The treatment of pemphigus and pemphigoid

The management of pemphigus and pemphigoid is briefly reviewed. Consideration is given to the use of three intravenous daily ‘bolus’ injections of high-dose methyiprednisolone for the treatment of pemphigus, and to long acting tetraeosactrin (Synacthen Depot) injections with concurrent azathioprine for rapid control of bullous pemphigoid in the elderly. The great value of topical and intralesional steroid therapy used in conjunction with an anti-staphylococcal systemic antibiotic is stressed.     

Therapy of pemphigus and pemphigoid

Severe cases of pemphigus vulgaris should be treated with prednisone in high dosage (180-360 mg daily). In milder cases, we prefer the combined therapy with prednisone 40 mg every other day and an immunosuppressive drug, i.e. azathioprin, cyclophosphamide, or methotrexate. For treatment of bullous pemphigoid, the combined therapy is usually sufficient.     

天疱疮与类天疱疮的治疗

天疱疮与大疱性类天疱疮是最为常见的获得性大疱病。 50年代,皮质类固醇的问世并应用于大疱病的治疗,使这 2个重症皮肤病的预后大大得到改善。早期制定的大剂量服用皮质类固醇方案虽然能控制皮疹,但长期服药的结果也带来一系列的不良反应,如糖尿病、高血压、骨质疏松、股骨头无菌坏死等,甚至因不良反应导致死亡,如严重的感染、消化道溃疡穿孔造成大出血等。目前,对大疱病的治疗可选择不同的药物,然而皮质类固醇仍是治疗天疱疮、类天疱疮的首选药物,但如何合理使用,如何减少和预防不良反应的发生则是临床医生共同探讨的问题。本文…     

The management of mucous membrane pemphigoid and pemphigus

Mucous membrane pemphigoid and pemphigus vulgaris are autoimmune blistering disorders in which many similar drugs and therapeutic strategies are utilized. In general, localized disease can be treated with topical agents. In contrast, patients with more severe and progressive disease usually require a combination of systemic corticosteroids and immunosuppressive medications. Oral corticosteroids, adjuvant immunosuppressive agents, antibiotics such as dapsone and immunomodulatory procedures like intravenous immunoglobulin are the main therapeutic agents used in treating these two disorders. Much of the morbidity and mortality associated with these disorders are related to the sites involved and to the drugs used for therapy. Treatment should be individualized based on severity, extent, and rate of progression of disease, comorbidities, and age of the patient. Serum levels of specific autoantibodies and indirect immunofluorescence titers, in certain cases, can be used to monitor response to therapy.     

The pathophysiological disorders and experimental therapy in pemphigus and pemphigoid

Guinea pig experiments (n = 196) with simulation of pemphigus vulgaris and bullous pemphigoid helped detect pathophysiologic and metabolic disorders similar for these autoimmune bullous dermatoses: decompensated endoproteinase-inhibitor system, impaired ratio of cyclic nucleotide to arachidonic acid oxigenase metabolism products concentrations in the blood of experimental animals. The key point in the treatment of animals with pemphigus vulgaris was endogenic proteinase inhibition, that in the treatment of bullous pemphigoid--blocking of mononuclear macrophagal histolytic potential.     

Coexistence of bullous pemphigoid and pemphigus foliaceus

A patient with bullous pemphigoid developed pemphigus foliaceus 5 years later. Mixed bullous diseases are rare and this is the first reported case of coexisting bullous pemphigoid and pemphigus foliaceus.     

The hormonal profile of patients with pemphigus and bullous pemphigoid

Radioimmunoassays of some hypophyseal, adrenal, thyroid, pancreatic, and sexual gland hormones, carried out in patients with pemphigus and bullous pemphigoid, have revealed increased levels of ACTH and hydrocortisone in their blood sera, vs. the reference values. Glucocorticoid therapy has normalized the ACTH level, and hydrocortisone content has proved to be below the norm. The blood plasma triiodothyronine level has been elevated, whereas insulin and glucagon lowered in the patients with pemphigus vs. those with bullous pemphigoid. In females of a menopausal age, suffering from pemphigus, increased levels of estradiol and reduced ones of testosterone have been detected, as compared to those suffering from bullous pemphigoid. These data evidence a varying functional activity of the endocrine glands in these dermatitides.     

Immune complexes in pemphigus and bullous pemphigoid

28 serum and 10 blister fluid specimens obtained from 28 pemphigus vulgaris (PV) patients were assayed for immune complexes using the polyethylene glycol (PEG)assay. 11% of sera and 30% of the blister fluids have elevated levels of immune complexes. Anti-intercellular cement substance (ICS) antibody could not be detected in PEG precipitates, but was present in the supernatants from the serum. However, anti-ICS antibody was found in 70 of the precipitated complexes from the blister fluid. 18 serum and 31 blister fluid specimens obtained from 18 bullous pemphigoid (BP) patients were assayed for immune complexes using the PEG assay. 17% of sera and 31% of the blister fluids have elevated levels of immune complexes. Antibasement membrane zone (BMZ) antibody could not be detected in the PEG precipitates, but was present in the supernatants obtained from the sera. Anti-BMZ antibody was found in 57% of the precipitated complexes from the blister fluids. This data further supports the hypothesis that the majority of the complexes in PV and BP are formed in situ.     

