Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid.
ABSTRACT 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.
SourceAvailable from: scielo.brAnais Brasileiros de Dermatologia 12/2005; 80(6):643-650. DOI:10.1590/S0365-05962005000700012 · 0.87 Impact Factor
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ABSTRACT: Mucous membrane pemphigoid (MMP) is a subepithelial autoimmune mucocutaneous disease. It most frequently affects the oral mucosa, followed by ocular and nasal mucosa, nasopharyngeal, anogenital, skin, laryngeal and esophageal mucosa. Two half-sisters developed mucous membrane pemphigoid at approximately the same age. The older sister presented with primarily mucosal disease, while the younger had a more cutaneous disease. The histopathology demonstrated a subepithelial blister and direct immunofluorescence showed deposition of IgG and C3 at the basement membrane zone of perilesional tissues in both sisters. Antibodies to human β4 integrin were present in the sera of both patients and correlated with disease activity. Both sisters carried the same HLADQβ1* 0301 allele. This is the first case of mucous membrane pemphigoid occurring in two half-sisters. Perhaps, it is the low incidence of mucous membrane pemphigoid that may account for the lack of reports on familial cases of the disease.Journal of Dermatological Case Reports 03/2014; 8(1):9-12. DOI:10.3315/jdcr.2014.1162
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ABSTRACT: Das orale vernarbende Pemphigoid ist eine chronische bullöse Autoimmundermatose der Mundschleimhaut, die vor allem die Gingiva und Wangenschleimhaut befällt. Die genaue Pathogenese dieser Erkrankung ist jedoch bislang noch nicht eindeutig geklärt, aber es gibt Hinweise, dass es sich unter anderem um eine von T-Lymphozyten vermittelte Erkrankung handelt. Aus diesem Grund führten wir eine topische, immunmodulatorische Behandlung mit Tacrolimus bei 2 Patienten mit lange bestehendem, therapieresistentem oralem vernarbendem Pemphigoid durch. Unter dieser Behandlung konnten innerhalb von 3 Monaten eine komplette Abheilung und unter Fortführung eine anhaltende Remission erzielt werden.Der Hautarzt 04/2004; 55(4). DOI:10.1007/s00105-004-0714-7 · 0.54 Impact Factor