Figurate Erythema: An Unusual Presentation of the Nonbullous Phase of Bullous Pemphigoid

2012 ◽  
Vol 16 (1) ◽  
pp. 61-63 ◽  
Author(s):  
Farheen Mussani ◽  
Sanjay Siddha ◽  
Cheryl F. Rosen
Keyword(s):  
Bullous Pemphigoid ◽  
Autoimmune Disorder ◽  
Unusual Presentation ◽  
Bullous Eruption ◽  
Papular Eruption

Background and Objective: Bullous pemphigoid, an autoimmune disorder, most commonly presents as a bullous eruption in patients over the age of 60. There may be a prodromal urticarial or papular eruption that evolves to bullae. Conclusion: We report a 46-year-old woman with a figurate erythema presentation of bullous pemphigoid.

Bullous pemphigoid autoantibodies, HIV-1 infection and pruritic papular eruption

AIDS ◽  
1991 ◽  
Vol 5 (4) ◽  
pp. 451-454 ◽  
Author(s):  
Sabine Kinloch-de Loës ◽  
Liliane Didierjean ◽  
Letizia Rieckhoff-Cantoni ◽  
Karine Imhof ◽  
Luc Perrin ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Papular Eruption ◽  
Hiv 1

scholarly journals Sindrom Hiper IgE dengan Manifestasi Bullous pemphigoid dan Striktur Esofagus

2016 ◽  
Vol 3 (2) ◽  
pp. 100
Author(s):  
Laras Budiyani ◽  
Adeputri Tanesha Idayu ◽  
Ika Prasetya Wijaya ◽  
Alvina Widhani
Keyword(s):  
Bullous Pemphigoid ◽  
Skin Disorder ◽  
Multidisciplinary Approach ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Hyper Ige Syndrome ◽  
Recessive Type ◽  
Complex Deficiency ◽  
Mucous Membranes ◽  
The Face

Sindrom hiper IgE (HIES) adalah suatu defisiensi kompleks imun primer yang jarang terjadi, memiliki manifestasi klinis yang beragam. Mutasi STAT 3 merupakan dasar dari sindrom hiper IgE klasik yang berhubungan dengan abnormalitas wajah, gigi, rangka, dan jaringan ikat yang tidak terlihat pada tipe resesif. Abses kulit berulang adalah gambaran utama pada HIES. Pemphigoid bullosa adalah kelainan autoimun yang memiliki karakteristik manifestasi bula pada kulit terkait membrane mukosa. Adanya gangguan autoimun yang terjadi secara bersamaan merupakan kelainan yang jarang. Striktur esofagus adalah salah satu manifestasi membran mukosa yang jarang terjadi pada kelainan kulit bulosa ini. Oleh sebab itu, manajemen pada kasus ini memerlukan pendekatan multidisiplin termasuk manajemen nutrisi dan infeksi.Kata kunci: pemphigoid bullosa, sindrom hiper IgE, striktur esofagus Hyper-IgE Syndrome with Bullous Pemphigoid and Esophageal StrictureHyper IgE syndrome (HIEs) is a rare primary immune complex deficiency that has diverse clinical manifestations. STAT 3mutation is the basis of hyper IgE classic syndrome which was associated with abnormalities of the face, teeth, skeletonand connective tissue that is not visible on recessive type. Recurrent skin abscesses are the main chracteristic of HIEs. Bullosa pemphigoid is an autoimmune disorder that is characterized by bullae manifestation on the skin associated with mucous membranes. An autoimmune disorder that occurs simultaneously is generally rare. Esophagus stricture is one of mucous membrane manifestations that is rarely occurred in this bullous skin disorder. Therefore, the management of these casesrequire a multidisciplinary approach including nutrition and infection management.Keywords: esophagus stricture, hyper IgE syndrome, pemphigoid bullosa

Bullous Pemphigoid: A 10-Year Study of Discordant Results on Direct Immunofluorescence

2018 ◽  
Vol 22 (5) ◽  
pp. 472-475 ◽  
Author(s):  
Jessica G. Fudge ◽  
Richard I. Crawford
Keyword(s):  
Bullous Pemphigoid ◽  
False Negative ◽  
Retrospective Chart Review ◽  
Autoimmune Disorder ◽  
Direct Immunofluorescence ◽  
Linear Pattern ◽  
Specimen Quality ◽  
Circulating Autoantibodies ◽  
Perilesional Skin ◽  
Specimen Sampling

Background: Bullous pemphigoid (BP) is the most common subepidermal autoimmune disorder characterized by tense bullae. It is associated with circulating autoantibodies against BP antigen-1 and BP antigen-2. Diagnosis is based upon clinical, histopathologic, and immunopathologic examination. Direct immunofluorescence (DIF) of perilesional skin highlights C3 with or without IgG in a linear pattern along the basement membrane. Objectives: We hypothesized that repeat biopsies may be required for a definitive DIF diagnosis of BP, as initial DIF evaluation may result in a false-negative result. Methods: A retrospective chart review was conducted on 1143 specimens collected for evaluation for BP. Cases from 2 Vancouver Coastal Health Authority laboratories from 2006 to 2016 were reviewed. Results were interpreted as positive, negative, or indeterminate based on pathologic description and specimen quality. Results: After meeting the inclusion criteria, 739 specimens were further evaluated. There were 289 cases of BP in the 10-year period. Five patients (1.73%; 95% confidence interval [CI], 1.50-1.96) required a second biopsy to support a BP diagnosis, and within this group, 1.04% of the 289 (95% CI, 0.811-1.27) were true successive negative-to-positive DIF results. Conclusions: DIF is the most reliable test used to diagnose BP; however, a small percentage of patients will initially have a negative result. False-negative or indeterminate results may be due to specimen sampling from lesional skin or due to a subthreshold quantity of immune complexes in the skin. Repeat biopsy is warranted despite an initial negative DIF if BP is clinically suspected.

scholarly journals Carbamazepine-induced bullous eruption or bullous pemphigoid?

