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2.
Ann Dermatol Venereol ; 147(6-7): 439-445, 2020.
Article in French | MEDLINE | ID: mdl-32245657

ABSTRACT

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare auto-immune blistering disease. We report a case of Brunsting-Perry pemphigoid diagnosed by immunoelectron microscopy (IEM). PATIENTS AND METHODS: A 46-year-old man presented very pruriginous vesicles on the face and neck present for 6 years and which were difficult to diagnose and treat. The appearance of atrophic scars and milium cycts evoked EBA, which was confirmed at IEM. Due to limited involvement of the face and the neck, we conclude on EBA of the Brunsting-Perry pemphigoid variant. Treatment with dapsone produced a favorable outcome. DISCUSSION: Diagnosis of EBA is often difficult. In a case review, Asfour et al. collated 60 cases of Brunsting-Perry pemphigoid. These patients had either anti-collagen VII or anti-BP180 and anti-BP230 antibodies. IEM showed cleavage either under the lamina densa or within the lamina lucida, suggesting that Brunsting-Perry pemphigoid is a subtype of EBA or bullous pemphigoid (BP), depending on the paraclinical elements, and localized to the head and neck. The majority of EBA-like cases required systemic therapy, whereas in the presence of BP antibodies, topical corticosteroids were effective. CONCLUSION: We report a case of EBA of the Brunsting-Perry pemphigoid type, diagnosed by IEM after 6 years of progression. We highlight the diagnostic and nosological difficulties of Brunsting-Perry pemphigoid. Classification of this dermatosis as a subtype of EBA or BP may enable effective adaptation of therapeutic management, which has not as yet been coded.


Subject(s)
Epidermolysis Bullosa Acquisita , Pemphigoid, Bullous , Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/diagnosis , Epidermolysis Bullosa Acquisita/drug therapy , Humans , Male , Middle Aged , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/complications , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy
3.
An Bras Dermatol ; 94(2): 133-146, 2019.
Article in English | MEDLINE | ID: mdl-31090818

ABSTRACT

Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.


Subject(s)
Pemphigoid, Bullous/diagnosis , Aged , Autoimmunity/physiology , Diagnosis, Differential , Fluorescent Antibody Technique/methods , Humans , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/etiology , Steroids/therapeutic use
4.
An. bras. dermatol ; 94(2): 133-146, Mar.-Apr. 2019. tab, graf
Article in English | LILACS | ID: biblio-1001150

ABSTRACT

Abstract: Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.


Subject(s)
Humans , Aged , Pemphigoid, Bullous/diagnosis , Steroids/therapeutic use , Autoimmunity/physiology , Fluorescent Antibody Technique/methods , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/drug therapy , Diagnosis, Differential
9.
Dtsch Med Wochenschr ; 131(8): 389-92, 2006 Feb 24.
Article in German | MEDLINE | ID: mdl-16479471

ABSTRACT

Bullous pemphigoid, the most frequent bullous autoimmune dermatosis of the adult, typically presents as disseminated tense blisters on normal or erythematous skin. The diagnosis can be confirmed by direct and indirect immunofluorescence, the detection of circulating autoantibodies against the basement membrane proteins collagen XVII/BP180 and BP230, and histopathology. Autoantibody reactivity against collagen XVII can be measured by ELISA and correlates with disease activity. The ELISA therefore provides a useful tool for monitoring disease activity. Treatment of bullous pemphigoid usually consists of topical and / or systemic steroids in combination with immunosuppressive agents. The intensity of skin involvement and the concurrent diseases and medications of the patient must be considered when selecting a certain treatment. Interdisciplinary cooperation between general practitioners, internists and other specialists facilitates the optimal adaptation of the medication and the early discovery of potential side effects.


Subject(s)
Pemphigoid, Bullous , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/adverse effects , Adrenal Cortex Hormones/therapeutic use , Adult , Aged , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents/adverse effects , Anti-Inflammatory Agents/therapeutic use , Autoantibodies/analysis , Azathioprine/administration & dosage , Azathioprine/adverse effects , Azathioprine/therapeutic use , Biopsy , Blotting, Western , Child , Clobetasol/administration & dosage , Clobetasol/adverse effects , Clobetasol/analogs & derivatives , Clobetasol/therapeutic use , Cyclophosphamide/administration & dosage , Cyclophosphamide/adverse effects , Cyclophosphamide/therapeutic use , Dapsone/administration & dosage , Dapsone/adverse effects , Dapsone/therapeutic use , Dermatologic Agents/administration & dosage , Dermatologic Agents/adverse effects , Dermatologic Agents/therapeutic use , Diagnosis, Differential , Drug Therapy, Combination , Enzyme-Linked Immunosorbent Assay , Female , Fluorescent Antibody Technique, Direct , Fluorescent Antibody Technique, Indirect , Humans , Immunoglobulins, Intravenous/administration & dosage , Immunoglobulins, Intravenous/adverse effects , Immunoglobulins, Intravenous/therapeutic use , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Male , Mycophenolic Acid/administration & dosage , Mycophenolic Acid/adverse effects , Mycophenolic Acid/analogs & derivatives , Mycophenolic Acid/therapeutic use , Niacinamide/administration & dosage , Niacinamide/adverse effects , Niacinamide/therapeutic use , Pemphigoid, Bullous/classification
10.
Exp Dermatol ; 13(2): 125-8, 2004 Feb.
Article in English | MEDLINE | ID: mdl-15009107

