymostatin, leupeptin, or antipain. These data confirm that PV IgG induces increased cell detachment in PECC and shows that this effect is specific for PV IgG, is dose-dependent, and may be inhibited by certain proteinase inhibitors.

Animals↗

PubMed2003

Deposition of nucleosomal antigens (histones and DNA) in the epidermal basement membrane in human lupus nephritis.

OBJECTIVE: Antinuclear autoantibodies complexed to nucleosomes can bind to heparan sulfate (HS) in the glomerular basement membrane. This binding is due to the binding of the positively charged histones to the strongly anionic HS. Nucleosomes and histones have been identified in glomerular deposits in human lupus nephritis. We investigated whether nucleosomes are present in the basement membrane of nonlesional skin of lupus patients. METHODS: Skin biopsy samples from patients with systemic lupus erythematosus (SLE) (30 with active lupus nephritis and 15 with inactive disease) and controls (with parapemphigus or diabetes) were stained for IgG, histones, DNA, and nucleosomes. RESULTS: IgG deposits were found in 87% of the patients with lupus nephritis, in 33% of the patients with inactive disease, and in 71% of the parapemphigus patients. Using polyclonal antihistone antibodies, histones were detected in 87% of lupus nephritis patients, but in none of the other SLE patients or the diabetes controls (P < 0.0001). Among the parapemphigus controls, 14% of samples stained positive in one of the polyclonal antihistone stainings (P < 0.0001). Using monoclonal antibodies, histones and DNA were identified in 21% of the lupus nephritis patients. Although none of the other groups showed positive staining for nucleosomes, 7% of the lupus nephritis biopsy samples were positive using antinucleosome monoclonal antibodies. Colocalization of nucleosomal antigens and IgG was confirmed using confocal laser microscopy. CONCLUSION: These findings suggest that nucleosome-mediated binding of autoantibodies to basement membranes may also occur at sites in the body other than in the glomerulus.

Adult↗

PubMed2003

[Dermatological diseases influenced by pregnancy].

Systemic lupus erythematosus Pregnancy can trigger-off or worsen the disease (60% of lupus flares). The renal damage and hypertension are important prognostic factors. Regarding the foetus, there is a 60% risk of prematurity. The risk of neonatal lupus is basically related to the presence of anti-Ro-SSA and anti-La-SSB antibodies and cannot not be foreseen. Multi-disciplinary surveillance is a necessity for any woman suffering from lupus and wishing to become pregnant. Other connective disorders Pregnancy is rare during scleroderma and has no influence on its progression except in the case of renal damage. Regarding dermatopolymyositis, pregnancy, which is rare, might trigger-off or worsen the disease. Bullous diseases Porphyria may be triggered-off or worsened during pregnancy. The various forms of pemphigus may appear or be enhanced by pregnancy. Various affections The influence of sarcoidosis on pregnancy is negligible. Complications are possible in patients exhibiting a type I or IV Ehlers-Danlos syndrome. Pregnancy can enhance the vascular complications of a pseudoxanthoma elasticum. An exacerbation is possible in the case of a type I neurofibromatosis. Progression varies in the case of psoriasis, atypical dermatitis, acne and Behçet's disease. Genital infections are frequent during pregnancy and often represent a risk for the infant (vaginal candidosis, viral condyloma, gonococci, herpes infection). Varicella The risk of severe neonatal varicella is high when the disease appears in the mother just before or after delivery.

Connective Tissue Diseases↗

PubMed2002

Spectrum of subepidermal immunobullous disorders seen at the National Skin Centre, Singapore: a 2-year review.

BACKGROUND: The subepidermal immunobullous disorders (SEIBDs) comprise bullous pemphigoid (BP), cicatricial pemphigoid (CP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LAD), dermatitis herpetiformis (DH), pemphigoid gestationis (PG) and bullous systemic lupus erythematosus (BSLE). They are thought to be rarer in the Far East than in western Europe. OBJECTIVE: This 2-year retrospective study investigates the spectrum seen at our centre and the minimum estimated incidence of each. PATIENTS AND METHODS: A total of 67 patients seen at the National Skin Centre (NSC), Singapore between January 1998 and December 1999 were diagnosed as having an SEIBD. Fifty-nine (88%) had BP, four (6%) had EBA, two (3%) LAD and two (3%) BSLE. There were no cases of CP, DH or PG diagnosed during this period. The minimum estimated incidence in our local population was 7.6, 0.5, 0.26 and 0.26 per million population per year, respectively. The mean age of onset was 77, 68, 65 and 31 years, respectively. RESULTS: BP is the commonest SEIBD seen locally, with an incidence at least equal to that in western Europe. It is diagnosed at our centre three times more frequently than pemphigus. There is a predilection for ethnic Chinese but not Indian. EBA is twice as common as in western Europe and shows a predilection for ethnic Indians. LAD is rare here compared to China, despite the predominant Chinese population. BSLE is also rare. In contrast to western Europe, CP, DH and PG are very rare in Singapore. CONCLUSIONS: This is the first study from this region to show that certain SEIBDs are not rarer in the Far East, as previously thought.

