Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is an autoimmune mucocutaneous, blistering disease which can lead to blindness and/or death from sudden asphyxiation, secondary to a scarring process. Conventional therapy for the treatment of MMP consists of high-dose systemic corticosteroids and/or immunosuppressive agents. Some patients do not respond to these treatments and develop multiple serious side effects, which can be potentially fatal. In such patients, alternative treatment modalities are needed. This study presents the use of intravenous immunoglobulin (IVIg) therapy in 15 patients with severe MMP whose disease was nonresponsive to the prolonged use of high-dose systemic corticosteroids and immunosuppressive agents and who developed multiple side effects to them. All 15 patients received an IVIg dose of 1-2 g/kg/cycle. The following objective parameters were used to assess the clinical outcome pre- and post-IVIg therapy: number of side effects, frequencies of recurrences and relapses, duration and total dosage of prednisone therapy, and the quality of life. The differences in these variables between the pre- and post-IVIg data were statistically analyzed using the SAS UNIVARIATE software running the two-sided Wilcoxon signed-rank and sign tests. A statistically significant difference was observed between pre- and post-IVIg therapy data when comparing the aforementioned variables. All 15 patients had an effective clinical response, were able to discontinue previous systemic therapies, and eventually achieved a prolonged clinical remission. IVIg improved the quality of life in all 15 patients and demonstrated a steroid-sparing effect. No serious side effects were observed. IVIg therapy is a safe and effective alternative modality in the treatment of patients with nonresponsive and progressive MMP and can induce a sustained clinical remission.
"In at least one of these trials, 15 MMP patients with multiple mucosal involvement experienced complete remission off therapy for an average of only 24 months (Sami et al, 2002a). Thus, it is still uncertain whether treatment regimens that get MMP under control can be eliminated to allow patients to be in drug-free remission or they should be continuously administered in some capacities. "
[Show abstract][Hide abstract] ABSTRACT: Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune subepithelial blistering diseases affecting primarily mucous membranes showing marked degree of clinical and immunological variability. We investigated four controversial topics: (i) Does oral pemphigoid (OP) really exist as a separate entity? (ii) Is mucous membrane pemphigoid curable? (iii) What is the best therapeutic option for MMP? (iv) Does exclusive oral IgA dermatitis exist as a distinct entity from MMP? Results from extensive literature searches suggested that (i) it is still unclear whether patients with OP could be considered as a distinct subset of MMP with specific clinical and immunological features; (ii) it is uncertain whether treatment regimens that get MMP under control can be eliminated to allow patients to be in drug-free remission or they should be continuously administered in some capacities; (iii) there is a concerning paucity of good-quality trials on MMP and available recommendations are solely based on generally small patients' cohorts or case series. Some of the 2002 consensus experts' opinions should be possibly updated, particularly regarding the safety of sulfa drugs; (iv) we did not find any strong evidence to support an exclusive oral (and perhaps also mucosal) form of LAD as a separate entity.
"Azathioprine (Dave and Vickers 1974) and MMF are generally less effective but may be used in patients who cannot tolerate cyclophosphamide or prednisone. Multiple reports have documented the effectiveness of high dose IVIg in the treatment of MMP, including patients who were refractory to other therapies (Ahmed and Moy 1981; Foster and Ahmed 1999; Heilborn et al 1999; Letko et al 2000; Ahmed and Colon 2001; Mackay and Rosen 2001; Sami et al 2002a, 2002b). "
[Show abstract][Hide abstract] ABSTRACT: Autoimmune bullous diseases result from an immune response to molecular components of the desmosome or basement membrane. Bullous diseases are associated with a high degree of morbidity and occasional mortality. Therapy of bullous diseases consists of suppressing the immune system, controlling inflammation and improving healing of erosions. The therapeutic agents used in the treatment of bullous diseases may be associated with high morbidity and occasional mortality. Successful treatment requires understanding of the pathophysiology of the disease process and the pharmacology of the drugs being used.
[Show abstract][Hide abstract] ABSTRACT: •
Bullous pemphigoid (BP) is a subepidermal autoimmune blistering skin disease, and is predominantly a disease of the elderly
in its classic presentation.
There are distinct clinical variants of this disease: bullous pemphigoid, cicatricial pemphigoid, and pemphigoid gestationis.
The autoimmune response in BP is directed against two hemidesmosomal antigens, BP180 (BPantigen2 or collagen XVII) and BP230
(BPantigen1). In CP, autoantibodies recognize multiple auto antigens, including BP180, BP230, laminin-5, laminin-6, and integrin
B4 subunit. Autoantibodies in pemphigoid gestations are maily reative with BP180.
The diagnosis of this condition is based on clinical findings, histology, and the demonstration of tissue bound immunoreactants
Therapy can range from topical glucocorticosteroids for mild disease, to oral antibiotics, to systemic glucocorticosteroids
alone or with cytotoxic drugs or intravenous immunoglobulin (IVIG).
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