Frequency of IgA antibodies in pemphigus, bullous pemphigoid and mucous membrane pemphigoid

Circulating and bound IgA antibodies can be found in the autoimmune blistering diseases, but their prevalence, clinical relevance and target antigens remain unknown. Thirty-two patients with pemphigus, 73 with bullous pemphigoid and 28 with mucous membrane pemphigoid were studied retrospectively. Direct immunofluorescence (DIF) analysis of IgG, IgA, IgM and C3 was carried out for all cases. Sera were studied by standard indirect immunofluorescence, indirect immunofluorescence on salt-split skin, immunoblotting for bullous pemphigoid and mucous membrane pemphigoid and ELISA for pemphigus. With DIF, we found IgA autoantibodies in 22 of all 133 cases. Circulating IgA antibodies to skin were detected in 2 of 3 IgA-DIF-positive patients with pemphigus, in 3 of 6 with bullous pemphigoid, and in 6 of 13 with mucous membrane pemphigoid. We confirm that the IgA reactivity is more frequently associated with mucous membrane involvement, especially in cases without critical involvement (5/8). The role of IgA and its antigenic specificity in these diseases remain unclear.     

Minocycline-induced hyperpigmentation in patients with pemphigus and pemphigoid

BACKGROUND: Immunosuppressive medications typically used to treat the immunobullous disorders pemphigus vulgaris, pemphigus foliaceous, and bullous pemphigoid can have serious adverse effects. The tetracycline family of antibiotic drugs has been shown to be effective in the treatment of these conditions with a more favorable side effect profile. Minocycline hydrochloride use has been associated with various forms of hyperpigmentation, and its incidence is well reported in acne vulgaris and rheumatoid arthritis. We examined a series of 9 patients treated with minocycline for pemphigus or pemphigoid, most of whom have developed cutaneous hyperpigmentation. OBSERVATIONS: Seven of 9 patients treated with minocycline, 50 mg daily (1 patient) or 100 mg twice daily (8 patients), for pemphigus vulgaris, pemphigus foliaceous, or bullous pemphigoid developed hyperpigmentation, which necessitated discontinuing therapy. Five of these patients had experienced notable clinical improvement of their immunobullous disease with minocycline therapy. The average duration of treatment was 8.2 months (range, 1-25 months). The second most common adverse effect in our group was oral candidiasis, which occurred in 2 patients. CONCLUSIONS: We found a favorable response to minocycline therapy in 5 of 9 patients. However, 7 patients developed localized hyperpigmentation as early as 1 month after starting medication use. This incidence of minocycline-induced hyperpigmentation is significantly higher in immunobullous disease than in acne vulgaris or rheumatoid arthritis. This increased incidence may be related to an increase in pigment deposition complexed with collagen during the remodeling process, subclinical inflammation, or glucocorticosteroid-induced skin fragility. The hyperpigmentation process was reversible, as most of our patients had fading of their pigmentation after minocycline cessation.     

得宝松皮损内注射治疗天疱疮和大疱性类天疱疮口腔粘膜损害

使用皮损内注射得宝松 (复方倍他米松 )治疗天疱疮、大疱性类天疱疮,并与未使用此疗法治疗的患者作一比较。口腔粘膜损害使用局部注射得宝松治疗组 (天疱疮＋大疱性类天疱疮 )29例,口腔粘膜损害平均愈合时间为 0.8± 0.8月;口腔粘膜损害未用局部注射得宝松治疗组 (天疱疮＋大疱性类天疱疮 )31例,口腔粘膜损害平均愈合时间为 1.5± 1.3月。经统计学比较分析 t=2.49, P< 0.01,两组口腔粘膜损害平均愈合时间具有显著性差异,前组明显短于后组。采用皮损内注射得宝松治疗口腔粘膜的顽固损害,药物直达病灶,疗效快,并可减少系统用药量,减少药物副作用。     

Phylogenetic studies with pemphigus and pemphigoid antibodies.

Indirect immunofluorescence (IF) studies were performed on skin from a variety of vertebrate specimens and IgG fractions from pemphigoid and pemphigus sera. Pemphigoid antigen was present in fish, amphibian, reptilian, avian and mammalian skin, whereas pemphigus antigen was observed in avian and mammalian skin only.     

Combined features of pemphigus and pemphigoid induced by penicillamine

D-Penicillamine is able to induce a bullous eruption with the clinical, histological and immunological features of pemphigus. We present a case that had all the characteristics of pemphigus, but also in vivo bound IgG against the dermo-epidermal junction, i.e. the immunological features of bullous pemphigoid. Since no subepidermal blistering was observed, it is concluded that the penicillamine-induced auto-antibodies against the basement membrane zone were biologically inactive.