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]
We report a patient of pemphigus foliaceus who was presented with verrucous surfaced plaque, which clinically mimicked pemphigus vegetans. [pesquisa.bvsalud.org]
Page Excerpt: Pemphigus foliaceus Presentation stereotypical Age and sex Adults usually and of both sexes Distribution of lesions Localized to photodistribution in pemphigus erythematosus; widespread in pemphigus foliaceus Individual lesions Macules, [derm101.com]
Entire Body System
- Sepsis
+ sepsis + multiple organ failure 1 25.0 PF + sepsis + pneumonia 1 25.0 PV = pemphigus vulgar; PF = pemphigus foliaceus. [scielo.br]
Untreated, most patients die within 5 years from secondary sepsis, electrolyte imbalance and/or emaciation. [apps.who.int]
Complications Possible complications of pemphigus include: Infection of your skin Infection that spreads to your bloodstream (sepsis) Malnutrition, because painful mouth sores make it difficult to eat Medication side effects, such as high blood pressure [mayoclinic.org]
If the bacteria get into your bloodstream, they can cause a life-threatening infection called sepsis. See your doctor if you have blisters on your skin, especially if they break open. Your doctor will ask about your symptoms and examine your skin. [healthline.com]
Sepsis with secondary infection. External therapy This section has been translated automatically. Consistent textile sun protection as well as the use of physical or chemical (e.g. [altmeyers.org]
- Fever
If you’ve been diagnosed with pemphigus already, you should contact your doctor if you develop: new blisters or sores a rapid spread in the number of sores fever redness or swelling chills weakness or achy muscles or joints Some people get better without [healthline.com]
Signs of a skin infection include: pus at a skin wound intense pain at the site of a blister red streaks out of a wound or blister fever exhaustion nausea Share on Pinterest Sun exposure and sunburn may trigger PF. [medicalnewstoday.com]
Azathioprine-induced drug fever has been observed in a pemphigus patient. [ijdvl.com]
- Chills
If you’ve been diagnosed with pemphigus already, you should contact your doctor if you develop: new blisters or sores a rapid spread in the number of sores fever redness or swelling chills weakness or achy muscles or joints Some people get better without [healthline.com]
The lesions progressed slowly, eventually covering her entire skin surface, accompanied by general malaise, chills, and edema of the lower extremities. [hindawi.com]
- Candidiasis
Significant secondary bacterial infection occurred in all cases at some stage of the disease while oral candidiasis occurred in 15 cases. Eczema herpeticum was seen in 4 patients, while 2 had extensive tinea corporis. [ijdvl.com]
[…] corticosteroids was associated with recurrence of disease 4. 5. in a 5-year-follow-up, all cases of pemphigus (vulgaris and foliaceus) were assessed on clinical development of prednisone side effects: infections (impetigo, septicemia, kidney infection, candidiasis [scielo.br]
When the disease is limited to the oral cavity, it is often misdiagnosed as an infection, including candidiasis or herpes. Half of PV patients will also present with cutaneous erosions. [clevelandclinicmeded.com]
Differential Diagnosis Candidiasis T. Cruris Pemphigus vegetans of the crural areas. Darrier‘s disease. Treatment Antibiotics: Hailey -Hailey disease characteristically responds to antibiotics locally and systemically. [drmhijazy.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]
Jaw & Teeth
- Oral Ulcers
Oral ulcers 5. Arthritis 6. Serositis 7. Renal disorder 8. الصفحة 637 - In vivo epiluminescence microscopy of pigmented skin lesions. I. Pattern analysis of pigmented skin lesions. J Am Acad Dermatol 17:571-583 6. [books.google.com]
Oral ulcers 5. Arthritis 6. Serositis 7. Renal disorder 8. Pagina 637 - In vivo epiluminescence microscopy of pigmented skin lesions. I. Pattern analysis of pigmented skin lesions. J Am Acad Dermatol 17:571-583 6. [books.google.ro]
Clinical Indications and Features of Pemphigus Subtypes Subtype Indications and Features Pemphigus vulgarisa Fragile, flaccid bullae that evolve into erosions, crusting, Nikolsky sign; oral ulcers are commonly an initial presenting symptom Three forms [arupconsult.com]
