Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis.
Summary of "Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis."
Summary Background: Immunoapheresis/immunoadsorption is a specific tool to remove immunoglobulins and immune complexes from the circulation. Immunoapheresis is successfully used in various autoantibody-mediated diseases (such as autoimmune renal disease and others). In dermatology immunoapheresis is increasingly applied as an adjuvant treatment for severe autoimmune bullous diseases. Case report: We successfully employed adjuvant immunoapheresis to treat a 57-year-old man with life-threatening pemphigus vulgaris and a 30-year-old pregnant woman with severe pemphigoid gestationis. Immunoapheresis induced a rapid improvement and almost complete clearance of clinical symptoms without notable side effects. The clinical improvement was paralleled by a decline of the pathologic circulating autoantibodies. Following stabilization of his disease with immunoapheresis, the pemphigus vulgaris patient was continued on rituximab and remained almost free of symptoms for the next 12 months. The patient with pemphigoid gestationis was subsequently treated with systemic corticosteroids until the symptoms of her self-limited disease ceased. Conclusion: Immunoapheresis might represent an excellent therapy for certain patients with severe pemphigus vulgaris or pemphigoid gestationis, unresponsive to conventional treatment regimens. We observed rapid improvement of clinical symptoms and no notable side effects.
Affiliation
Department of Dermatology, Krankenanstalt Rudolfstiftung, Vienna, Austria.
Journal Details
This article was published in the following journal.
Name: Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
ISSN: 1610-0387
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20812992
- DOI: http://dx.doi.org/10.1111/j.1610-0387.2010.07500.x
Medical and Biotech [MESH] Definitions
Pemphigoid Gestationis
An itching, autoimmune, bullous SKIN disease that occurs during the last two trimesters of PREGNANCY and PUERPERIUM. It also affects non-pregnant females with tissue of PLACENTA origin, such as CHORIOCARCINOMA; or HYDATIDIFORM MOLE. It exhibits antigenic and clinical similarity to bullous pemphigoid (PEMPHIGOID, BULLOUS). This disease does not involve herpes viruses (old name, herpes gestationis).
Acantholysis
Separation of the prickle cells of the stratum spinosum of the epidermis, resulting in atrophy of the prickle cell layer. It is seen in diseases such as pemphigus vulgaris (see PEMPHIGUS) and DARIER DISEASE.
Pemphigoid, Bullous
A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.
Hla-dr Antigens
A group of D-related human leukocyte antigens that are polymorphic glycoproteins found on lymphoid cells. They consist of alpha and beta chains and their inheritance differs from that of the DQ and DP antigens; their presence seems to be associated with certain skin diseases like pemphigus vulgaris, dermatitis herpetiformis, and type I diabetes.
Desmoglein 3
A desmosomal cadherin that is an autoantigen in the acquired skin disorder PEMPHIGUS VULGARIS.
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