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


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.


Available from: Michael Hertl, Jan 31, 2014
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    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.
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    ABSTRACT: Context: Bullous pemphigoid is mediated by autoantibodies primarily targeting two structural proteins of basement membrane hemidesmosomes, BP180 (BPAG2; collagen XVII) and BP230 (BPAG1). Case Report: A 70-year-old Caucasian male patient was evaluated for a seven day history of multiple itching, erythematous blisters on his extremities. Biopsies for hematoxylin and eosin examination, direct immunofluorescence and indirect immunofluorescence (including salt split skin analysis) were performed. Results: Hematoxylin and eosin examination demonstrated a subepidermal blister. Within the blister lumen, numerous eosinophils and lymphocytes were noted. Direct and indirect immunofluorescence revealed linear deposits of IgG, Complement/C3 and fibrinogen at the basement membrane zone of the skin and surrounding selected dermal blood vessels and sweat glands. Positive intracytoplasmic staining for anti-human IgD was noted in most of the epidermis, as well as surrounding some dermal blood vessels. Indirect immunofluorescence utilizing monkey esophagus substrate demonstrated strong positivity within the endomysium for IgG antibodies. Conclusion: We report a unique case of bullous pemphigoid with reactivity to eccrine sweat glands, and selected dermal blood vessels. In addition, the observed reactivity of anti-human IgD, and of IgG to monkey esophagus endomysium warrant further investigation.

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