ABSTRACT

Mutations in the gene COL17A1 cause non-Herlitz junctional epidermolysis bullosa. Here, we describe a patient who, despite two heterozygous mutations in COL17A1, has an extremely mild form of the disease missing most of the characteristic clinical features. DNA analysis revealed a frame-shift mutation 3432delT and a nonsense mutation 2356C-->T (Q751X). cDNA analysis showed that the deleterious effect of the latter mutation was skirted by deleting the premature termination codon containing exon 30. In this way, the reading frame was restored, resulting in a 36 nucleotides shorter mRNA transcript. Immunoblot analysis showed expression of the 180-kDa bullous pemphigoid antigen (BP180) with a slightly higher SDS-PAGE mobility, in line with the deletion of 12 amino acids from the COL15 domain. Immunofluorescence of skin sections showed diminished, but correctly localised expression of BP180, and this, in concert with the mild clinical phenotype, suggests that this COL15 mutated BP180 is still partly functional.


Subject(s)
Autoantigens/genetics , Collagen/genetics , Epidermolysis Bullosa/genetics , Frameshift Mutation/genetics , Pemphigoid, Bullous/genetics , Base Sequence , Carrier Proteins , Cytoskeletal Proteins , Dystonin , Epidermolysis Bullosa/classification , Exons , Humans , Nerve Tissue Proteins , Non-Fibrillar Collagens , Pemphigoid, Bullous/classification , RNA, Messenger/genetics , Sequence Deletion , Skin/pathology , Transcription, Genetic , Collagen Type XVII
12.
N Engl J Med ; 346(5): 321-7, 2002 Jan 31.
Article in English | MEDLINE | ID: mdl-11821508

ABSTRACT

BACKGROUND: Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent topical corticosteroids could decrease mortality while controlling disease. METHODS: A total of 341 patients with bullous pemphigoid were enrolled in a randomized, multicenter trial and stratified according to the severity of their disease (moderate or extensive). Patients were randomly assigned to receive either topical clobetasol propionate cream (40 g per day) or oral prednisone (0.5 mg per kilogram of body weight per day for those with moderate disease and 1 mg per kilogram per day for those with extensive disease). The primary end point was overall survival. RESULTS: Among the 188 patients with extensive bullous pemphigoid, topical corticosteroids were superior to oral prednisone (P=0.02). The one-year survival rate was 76 percent in the topical-corticosteroid group and 58 percent in the oral-prednisone group. Disease was controlled at three weeks in 92 of the 93 patients in the topical-corticosteroid group (99 percent) and 86 of the 95 patients in the oral-prednisone group (91 percent, P=0.02). Severe complications occurred in 27 of the 93 patients in the topical-corticosteroid group (29 percent) and in 51 of the 95 patients in the oral-prednisone group (54 percent, P=0.006). Among the 153 patients with moderate bullous pemphigoid, there were no significant differences between the topical-corticosteroid group and the oral-prednisone group in terms of overall survival, the rate of control at three weeks, or the incidence of severe complications. CONCLUSIONS: Topical corticosteroid therapy is effective for both moderate and severe bullous pemphigoid and is superior to oral corticosteroid therapy for extensive disease.


Subject(s)
Clobetasol/administration & dosage , Glucocorticoids/administration & dosage , Pemphigoid, Bullous/drug therapy , Prednisone/administration & dosage , Administration, Oral , Administration, Topical , Aged , Aged, 80 and over , Clobetasol/adverse effects , Glucocorticoids/adverse effects , Hospitalization , Humans , Length of Stay , Ointments , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/mortality , Prednisone/adverse effects , Proportional Hazards Models , Recurrence , Survival Rate
13.
Harefuah ; 140(11): 1049-53, 1117, 2001 Nov.
Article in Hebrew | MEDLINE | ID: mdl-11759380

ABSTRACT

Pemphigus is an autoimmune blistering disease of skin and mucous membranes. The classic types of pemphigus are pemphigus vulgaris and pemphigus foliaceus. In this review we summarize recent advancement in the etiology and the pathogenesis of pemphigus. Desmogleins--transmembrane glycoproteins involved in intracellular adhesion--were recognized as targets of pemphigus antibodies. It was found that the distribution and the expression of desmogleins can explain the difference in the localization of lesions in pemphigus vulgaris and pemphigus foliaceus. Pemphigus develops in a two-step process. The first step leads to the presence of a low titer of autoantibody, the second step results in a significant increase in the antibody titer which causes the clinical stage of the disease. Selective presentation of self peptides can explain the Major Histocompatibility Complex (MHC)--linked susceptibility to autoimmune diseases including pemphigus and rheumatoid arthritis. Peptides selective for the disease-associated molecules can be identified and used to search for microbiologic factors that can take part in the pathogenesis of pemphigus.


