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  • 1
    Electronic Resource
    Electronic Resource
    Pemphigoid gestationis ohne Blasenbildung (2000)
    Springer
    Der Hautarzt 51 (2000), S. 25-30 
    Details
    ISSN: 1432-1173
    Keywords: Schlüsselwörter ; Herpes gestationis ; Schwangerschaftsdermatose ; Bullöses Pemphigoid ; BP180 ; Pruritus ; Key words ; Herpes gestationis ; Dermatoses of pregnancy ; Bullous pemphigoid ; BP180 ; Pruritus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background and Objective: Pemphigoid gestationis (PG) is a rare pregnancy-associated autoimmune bullous disease characterized by autoantibodies to the 180 kD bullous pemphigoid antigen (BP180). The clinical spectrum of PG is polymorphic and for diagnostic purposes, a skin biopsy is usually taken demonstrating the deposition of autoantibodies. Patients and Methods: From 2 patients, skin biopsies were obtained for histopathologic and immunofluorescence studies. Circulating autoantibodies were characterized by immunoblotting and ELISA using a recombinant form of the immunodominant BP180 NC16 A domain. Results: The 2 PG patients described here did not show blisters but complained about severe itching. In the first case, PG presented in the first trimester of the second pregnancy as an erythema-multiforme-like disease. The second patient developed urticarial plaques a few days after delivery. PG was diagnosed by the detection of autoantibodies against recombinant BP180 NC16 A by immunoblot and ELISA analysis and confirmed by linear deposits of C3 at the cutaneous basement membrane zone on direct immunofluorecence microscopy. Skin lesions healed with oral prednisolon. Conclusions: In our two patients, non-bullous PG could be diagnosed by serological tests. Immunmoblotting and ELISA might be sensitive and specific tools when screening sera of patients with pruritic skin lesions in pregnancy for the presence of autoantibodies to BP180. In some cases, these newer techniques may make a skin biopsy unnecessary.
    Notes: Zusammenfassung Hintergrund und Fragestellung: Das Pemphigoid gestationis (PG) ist eine seltene, schwangerschaftsspezifische Autoimmundermatose mit Autoantikörpern gegen das 180 kD bullöse Pemphigoid Antigen (BP180). Das klinische Bild ist polymorph. Die Diagnose des PG wird meist anhand einer Biopsie mit Nachweis der Autoantikörper in der Haut der Patientinnen gesichert. Patienten/Methodik: Bei 2 Patientinnen wurden Hautbiopsien histopatholgisch und immunfluoreszenzoptisch untersucht. Die Autoantikörper wurden mittels Immunoblot und ELISA unter Verwendung einer rekombinanten Form der immundominanten BP180-NC16-A-Domäne charakterisiert. Ergebnisse: Bei den beiden vorgestellten Patientinnen verlief das PG ohne Blasenbildung, jedoch mit starkem Juckreiz. Bei unserer ersten Patientin manifestierte sich die Erkrankung im 1. Trimenon unter dem Bild eines Erythema multiforme, der 2. Fall trat wenige Tage postpartal unter dem Bild einer Urtikaria auf. Die Diagnose wurde jeweils durch den Nachweis von Antikörpern gegen rekombinantes BP180 NC16 A im Immunoblot und ELISA und durch lineare C3-Ablagerungen an der Basalmembran in der direkten Immunfluoreszenz gesichert. Die Hautveränderungen heilten bei beiden Patientinnen unter oraler Prednisolontherapie ab. Schlussfolgerung: Bei den beiden vorgestellten Patientinnen wurde anhand von Serumuntersuchungen ein PG ohne Blasenbildung diagnostiziert. Sowohl Immunoblot als auch ELISA erscheinen aufgrund ihrer hohen Sensitivität und Spezifität als Screeningverfahren zum Nachweis von Autoantikörpern gegen BP180 bei pruriginösen Schwangerschaftsdermatosen geeignet. In der Zukunft dürften sie mitunter die bisher unverzichtbare Hautbiopsie zur Diagnose eines PG ersetzen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050006
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  • 2
    Electronic Resource
    Electronic Resource
    Increased incidence of pemphigus vulgaris in southern Europeans living in Germany compared with native Germans (2002)
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 16 (2002), S. 0 
    Details