Clinical Features Pemphigus Vulgaris The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. [pemphigus.org]
Musculoskeletal
- Osteoporosis
They can include: Infections Rashes High blood pressure Low bone density (osteoporosis) At least 75% of people with pemphigus will have a complete remission, or no evidence of the disease, after 10 years of treatment. [webmd.com]
These drugs have many side effects, including: an increased likelihood of becoming infected osteoporosis cataracts glaucoma increased blood sugar diabetes a loss of muscle mass stomach ulcers water retention You may need to take supplements such as calcium [healthline.com]
They are treated mainly with systemic corticosteroids, which may provoke osteoporosis; atherosclerosis, higher blood pressure, insulin resistance, glucose intolerance, hyperlipidemia and abdominal obesity. [scienceopen.com]
[…] clinical manifestations; d) 1 mg/kg/daily in not extensive and progressive skin disease of pemphigus foliaceus 8 ; e) 1mg/kg/daily for both pemphigus in the presence of relative contraindications to corticosteroids: peptic ulcer, diabetes, cataracts, osteoporosis [scielo.br]
If patients will be on chronic corticosteroids (at least 5mg daily prednisone equivalent for at least 3 months), osteoporosis counseling and prevention is indicated. [dermatologyadvisor.com]
Skin
- Blister
(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]
The diagnosis of fogo selvagem is based on both the local prevalence of the disease and its characteristic blisters and a positive Nikolsky sign (where gently rubbing affected skin causes a blister to form). [dermnetnz.org]
The blisters don’t itch. They can be painful. Blisters may then appear on the skin and sometimes on the genitals. Pemphigus foliaceus Pemphigus foliaceus doesn’t cause blisters in the mouth. The blisters first appear on the face and scalp. [healthline.com]
Pathol. any of several diseases, often fatal, characterized by blisters on the skin and mucous membranes. [1770 80; < NL < Gk pemphig (s. of pémphix) bubble + L us n.… … Universalium [translate.deacademic.com]
- Skin Disease
It is a very comprehensive and practical guide and should not be missed by those treating patients with skin diseases. [books.google.de]
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]
- Nikolsky's Sign
It is manifested clinically by involvement of healthy-appearing skin that blisters when rubbed (Nikolsky sign). PF usually does not involve mucous membranes. In 1844, Pierre Louis Alphee Cazenave reported the first authenticated description of PF. [imagejournals.org]
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]
- Ulcer
Ulceration of mucosal surfaces is common and can be extensive. Untreated, most patients die within 5 years from secondary sepsis, electrolyte imbalance and/or emaciation. [apps.who.int]
Oral ulcers 5. Arthritis 6. Serositis 7. Renal disorder 8. الصفحة 637 - In vivo epiluminescence microscopy of pigmented skin lesions. I. Pattern analysis of pigmented skin lesions. J Am Acad Dermatol 17:571-583 6. [books.google.com]
Bhattacharya* A 72 year old gentleman, known to have Diabetes mellitus, Hypertension and Ulcerative colitis, was admitted to the Kothari Medical Centre, Kolkata, India, in October 2015 manifesting bullous lesions and erythroderma all over the body for [imagejournals.org]
Oral ulcers 5. Arthritis 6. Serositis 7. Renal disorder 8. Pagina 637 - In vivo epiluminescence microscopy of pigmented skin lesions. I. Pattern analysis of pigmented skin lesions. J Am Acad Dermatol 17:571-583 6. [books.google.ro]
Intravenous (IV) feeding If your mouth ulcers are severe, you may not be able to eat without pain. You may need to be fed through your veins. This involves using an intravenous (IV) connection. [healthline.com]
- Pruritus
IgG to BPAG (bullous pemphigoid antigen; component of hemidesmosome ) – subepidermal nonacantholytic blisters from basal cell vacuolization – DIF: continuous linear deposition of IgG along basement membrane with complement fixation – tense bullae with pruritus [usmle287.wordpress.com]
[…] with pemphigus vulgaris but also possible from mother with pemphigus foliaceus IgA pemphigus (2 major subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis) Fragile blisters filled with fluid that evolve into pustules, pruritus [arupconsult.com]
