Presentation
In this study of skin biopsy specimens from 14 patients we present evidence that complement and immunoglobulins were present by direct immunofluorescence in the epidermal intercellular spaces in all patients. [ncbi.nlm.nih.gov]
Entire Body System
-
Fever
Fever, malaise, headache, muscle pain, chills, anorexia, and weight loss occur. The intensity of the symptoms is directly proportional to the spread of the lesions. [kundoc.com]
-
Chills
Fever, malaise, headache, muscle pain, chills, anorexia, and weight loss occur. The intensity of the symptoms is directly proportional to the spread of the lesions. [kundoc.com]
-
Constitutional Symptom
Secondary bacterial infection is common which may lead to constitutional symptoms, malodorous smell of the mucous membrane, and skin lesions. Differential Diagnosis of Pemphigus ‘Pemphigus syphiliticus‘: occurs in some babies. [drmhijazy.com]
-
Anorexia
Fever, malaise, headache, muscle pain, chills, anorexia, and weight loss occur. The intensity of the symptoms is directly proportional to the spread of the lesions. [kundoc.com]
Musculoskeletal
-
Myalgia
(“/a) Gastralgia Arthalgia Myalgia Insomnia Psychic changes 38 14 10 16 1 (57.5) (21.2) (15.1) (24.2) (1.5) Cushingoid features (hirsutism, acne, straia, moon face) 41 (62.1) Prednisolone (47 patients) No. (%) 17 6 4 6 (36.1) (12.7) (8.5) (12.7) Triamcinolone [kundoc.com]
Skin
-
Blister
Abstract Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. [ncbi.nlm.nih.gov]
(Simuliidae), suggesting an environmental link Management Steroids fogo selvagem (fo'go, goo sel-va'zhem) [Portuguese, literally “wild fire”], FS An autoimmune disease that causes blistering of the skin of the head, neck or trunk, esp. when the skin [medical-dictionary.thefreedictionary.com]
-
Skin Disease
Abstract Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. [ncbi.nlm.nih.gov]
• Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. [jamanetwork.com]
Abstract Pemphigus foliaceus (PF) is a blistering autoimmune skin disease rare in most of the world but endemic in certain regions of Brazil. [medworm.com]
Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. [pubfacts.com]
-
Nikolsky's Sign
The intra-epithelial bulla is acantholytic and Nikolsky’s sign is present, thus differentiating this entity from dermatitis herpetiformis. [kundoc.com]
They will look for an indicator of the condition called Nikolsky’s sign. A positive Nikolsky’s sign is when your skin shears off easily when the surface is wiped sideways with a cotton swab or a finger. [healthline.com]
-
Plantar Hyperkeratosis
The scalp is thickly crusted and palmo-plantar hyperkeratosis occurs. Hyperpigmented macules develop after some years and are a favorable prognostic sign,’ heralding the next stage. [kundoc.com]
-
Follicular Plugging
In older lesions, the histopathology depends on the clinical aspect and may show acanthosis, papillomatosis, hyperkeratosis, follicular plugging, and parakeratosis, with only occasional evidence of acantholysis at some places in the upper epidermis. [kundoc.com]
Neurologic
-
Dysesthesia
It often causes a burning dysesthesia, from which its popular name is derived. The disease is typically found in Brazil or Colombia and has been associated with chronic or recurrent exposure to black flies. Synonym: endemic pemphigus foliaceus. [medical-dictionary.thefreedictionary.com]
-
Meningism
They were classified as pulmonary (tuberculosis, pneumonia and bronchial pneumonia), cardiac (endocarditis, pericarditis), hepatic (fulminant hepatitis, cirrhosis), renal (diffuse acute glomerulonephritis, subacute glomerulonephritis), nervous(meningitis [kundoc.com]
Workup
Microbiology
-
Complement Fixing Antibody
Complement fixing antibodies were found in four patients. However, the distribution of IgG subclasses was incompatible with their known characteristics in terms of complement activation. [ncbi.nlm.nih.gov]
-
Trypanosoma Cruzi
Di Zenzo G, Zambruno G, Borradori L Profile of Trypanosoma cruzi reactivity in a population at high risk for endemic pemphigus foliaceus (Fogo selvagem). [medicbind.com]
HLA Type
-
HLA-DR2
Typing for alleles of the HLA- DR2 group. Hum Immwol 1991: 31: 139-44. Gao X, Moraes R, Miller S, Stastny P. DNA typing for class I1 HLA antigens with allele-specific or groupspecific amplification. V. Typing for subsets of HLA-DR1 and DR'Br'. [docslide.com.br]
Treatment
Autoantibodies deposited in the intercellular spaces in titers ranging from 1:10 to more than 1:1280, and the titers drastically decreased during treatment. [ncbi.nlm.nih.gov]
