Article

[Autoimmune bullous skin disorders].

Klinik für Dermatologie und Allergologie, Marburg.
Therapeutische Umschau 09/2010; 67(9):465-82. DOI: 10.1024/0040-5930/a000080
Source: PubMed

ABSTRACT Autoimmune bullous skin disorders are rare, potentially fatal disorders of skin and mucous membranes which are associated with IgG or IgA autoantibodies against distinct adhesion molecules of the epidermis and dermal epidermal basement membrane zone, respectively. These autoantibodies lead to a loss of skin adhesion which shows up clinically as the formation of blisters or erosions. In pemphigus, loss of adhesion occurs within the epidermis while in the pemphigoids, linear IgA dermatosis, epidermolysis bullosa acquisita and dermatitis herpetiformis, loss of adhesion takes place within or underneath the basement membrane zone. The autoantigens of these disorders are largely identified and characterized. Making the diagnosis of autoimmune bullous skin diseases is based on histology and direct immunofluorescence of perilesional skin and the serological detection of autoantibodides by indirect immunofluorescence and recombinant autoantigens. Therapeutically, systemic treatment with glucocorticoids is combined with immunosuppressive adjuvants which allow for the fast reduction of systemic steroids. A prospective trial in pemphigus showed that adjuvant treatment with azathioprine, mycophenolate mofetil and cyclophosphamide, respectively, led to a significant reduction of the cummulative dose of systemic steroids until complete clinical remission was achieved. In bullous pemphigoid, topical treatment with clobetasol led to complete clinical remissions without major side effects seen when glucocorticoids were applied systemically. Therapeutic depletion of B cells by rituximab as a second line therapy has significantly improved the overall prognosis of pemphigus. Comparable controlled therapeutic trials have not yet been performed in dermatitis herpetiformis and epidermolysis bullosa acquisita.

Full-text

Available from: Michael Hertl, Jan 31, 2014
0 Followers
 · 
142 Views
  • Clinical lymphoma, myeloma & leukemia 08/2011; 11(6):517-20. DOI:10.1016/j.clml.2011.06.014 · 1.93 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pemphigus erythematosus (PE), auch bekannt als Senear-Usher-Syndrom, ist ursprünglich als eine Variante des Pemphigus mit klinischen Eigenschaften des Lupus erythematodes beschrieben worden. Heutzutage wird Pemphigus erythematosus als eine lokalisierte Form des Pemphigus foliaceus betrachtet und den blasenbildenden Autoimmundermatosen zugeordnet. Die autoantigene Struktur stellt das Protein Desmoglein 1, ein desmosomales Adhäsionsmolekül epidermaler Keratinozyten, dar. Wir berichten über einen 69-jährigen Mann mit seit ca. 3 Monaten auftretenden Erosionen im Bereich der Wangen, die sich im Verlauf auch am Stamm manifestierten. In Zusammenschau des klinischen Verlaufs, der Histopathologie sowie der Immunfluoreszenzuntersuchungen konnte die Diagnose eines Pemphigus erythematosus mit Übergang in einen Pemphigus foliaceus gestellt werden.
    Der Hautarzt 05/2012; 63(5). DOI:10.1007/s00105-012-2374-3 · 0.54 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: As the frequency and characteristics of chronic pruritus in autoimmune dermatoses (AID) have not yet been investigated, the present study aimed at characterizing pruritus in a representative group of patients with AID. A total of 35 patients (80% women) with AID were included, divided into 3 main groups (group 1; n = 19: bullous pemphigoid (BP), pemphigus vulgaris (PV); group 2; n = 9: scleroderma (SSc), morphea (Mo); group 3; n = 7: lupus erythematosus (LE), dermatomyositis (DM). Demographic data and pruritus characteristics were obtained by standardized questionnaires and statistically evaluated by SPSS 20.0. In group 1 (BP/PV) and group 3 (LE/DM), pruritus preceded the initial diagnosis of AID (2.1 ± 7.6 years and 9.5 ± 16.0 years). Patients in group 2 (SSc/Mo) reported pruritus initially 2.8 ± 8.6 years after the initial diagnosis. In group 1 (BP/PV) significantly (p < 0.05) more excoriations and relief by scratching were observed than in groups 2 (SSc/Mo) and 3 (LE/DM). While pruritus occurred as a prodromal symptom of BP/PV and LE/DM, it was only detected once the initial diagnosis of SSc/Mo was made. In contrast to BP/PV, the other forms of AID were associated with mechanically inducible pruritus with dysesthetic qualities. All forms of AID were associated with intensive pruritus which had a significant impact on quality of life.
    Der Hautarzt 06/2012; 63(7):558-66. DOI:10.1007/s00105-011-2319-2 · 0.54 Impact Factor