The clinical spectrum is broad; however, patients can have intense pruritus at any stage of the disease. Blisters are subepidermal. Oral involvement is rare. [clevelandclinicmeded.com]
Pruritus may or may not be present. Mucous membranes are usually spared. [pemphigus.org]
Topical betamethasone was started, but was replaced by prednisolone, 2 mg/kg/day, because of lesion spreading, severe pruritus, and disturbed sleep. [adc.bmj.com]
Neurologic
- Irritability
الصفحة 399 - Application of retinol to human skin in vivo induces epidermal hyperplasia and cellular retinoid binding proteins characteristic of retinoic acid, but without measurable retinoic acid levels or irritation. [books.google.com]
Pagina 399 - Application of retinol to human skin in vivo induces epidermal hyperplasia and cellular retinoid binding proteins characteristic of retinoic acid, but without measurable retinoic acid levels or irritation. [books.google.ro]
Chronic skin infections due to ruptured and irritated blisters. The blisters and lesions caused by PF can look alarming, but they do not indicate the presence of another condition. On its own, pemphigus is not life-threatening. [medicalnewstoday.com]
The treatment for the blisters may also include: numbing lozenges for mouth blisters soothing lotions wet dressings pain medications soft-food diets avoidance of spicy or acidic foods that may irritate the blisters avoidance of too much sun exposure If [healthline.com]
Since pemphigus vulgaris can affect the mucous membranes in your mouth, you may need to adjust your diet so that your foods are bland and soft to avoid irritating the blisters in your mouth. [my.clevelandclinic.org]
Workup
Microbiology
- 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]
Treatment
Alongside technology, new drugs, methods and treatments are continuously developed for the treatment of all common skin diseases. [books.google.de]
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]
(no treatment, treated, and post-treatment). [journal.frontiersin.org]
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]
Biologics: This is a newer treatment option. One biologic, rituximab, seems promising. It appears to offer safe treatment. [aad.org]
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]
The objective of this analysis is to compare pemphigus vulgaris and pemphigus foliaceus, concerning demographic data, clinical course, and prognosis. Thus, the authors are sharing their experience in the management of these two major variants. [emjreviews.com]
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]
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]
Inflammatory cytokines and apoptosis are also involved. [15] 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]
Shoenfeld received the EULAR prize in 2005, in Vienna, Austria: "The infectious etiology of anti-phospholipid syndrome". [books.google.ro]
Epidemiology
The disease follows the course of streams and creeks and vanishes after urbanization of the endemic areas. [10] Epidemiologic findings indicate that peasants exposed to black fly bites are 4.7 times more likely to develop fogo selvagem than those who [emedicine.medscape.com]
[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]
Epidemiologic data strongly support the notion that this disease is caused by exposure to an environmental agent or agents. [ncbi.nlm.nih.gov]
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]
The pathophysiological process is controversial, but it is certain that pemphigus vulgaris causes mucosal and skin lesions, reaching the suprabasal layer by producing desmoglein-3. [scielo.br]
Objectives: Review the pathophysiology of pemphigus foliaceus. Describe the typical presentation of pemphigus foliaceus. Outline the treatment options for pemphigus foliaceus. [ncbi.nlm.nih.gov]
[…] 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]
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]
Prevention
These can prevent the blisters from getting infected if they break open. If blisters cover a lot of your skin, you might need to stay in the hospital for treatment. Doctors and nurses will clean and bandage your sores to prevent infection. [healthline.com]
Dermatologist-approved lesson plans, activities you can use Free to everyone, these materials teach young people about common skin conditions, which can prevent misunderstanding and bullying. [aad.org]