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background The two major subtypes of pemphigus include: pemphigus vulgaris (PV) and pemphigus foliaceus. Only limited data are available on the epidemiology of these diseases.Objective The aim of the present study was to estimate the gender- and age-specific incidences of PV in two well-defined regions of Germany and to compare the incidences among native Germans with those in citizens from other countries living in Germany.Methods We performed a retrospective review of records from all patients that were diagnosed with PV at the Departments of Dermatology in Würzburg and Mannheim between 1989 and 1997.Results During the observation period, 14 patients were diagnosed with PV coming from an area with 1.46 million residents. With regard to the patients’ age, the highest incidence for women was found in the 51–65-year-old-age group with 2.34 [95% confidence interval (CI): 0.36; 7.76] new cases per 1 million inhabitants per year. The highest incidence for men was observed in the over 65-year-old age group with 3.72 (95% CI: 0.95; 9.8) cases/million per year. In the 51–65-year-old age group, we found a 25-fold higher incidence of PV in foreigners living in Germany compared with native Germans. The age-adjusted incidence of PV was ninefold higher in foreigners compared with native Germans. Interestingly, all non-German patients came from two southern European countries (Turkey and Italy).Conclusions The age-adjusted incidence of PV differs between native Germans and foreigners living in Germany. Further studies are necessary to address the risk factors (genetic and/or environmental) that contribute to this difference.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1468-3083.2002.00384.x
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  • 3
    Electronic Resource
    Electronic Resource
    Tufted hair folliculitis associated with pemphigus vulgaris (2003)
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 17 (2003), S. 0 
    Details
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Tufted hair folliculitis (THF) is a rare disease which is characterized by the emergence of multiple hairs from widely dilated follicular orifices surrounded by an inflammatory infiltrate resulting in scarring alopecia. The pathogenesis is not yet fully understood. Although colonization with Staphylococcus aureus could not always be detected and systemic treatment with antibiotics alone is not sufficient, this microorganism is considered to play an important role. Around 30 patients with THF have been reported since the first publication. We present a patient with pemphigus vulgaris who developed THF. To our knowledge, this is the fourth case with an association of these two entities. Amongst other causing mechanisms, the autoimmune reaction may play an important role for the development of THF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1468-3083.2003.00664.x
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  • 4
    Electronic Resource
    Electronic Resource
    Childhood epidermolysis bullosa acquisita: a novel variant with reactivity to all three structural domains of type VII collagen (2002)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 147 (2002), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Most patients with epidermolysis bullosa acquisita develop an autoimmune response to the non-collagenous (NC) 1 domain of type VII collagen. We report a 4-year-old girl of white European descent presenting with widespread blistering disease involving the face, hands, genital area and oral mucosa. Histopathology revealed subepidermal blisters, and linear deposits of IgG and C3 were seen along the dermal-epidermal junction on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. On indirect IF microscopy, circulating autoantibodies exclusively stained the dermal side of 1 mol L−1 NaCl-split skin. The patient's IgG autoantibodies labelled a 290-kDa protein on Western blotting of dermal extracts, and reacted with the NC1, NC2 and triple helical domains of type VII collagen on immunoblotting of recombinant and cell-derived fragments obtained by pepsin and collagenase digestion of the full-length protein. Oral methylprednisolone and dapsone led to clearance of lesions, which healed with mild scarring and milia formation. Treatment was discontinued after 1 year and the patient has now been in remission for more than 3 years.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2002.04863.x
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  • 5
    Electronic Resource
    Electronic Resource
    Cicatricial pemphigoid with circulating autoantibodies to β4 integrin, bullous pemphigoid 180 and bullous pemphigoid 230 (2001)
    Oxford, UK : Blackwell Science, Ltd