1984 ◽  
Vol 60 (702) ◽  
pp. 307-307 ◽  
Author(s):  
A. Ingber ◽  
M. H. Grunwald ◽  
E. J. Feuerman
Keyword(s):  
Bullous Pemphigoid ◽  
Bullous Eruption

scholarly journals A Case Report on Idiopathic Severe Bullous Pemphigoid

2020 ◽  
Vol 10 (4-s) ◽  
pp. 223-225
Author(s):  
Fizza Khan Mohammadi ◽  
Sultana Zoha ◽  
Mohammed Baleeqhuddin ◽  
Salman Mir Ali ◽  
Abdullah Md. Faizan
Keyword(s):  
Bullous Pemphigoid ◽  
Autoimmune Disorder ◽  
The Body ◽  
Topical Steroids ◽  
Adequate Treatment ◽  
Systemic Corticosteroids ◽  
Bullous Lesion ◽  
Clinical Presentations ◽  
Chief Complaints ◽  
Laboratory Investigations

Bullous Pemphigoid (BP) is an autoimmune disorder which exploits the immune system and affecting sub-epidermal region of skin causing mild itching to infection and blistering of sub-epidermal region. Clinical presentations are pruritis, urticarial, papular lesions. These later evolve to bullae in weeks to months and are typically present in the axillae, on the flexor surface of the forearms, medial thighs, trunk, and abdomen. The treatment for bullous pemphigoid is systemic corticosteroids, topical steroids in combination with nicotinamide plus tetracycline, minocycline or doxycycline have shown success in multiple cases. Immunosuppressive therapy is used when steroids do not control the disease or if patients have contraindications for systemic corticosteroid treatments. Patient was brought to emergency department with chief complaints itching, redness, bullous lesion all over the body for 2 months.Oral complaints including solitary erosion seen on right side of buccal mucosa, pharyngeal erythema. Laboratory investigations were carried out. Patient was diagnosed based on physical examination. Patient was provided with adequate treatment and counseling. Keywords: Bullous Pemphigoid, corticosteroid, Autoimmune disorder, sub-epidermal region.

Bullous Pemphigoid in Infancy: A Case Report

PEDIATRICS ◽  
1977 ◽  
Vol 59 (6) ◽  
pp. 942-945
Author(s):  
William M. Gould ◽  
David A. Zlotnick
Keyword(s):  
Case Report ◽  
Bullous Pemphigoid ◽  
Oral Lesions ◽  
Bullous Eruption ◽  
Hands And Feet

Bullous pemphigoid occurs rarely in children. Bean and Jordan1 found only eight cases reported as of 1974 and the youngest of these patients was 2 years of age. Presented here is the case of a 3½-month-old infant who developed this disease. CASE REPORT A 3½-month-old boy developed a progressively spreading bullous eruption (Fig. 1 and 2). Within a week the hands and feet were covered with large, tense bullae lying on a base of erythema, and scattered vesicles appeared on the back of the neck, the trunk, and the rims of the ears. There were no oral lesions. The child was fussy and irritable but had no fever.

scholarly journals Gambaran Dermal-Epidermal Junction Pada Bullous Pemphigoid Dan Epidermolysis Bullosa Acquisita

Medicinus ◽  
2018 ◽  
Vol 6 (3) ◽  
Author(s):  
Caroline Budiman ◽  
Mutia Devi ◽  
HM Athuf Thaha
Keyword(s):  
Autoimmune Disease ◽  
Basement Membrane ◽  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Intermediate Filament ◽  
Autoimmune Disorder ◽  
Epidermolysis Bullosa Acquisita ◽  
Bullous Pemphigoid Antigen ◽  
Xvii Collagen

Epidermis and dermis of the skin attach each other because of the role of a structure which is called dermal-epidermal junction. The dermal-epidermal junction can be divided into three distinct zones named hemidesmosome-keratin intermediate filament complex, basement membrane, and anchoring fibril. Each part of the distinct zone has significant components for the integrity of the skin. Bullous pemphigoid is an autoimmune disorder caused by autoantibody against a 180-kDa molecule now called Bullous Pemphigoid Antigen 2 (BPAG2) or type XVII collagen within basement membrane. To date, the patophysiology and the components of dermal-epidermal junction that induce the blister formation remain unclear. Epidermolysis bullosa acquisita is an autoimmune disease with sub-epidermal blistering associated with autoimmunity to the collagen within anchoring fibrils. The pathophysiology and etiology—again—remain unknown.

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