الصفحة 7 - A et al: A comparison of the efficacy and safety of adapalene gel 0.1% and tretinoin gel 0.025% in the treatment of acne vulgaris: A multicenter trial. [books.google.com]
If you believe that you are suffering from any of the symptoms of Brazilian Pemphigus Foliaceus it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition [medigest.uk]
Prognosis
In general, PF and PE have a better prognosis than pemphigus vulgaris. However, prior to the advent of glucocorticoid therapy, the mortality rate was as high as 60%. [dermnet.com]
[…] malignancy, and our present observation draws ... more Abstract Fogo Selvagem (Wildfire) is a cutaneous disease characterized by a specific epidemiology, generalized distribution of bullae followed by an exfoliative erythroderma, and frequently, a grave prognosis [scinapse.io]
With adequate treatment, the prognosis of fogo selvagem is good with no long-term sequelae. See smartphone apps to check your skin. [Sponsored content] Related information References Abréu-Vélez A, Reason M, Howard S & Roselino A. [dermnetnz.org]
Key words: pemphigus; vulgaris; foliaceus; epidemiology; mortality; prognosis. Accepted May 22, 2017; Epub ahead of print May 24, 2017 Acta Derm Venereol 2017; 97: xx–xx. [medicaljournals.se]
Brazil Fogo Selvagem (Wildfire) is a cutaneous disease characterized by a specific epidemiology, generalized distribution of bullae followed by an exfoliative erythroderma, and frequently, a grave prognosis, if untreated. [kundoc.com]
Etiology
The possible relationship of an elevated level of IgE with respect to other aberrations of immunologic responsiveness and the suggested infectious etiology of BPF are considered. [ncbi.nlm.nih.gov]
The etiology i... While environmental factors are critical for the development of autoimmunity, the inciting agents that trigger autoantibody formation remain elusive. [medworm.com]
Inflammatory cytokines and apoptosis are also involved. [16] Its etiology is unknown, but blister formation appears to be mainly IgG4 mediated. [emedicine.medscape.com]
Additionally, we are investigating the etiology of Brazilian PF (Fogo Selvagem) as a joint project with Brazilian investigators. The research projects of this laboratory are supported by NIH. [med.unc.edu]
Epidemiology
Epidemiologic data strongly support the notion that this disease is caused by exposure to an environmental agent or agents. [ncbi.nlm.nih.gov]
[Pg. wild fire] A form of pemphigus foliaceus, an autoimmune blistering disorder, which is endemic in rural Colombia and Brazil Epidemiology Fogo selvagem affects children and young adults and is distributed along rivers within the flying range of blackflies [medical-dictionary.thefreedictionary.com]
Pathophysiology
Patient case no. 10989 Date added 03 June 2011 Patient details Age Adult Location Brazil Localisation Trunk / entire trunk Description Pemghigus foliaceus in a patient from Brazil, where fogo selvagem is prevalent Primary Lesions Bulla / blister Crust Pathophysiology [dermquest.com]
However, because the desmoglein ELISA measures both pathogenic as well as nonpathogenic antibodies (see “Pathophysiology” below), positive desmoglein ELISA index values may still be observed in patients in remission. [dermatologyadvisor.com]
[…] death occurring in PF.65,76 Infection is often the cause of death, and therapy is frequently a contributing factor because it causes the immunosuppression necessary to treat active disease.77 The effective management of PF requires a knowledge of the pathophysiology [mdedge.com]
The pivotal contributions of Jean-Claude Bystryn have been summarized. [9] Pathophysiology Superficial blisters in pemphigus foliaceus are induced by immunoglobulin G (IgG) (mainly IgG4 subclass) autoantibodies directed against a cell adhesion molecule [emedicine.medscape.com]
Prevention
Treatment is aimed at reducing pain and symptoms and preventing complications like infection. It involves one or more drugs and other methods. [healthline.com]
Consequently, elevations of circulating plasma FFA concentration can be prevented if large amounts of insulin can be secreted. [scienceopen.com]
Switching to another medicine can also help prevent possible side effects that can occur when someone takes a corticosteroid for a long time. [aad.org]
IVIG is normally given with other therapy, such as steroids and immunosuppressives, to prevent rebound of disease as the therapy is tapered. [rarediseases.org]
The adaptive immune system, also known as the acquired immune system or, more rarely, as the specific immune system, is composed of highly specialized, systemic cells and processes that eliminate or prevent pathogen growth. [theuniversalmatrix.com]