Subject(s)
Pemphigus/physiopathology , Autoimmune Diseases/classification , Humans , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/physiopathology , Pemphigus/classification , Pemphigus/immunology
14.
Tunis Med ; 78(10): 584-8, 2000 Oct.
Article in French | MEDLINE | ID: mdl-11190743

ABSTRACT

We report forty-seven cases of bullous pemphigoid recorded in the dermatology department of Charles Nicolle hospital in Tunis during 16 years. In Tunisia, bullous pemphigoid is at the second rank of acquired autoimmune bullous skin diseases, after pemphigus. The profile of bullous pemphigoid in our series differ from that reported in the literature by the more young age (67.2 years) and the male predilection but don't present any clinical an epidemiological particularity. Three atypicals forms were observed: a vesicular form, a localized form and a infantile form. Systemic corticosteroids were choice treatment for our patients.


Subject(s)
Pemphigoid, Bullous/epidemiology , Pemphigoid, Bullous/pathology , Adolescent , Adult , Age Distribution , Aged , Aged, 80 and over , Anti-Inflammatory Agents/therapeutic use , Child , Female , Humans , Incidence , Male , Middle Aged , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/drug therapy , Retrospective Studies , Sex Distribution , Steroids , Tunisia/epidemiology
16.
Rev. Fac. Odontol. Univ. Chile ; 17(1): 32-8, ene.-jun. 1999. tab
Article in Spanish | LILACS | ID: lil-260160

ABSTRACT

El pengigoide (PMB o penfigoide de las membranas mucosas PMM) es una enfermedad de las mucosas, piel o de piel y mucosas, de naturaleza autoinmune que se caracteriza por la formación de ampollas. En esta ocasión se presenta una revisión de 55 casos, realizada en el IREPO (Instituto de Referencia de Patología Oral) y en el Servicio de diagnósticos de la Escuela Dental, entre los años 1978 y 1998, en todos los pacientes el diagnóstico fue confirmado con biopsia


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Gingival Diseases/diagnosis , Palate, Soft , Pemphigoid, Bullous/diagnosis , Adrenal Cortex Hormones/therapeutic use , Betamethasone/therapeutic use , Biopsy , Clinical Evolution , Hydroxychloroquine/therapeutic use , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/etiology
17.
Dermatol. venez ; 37(2): 35-8, 1999. tab, graf
Article in Spanish | LILACS | ID: lil-263246

ABSTRACT

Se revisa la correlación histopatológica e inmunofluorescencia directa (IFD) de piel en 127 casos con los diagnósticos de pénfigo, penfigoide ampollar y dermatitis herpetiforme en el servicio de dermatología del Hospital Universitario de Caracas, durante un período de 15 años. El 88,46 por ciento de pacientes con pénfigo tuvo IFD positiva; los casos de penfigoide ampollar y dermatitis herpetiforme el 77,7 por ciento y 81,25 por ciento. La correlación histopatológica e IFD fue de 85,48 por ciento, 67,85 por ciento y 86,48 por ciento para pénfigo, penfigoide ampollar y dermatitis herpetiforme respectivamente


Subject(s)
Humans , Male , Female , Dermatitis , Fluorescent Antibody Technique, Direct , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/complications , Pemphigus
18.
Br J Dermatol ; 137(4): 599-604, 1997 Oct.
Article in English | MEDLINE | ID: mdl-9390339

ABSTRACT

A 64-year-old man presented with a bullous eruption which clinically and histopathologically resembled dermatitis herpetiformis. However, direct immunofluorescence analysis showed IgG deposits at the basement membrane zone, indicating a relationship with bullous pemphigoid or epidermolysis bullosa acquisita. Indirect immunofluorescence studies on salt-split skin showed binding of IgG mainly on the dermal side of the blister. Immunoblot analysis revealed a novel 200 kDa dermal antigen that could be associated with a major pathogen in this blistering disease. The histopathological similarity to dermatitis herpetiformis and the immunofluorescence findings indicating bullous pemphigoid or epidermolysis bullosa acquisita seem typical of a distinct subepidermal blistering disease characterized by this 200 kDa antigen. However, the pathogenetic role of autoantibodies against this antigen should be further elucidated before confirming whether this case represents a novel subepidermal blistering disease or a special variant of bullous pemphigoid.


Subject(s)
Dermatitis Herpetiformis/pathology , Skin Diseases, Vesiculobullous/pathology , Autoantigens/analysis , Basement Membrane/immunology , Diagnosis, Differential , Fluorescent Antibody Technique , Humans , Immunoglobulin G/analysis , Male , Middle Aged , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/immunology , Skin Diseases, Vesiculobullous/immunology
19.
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