    British journal of dermatology 145 (2001), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Cicatricial pemphigoid is a heterogeneous group of autoimmune subepidermal blistering diseases associated most commonly with autoantibodies to bullous pemphigoid (BP)180 and less frequently with those to laminin 5 or type VII collagen. In addition, a few cases have been described with autoantibodies to the β4 subunit of α6β4 integrin. We describe a patient with extensive disease of ocular, oral, pharyngeal, laryngeal and genital mucous membranes that healed with scarring of conjunctivae. IgG autoantibodies bound to the dermal–epidermal junction on direct immunofluorescence (IF) microscopy and to the epidermal side of 1 mol L−1 NaCl-split skin on indirect IF microscopy. Our patient's circulating IgG recognized a 205-kDa protein in extracts of 293T cells transfected with the β4 subunit of α6β4 integrin and in the cell extract of DJM-1 cells. Our patient's IgG and IgA autoantibodies also reacted with full-length BP180 derived from epidermal extracts and the ectodomain of BP180 (LAD-1) derived from culture supernatant of keratinocytes. In addition, a weak IgG reaction with BP230 was noted. The disease rapidly responded to dexamethasone-cyclophosphamide pulse therapy, and immunoblot reactivity to both β4 integrin and BP180 decreased according to disease activity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2001.04543.x
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  • 6
    Electronic Resource
    Electronic Resource
    IgG, IgA and IgE autoantibodies against the ectodomain of desmoglein 3 in active pemphigus vulgaris (2001)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 144 (2001), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background IgG autoantibodies against desmoglein (Dsg) 3 play a key part in the pathogenesis of pemphigus vulgaris (PV), the most severe autoimmune bullous disorder. Objectives To determine whether immunoglobulin isotypes other than IgG are detectable in the sera of patients with PV and whether a particular immunoglobulin subtype is associated with a distinct clinical phenotype of PV. Methods Sera from 41 patients with acute-onset, chronic active, and remittent PV disease with mucosal and cutaneous lesions were assayed against a baculovirus-expressed Dsg3 protein by immunoblot analysis. Results In acute-onset PV, Dsg3-reactive IgG1 was detected in nine of 15 (60%), IgG4 in 14 of 15 (93%), IgA in nine of 15 (60%) and IgE in two of 15 (13%) sera. In chronic active PV, Dsg3-reactive IgG1 was detected in 11 of 18 (61%), IgG4 in 16 of 18 (89%), IgA in 13 of 18 (72%) and IgE in two of 18 (11%) sera. In contrast, sera from patients with remittent PV disease contained only Dsg3-reactive IgG1 in six of eight (75%) and IgG4 in four of eight (50%) cases, but not Dsg3-reactive IgA or IgE. Conclusions In extension of previous findings, our study demonstrates that, in addition to IgG autoantibodies, IgA and occasionally IgE autoantibodies reactive with Dsg3 are present in acute and chronic active PV. The detection of Dsg3-reactive autoantibodies of the IgG4, IgA and IgE subclasses in active PV provides additional evidence that PV is a T-helper 2-regulated autoimmune disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2001.04228.x
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  • 7
    Electronic Resource
    Electronic Resource
    Autoantibodies in anti-p200 pemphigoid stain skin lacking laminin 5 and type VII collagen (2000)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 143 (2000), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report the case of a patient with a widespread bullous skin disease and linear deposits of IgG and C3 at the dermal–epidermal junction using direct immunofluorescence microscopy. Indirect immunofluorescence analysis demonstrated circulating IgG autoantibodies that stained, like autoantibodies to laminin 5 and type VII collagen, the dermal side of 1 mol L−1 NaCl-split human skin. By immunoblotting dermal extracts, the patient’s serum, like serum samples from two control patients, reacted with a 200-kDa protein. Using immunoelectron microscopy, the serum labelled a component of the lower lamina lucida, but not the lamina densa/sublamina densa region, distinguishing this from the type VII collagen localization pattern. By immunofluorescence microscopy on skin sections from patients lacking either laminin 5 (Herlitz’s epidermolysis bullosa) or type VII collagen (recessive dystrophic epidermolysis bullosa of Hallopeau–Siemens), the patient’s serum retained reactivity with these test substrates. The patient’s disease responded rapidly to the use of topical corticosteroids and lesions healed without scarring or milia formation. Our results provide strong evidence for the hypothesis that the 200 kDa autoantigen is different from laminin 5 and type VII collagen. For this new disease, we propose the designation ‘anti-p200 pemphigoid’.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2000.03841.x
