Pemphigus with clinical, histological and immunological features of both vulgaris and foliaceus subtypes.
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Autoantibodies↗
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Rituximab in refractory autoimmune bullous diseases.
Treatment of autoimmune blistering diseases consists of systemic glucocorticosteroids usually in combination with additional immunosuppressants such as azathioprine and mycophenolate mofetil or immunomodulators such as dapsone, antibiotics, intravenous immunoglobulins, and immunoadsorption. In some patients, these treatment regimens are not sufficient to control disease activity and/or lead to intolerable adverse events. Rituximab, originally developed for the treatment of non-Hodgkin's lymphoma, is an anti-CD20 humanized monoclonal antibody leading to transitory B-cell depletion. For this indication, rituximab is widely employed, and severe side-effects rarely observed. Subsequently, the B-cell-depleting effect of rituximab has been exploited successfully in various autoimmune disorders, including autoimmune blistering diseases. Here, we review the effect of rituximab in such diseases. To date, application of rituximab has been reported in 26 treatment-resistant patients with the vulgaris, foliaceus, and paraneoplastic variants of pemphigus as well as in bullous pemphigoid and epidermolysis bullosa acquisita. All but a single patient showed clinical improvement with reduction of lesion formation. In about a third, a clinical remission requiring further immunsuppressive medication was achieved, and in about a quarter, complete remission was induced. In addition, the mode of action and adverse events of rituximab as well as adjuvant immunosuppressive treatments, and the effect on levels of circulating autoantibodies in these patients are discussed.
Antibodies, Monoclonal↗
[The indirect immunofluorescence test for the identification of auto-immune skin diseases (author's transl)].
The indirect immunofluorescence test has acquired great importance in the diagnosis of auto-immune skin diseases. Since it is sufficient only to send a sample of whole blood or blood serum to carry out the test, it is also of practical interest to the general practitioner. The test should be called upon if any of the following auto-immune skin diseases are suspected: pemphigus vulgaris (including p. vegetans, p. foliaceus, p. erythematosus), bullous pemphigoid (including benign mucosal pemphigoid) lupus erythematodes (systemic) and diffuse scleroderma.
Autoimmune Diseases↗
[Bullous pemphigoid with pemphigus type antibodies in vivo. 2 cases].
Pemphigus vulgaris, whether of the vulgaris or foliaceus variety, and bullous pemphigoid (BP) are two groups of auto-immune bullous diseases which in most cases can easily be differentiated on the basis of clinical, histological and, mainly, immunopathological data. Like cicatricial pemphigoid, BP may be accompanied with circulating pemphigus-like antibodies (PLA) which are not detected in vivo by direct immunofluorescence (IF). However, a true pemphigus-BP association, as reported first by Chorzelski et al., is exceptional. Two cases of BP immunolabelled with pemphigus-like antibodies at direct IF are reported, raising a discussion on this particular association. The first case concerns a 62-year old man presenting with extensive psoriasis treated with salicylated vaseline and topical corticosteroids. The patients was admitted for a disseminated, symmetrical and pruriginous bullous eruption made up of tense bullae on healthy and psoriatic skin or on an urticarial background, without Nikolsky's sign. Pathological examination of a recent bulla showed subepidermal detachment without acantholysis. Direct cutaneous IF revealed linear labelling of the basement membrane zone with IgG, C3 and C1q, and labelling of the inter-cellular substance of the epidermis with IgG. Indirect IF on O+ human skin demonstrated antibodies of the pemphigoid type (1/128) and of the pemphigus type (1/64). Standard laboratory examinations only showed moderate blood eosinophilia (950/mm3) and a rise in total IgE. Under systemic corticosteroid therapy (prednisone 1 mg/kg/day) and azathioprine (2 mg/kg/day) the bullae rapidly disappeared.(ABSTRACT TRUNCATED AT 250 WORDS)
Aged↗
Plasmapheresis as a supplementary treatment in pemphigus.
Four cases with pemphigus 3 p. vulgaris and one p. foliaceus were treated with plasmapheresis. The volume of plasma exchange was 500-800 ml once a week for 6 weeks. In one patient the procedure was repeated four times, within 8 months. All patients were treated with prednisone and azathioprine or cyclophosphamide at the same time, but in lower dosages. The results were satisfactory and the remission after the treatment lasted for 11 months to 2 years.
Adult↗
Pemphigus: an epidemiological study of patients treated in Finnish hospitals between 1969 and 1978.
Between 1969 and 1978, 44 pemphigus (p.) patients were treated in Finnish hospitals. The number of new cases was 36, giving an annual incidence of 0.76 cases per million inhabitants. The male/female ratio was 0.9:1. Nine patients had p. vulgaris (p.vu.); one p. vegetans (p.ve.); 9, p. foliaceus (p.fo.); 22, p. erythematodes (p.er.); and 3, p.NUD (p.NUD). One patient with p.er. also had myasthenia gravis. The mean age at onset was 57 1/2 years. The disease started in the mouth only in p.vu. (78% of cases) and in p.NUD (67%). Prednisone (or its derivatives) was most commonly used for systemic treatment, the mean initial dose being 61.4 mg/day. P.vu. and p.NUD required higher initial doses of prednisone and a longer duration of the high-dose treatment. The smallest mean daily dose of prednisone required to achieve a remission of at least 6 months was 5.2 mg. The mean duration of remission was 30 1/2 months. The frequency of side effects of systemic drug therapy was low (18%), possibly because only comparatively low doses of prednisone were used in Finland. The cause of death in 3 (6.8%) out of 44 patients was attributable either to p. itself or to its treatment. The mean annual number of hospitalization days for the whole material was 16.4 days and the mean annual number of hospital admissions 0.7.
Adolescent↗
[Clinical consequences of the altered function of the epidermal desmosomes in man].
The epidermic desmosomes are formed in their molecular structure by transmembrane components from the cytoplasmic plaque. These are linked up to the tonofilaments of keratin forming a continuous proteinic web which is crucial to keep the tissue integrity. The functional and structural deterioration of the desmosomes produces in human clinic a series of very typical skin diseases, the most frecuent of which is the penghigus. In them, some molecular parts of the desmosomes become strange and antigenic and trigger to an autoimmune reaction which produces a blistering disease of serious prognosis. Depending on the affected molecules the clinical manifestation are different and as Antholt says there is to make a distinction of the following varieties: phenfigus vulgaris and vegetant, phenfigus foliaceus and its varietie, phenfigus of herpes shape. For is study, it is necessary to bear in mind the clinic, the histopathology and inmunopathology (Inmunofluorescence). Some other stranger diseases are of a hereditary genetic origin because of mutations in the domains of the desmosomal molecules which produce clinical manifestations of keratodermia, hypotricosis, woolly hair, skin frailty and cardiomyopapathy. Finally, there are bacteriological staphylococcal toxins, "exfoliatin A-toxin" which produce a separation of the extracellular domain of the desmoglein 1 causing the "staphylococical scalded skin syndrome".
Desmosomes↗