Adult↗

PubMedDate not supplied

Blood dehydroepiandrosterone sulphate (DHEAS) levels in pemphigoid/pemphigus and psoriasis.

Serum dehydroepiandrosterone sulphate (DHEAS) levels and diagnostic autoantibody titers were measured in patients with pemphigoid/pemphigus [n = 46/4; 21 men and 29 women, 42 to 93 years of age (mean 79)]. Twenty-four patients were either on peroral Prednisolone (n = 11), or topical treatment with betamethasone (n = 13), and the other 26 were either receiving non-steroidal drugs or were untreated. Their DHEAS levels were compared to those of 20 patients with psoriasis, and to 23 patients with secondary osteoarthritis (OA). Assessing the patients by group, the mean DHEAS level was markedly lower in the pemphigoid/pemphigus than in the psoriasis and OA patients (geometric mean 600 vs. 2130 and 2100 nmol/l, respectively; p < 0.001). This difference was independent of steroid treatment. No correlation was found between the DHEAS levels and antibody titers. The low levels found in pemphigoid/pemphigus are concordant with those reported for systemic lupus erythematosus, rheumatoid arthritis and polymyalgia rheumatica/giant cell arteritis. DHEAS deficiency is a permanent feature in these autoimmune diseases, and may contribute to their etiology and/or pathophysiology.

Adult↗

PubMed1979

Immunosuppressive therapy in collagen vascular diseases.

Over the past ten to fifteen years cytotoxic drugs have gradually been added to the therapeutic armamentarium of physicians other than oncologists, particularly dermatologists. They are being used to treat nonmalignant cutaneous diseases such as pemphigus vulgaris, bullous pemphigoid, and psoriasis. The rationale for their use in these diseases is conjectural, yet those dermatologists who use them feel strongly not only that cytotoxic drugs are effective, but that when used properly, they are relatively safe. The immunosuppressive activity of cytotoxic drugs has been accepted and widely acclaimed in immunology, renology, and transplant medicine. However, their usefulness in two autoimmune diseases frequently managed by dermatologists is widely disputed. In this short discussion the rationale for the use of cytotoxic agents and evidence for their effectiveness when used appropriately in progressive systemic sclerosis and systemic lupus erythematosus is presented.

Antibody Formation↗

PubMed1995

Mechanisms of drug eruptions: Part I.

The pathogenetic mechanisms underlying common, and less common but severe, adverse cutaneous drug reactions are reviewed. Pharmacogenetic variability may account for a susceptibility to serious drug reactions to sulphonamides and anticonvulsants, as well as to lupus erythematosus (LE)-like syndrome. Exanthematous drug reactions may have an immunological basis. Cell mediated cutaneous drug reactions, including lichenoid reactions, LE-like syndrome, fixed drug eruption, erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis, will inevitably involve elements of the skin immune system. Graft-versus-host disease provides a useful model for aspects of these drug-induced disorders. Urticaria, angioedema, anaphylaxis and anaphylactoid reactions may involve Type I immunoglobulin (Ig)-mediated or Type III hypersensitivity, or may be caused by pharmacological, non-allergic means. Drug-induced vasculitis, serum sickness and the Arthus phenomenon are manifestations of the immune complex disease. Drug-induced pemphigus may involve immune dysregulation, but several thiol-containing drugs are able to cause antibody-independent acantholysis directly.

Drug Eruptions↗

PubMed1982

[Plasmapheresis in autoimmune syndromes. Indications. Results].

Since plasma is largely involved in autoimmune diseases, plasma exchanges have been used to treat these patients. In the first part of this paper the authors review the indications for plasmapheresis, which they divide into two groups: autoimmune diseases proper, and diseases with autoimmune phenomena. In the second part, the results available in the literature are analyzed. In many cases, it is extremely difficult to form an opinion, since the methods vary from one group of workers to the other. Unquestionably, improvement was observed in bullous pemphigus and pemphigoid, in Goodpasture's syndrome, in glomerulonephritis with autoantibodies and in myasthenia. In other cases, dramatic improvement was sometimes observed (notably in some cases of acute polyradiculoneuritis), but the small number of patients in each series makes it impossible to separate the effects of plasma exchanges and those of the immunosuppressive treatment frequently administered concomitantly.

Anemia, Hemolytic, Autoimmune↗

PubMed1990

The photoaggravated dermatoses.

The photoaggravated dermatoses are diverse diseases adversely affected by sunlight or artificial ultraviolet (UV) exposure. The role of UVR is often well-recognized, as in lupus erythematosus (LE) and bullous pemphigoid (BP). Immunologic theories for the cause of the photosensitivity in lupus erythematosus are the production of UV-DNA antibodies with subsequent immune complex formation, a partial defect in DNA repair following UV exposure, and the release or stimulation of potent cutaneous immunologic mediators. However, mechanisms are still not fully elucidated. Photosensitivity has also been described in erythema multiforme, actinic lichen planus, viral exanthems, cutaneous T-cell lymphoma, eczema, and psoriasis. Treatment is restriction of light exposure, use of high protection sunscreens, and treatment of the underlying disorder.

Animals↗