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  • 8
    Electronic Resource
    Electronic Resource
    Epidermolysis bullosa acquisita with ultraviolet radiationsensitivity (2000)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 142 (2000), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A 37-year-old male patient developed a bullous eruption and erythematous plaques mainly in exposed areas following prolonged sun exposure. In addition, blisters were noted on oral and nasal mucous membranes. Histopathological examination of a lesional skin biopsy revealed a subepidermal blister. Linear deposition of IgG and C3 at the epidermal basement membrane zone was revealed by direct immunofluorescence microscopy of a perilesional skin biopsy. Indirect immunofluorescence on 1 mol/L salt-split skin showed binding of autoantibodies to the dermal side of the split. Immunoblot analysis of dermal extracts demonstrated that the patient’s serum contained IgG antibodies against type VII collagen, whereas no reaction was seen with epidermal extracts or by enzyme-linked immunosorbent assay using a recombinant form of bullous pemphigoid 180. Standardized ultraviolet (UV) radiation provocation induced blistering with both UVA (13·5 J/cm2) and UVB (0·04 J/cm2) within 24 h clinically and histologically. External and systemic UV-protective medication and nine cycles of high dosage immunoglobulins given intravenously (1·2 g/kg body weight over 2–3 days every 4 weeks) resulted in the cessation of blister formation. To the best of our knowledge, this is the first report of a case of epidermolysis bullosa acquisita with sensitivity to UV.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2000.03368.x
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  • 9
    Electronic Resource
    Electronic Resource
    Successful treatment of refactory mucosal lesions of pemphigus vulgaris using intravenous gammaglobulin as adjuvant therapy. (1996)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 135 (1996), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1996.tb03913.x
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  • 10
    Electronic Resource
    Electronic Resource
    Evidence for cell-mediated immune mechanisms in the pathology of pemphigus (1993)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 128 (1993), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Pemphigus is an autoimmune blistering disease in which autoantibodies have clearly been shown to be pathogenic. Because autoantibodies are also found in uninvofved skin, further mechanisms may be important in the development of pemphigus lesions. In addition to granulocytes, mononuclear cells are commonly found in pemphigus lesions. To elucidate the role of mononuclear cells in the pathology of this disease, we determined the levels of soluble interleukin-2 receptor in blister fluid and serum samples from pemphigus patients prior to treatment. The interleukin-2 receptor (IL-2R) is expressed on activated mononuclear cells. Depending on its rate of synthesis, a portion is released from the cell surface in a soluble form (sIL-2R), In blister fluid, sIL-2R levels were 2186±288 U/ml (±SD), which was significantly higher than levels in concurrently obtained serum samples (1299±165 U/ml: P〈0.001). In suction blisters in volunteers, and in patients with second-degree burns or friction-induced bullae, sIL-2R levels were normal in both blister fluid and serum. In pemphigus patients, sIL,-2R serum levels continuously declined during systemic therapy, correlating with disease activity, Immunohistological studies demonstrated a marked increase in IL-2R+ cells in both the epidermis and dermis of lesional skin compared with perilesional skin. In the dermis, CD3+ T cells predominated, whereas monocytes/macrophages were most frequent in the epidermis. In pemphigus vulgaris, monocytes/macrophages were restricted to the basal keratinocytes. whereas in pemphigus foliaceus, they were found throughout the lesional epidermis. Our data indicate that activated mononuclear cells are present in lesional skin of pemphigus patients, and may contribute to the pathology of this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1993.tb00259.x
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