What is Fogo Selvagem?
Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate.
What does Acantholytic mean?
Acantholysis means loss of coherence between epidermal cells due to the breakdown of intercellular bridges. It is an important pathogenetic mechanism underlying various bullous disorders, particularly the pemphigus group, as well as many non-blistering disorders.
What drugs cause pemphigus?
The thiol group of drugs is the most common agent implicated in drug-induced pemphigus. Drugs known to cause pemphigus include amoxicillin, ampicillin, captopril, [19, 20] cephalosporins, penicillamine, penicillin, pyritinol, and rifampin.
What is Brazilian pemphigus?
Endemic pemphigus foliaceus is a variant of pemphigus, a group of autoimmune intraepithelial blistering skin diseases characterized by keratinocyte detachment (acantholysis) and consequent intraepithelial blister formation. Endemic pemphigus foliaceus primarily occurs in Brazil, where it is known as fogo selvagem (FS).
What is Senear Usher syndrome?
Senear–Usher syndrome refers to a combination of lupus erythematosus and pemphigus foliaceous. [1] Treatment of this condition usually comprises of steroids and immunosuppressants. However, occurrence of Senear–Usher syndrome with bipolar disorder has been very rarely described.
What is Pemphigus herpetiformis?
Pemphigus herpetiformis is a rare subtype of intraepidermal autoimmune bullous diseases that shares clinical characteristics with dermatitis herpetiformis and immunologic features with pemphigus. It usually has an indolent course and is less life-threatening than many forms of pemphigus.
What is Suprabasal acantholysis?
Suprabasal acantholysis, a common feature of several inflammatory skin diseases, develops exclusively within the germinative cellular pool of the epidermis. The process induces repair mechanisms by proliferation of cells in the suprabasal layers in whatever conditions it occurs, except in pemphigus vulgaris.
What causes acantholysis?
However, other causes of acantholysis are severe spongiosis (any acute or subacute inflammatory dermatosis), ballooning degeneration (viral infection), proteolytic enzymes released by neutrophils (bacterial and fungal dermatoses), and neoplastic transformation (squamous cell carcinoma, actinic keratosis).
How does pemphigus start?
Pemphigus vulgaris.
This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful but don’t itch. Blisters in your mouth or throat may make it hard to swallow and eat.
Will pemphigus ever go away?
In some cases, pemphigus vulgaris will go away once the trigger is removed. The condition causes the immune system to fight against the body’s own cells in the same way that it fights off invading germs.
What is Rowell syndrome?
Rowell syndrome (RS) is a rare disease characterized by a combination of lupus erythematosus (LE) and erythema multiforme (EM)-like lesions and a characteristic immunologic pattern, including speckled pattern of antinuclear antibody (ANA), positive anti-Ro/SSA or anti-La/SSB, and positive rheumatoid factor (RF).
Can you have DH and not celiac?
The symptoms of dermatitis herpetiformis are extremely itchy and blistering skin. Sometimes referred to as gluten rash or celiac rash, DH is a chronic condition that is considered to be the skin form of celiac disease. Not all people with celiac disease develop DH, but most people with DH have celiac disease.
What triggers dermatitis herpetiformis?
DH is caused by the body’s immune system reacting to a protein called gluten in foods containing wheat, barley and rye. This reaction causes a skin rash to develop.
What does Suprabasal mean?
Above a basal layer
suprabasal (comparative more suprabasal, superlative most suprabasal) Above a basal layer.
Can Grover’s disease spread?
Grover’s disease is not contagious, even when another person comes into contact with the rash.
Can stress cause Grover’s disease?
The disease is most likely caused by blocked sweat ducts and can begin rather suddenly, resulting in itchy spots on a person’s central back, mid-chest, and on occasion, elsewhere on the person’s body. Grover’s disease often follows heat stress.
Does pemphigus ever go away?
Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. Pemphigus isn’t contagious. It’s a lifelong condition that can be managed with ongoing medical treatment.
Who is at risk for pemphigus?
Middle-aged or older people are most likely to get pemphigus, including the most common type, pemphigus vulgaris. This type usually begins between 50 and 60 years of age. Your risk of getting pemphigus vulgaris increases if you have: Jewish ancestry, especially Ashkenazi Jewish heritage.
Can you live a normal life with pemphigus?
For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while. Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type.
How does pemphigus cause death?
One study of 159 patients with pemphigus vulgaris admitted to the hospital from 1980 to 1998 documented a mortality rate of 8.8 % (14 patients) during hospital treatment; the main causes of death were cardiopulmonary failure and sepsis (Ljubojevic et al., 2002).
What is Chilblain lupus?
Chilblain lupus erythematosus is a chronic form of lupus erythematosus seen especially in women, affecting the fingertips, rims of ears, calves, and heels. CHLE lesions may be clinically indistinguishable from simple chilblains or perniosis, which is seen in healthy individuals.
What causes dermatitis herpetiformis to flare up?
DH is caused by a sensitivity or intolerance to gluten. Gluten is a protein found in wheat and grains. When you have DH and eat food with gluten, the gluten triggers an immune reaction. This causes material called IgA antibodies to be deposited in the skin.
What can be mistaken for dermatitis herpetiformis?
Dermatitis herpetiformis is often mistaken for other skin conditions like eczema, psoriasis, scabies, shingles, and allergic or contact dermatitis. What differentiates dermatitis herpetiformis is that it tends to affect both sides of the body at the same time. Can a Gluten Rash Go Into Remission?
How long does it take for DH to go away?
Dermatitis Herpetiformis Treatment
It may take about six months to achieve some improvement in the skin condition and up to two years or more to get total control through the gluten-free diet alone.
What are Suprabasal cells?
As the cells move to the upper layers of the epidermis they mature and eventually form cornified cells. The suprabasal cell layer lies directly above the basal layer and is composed of five to ten layers of cells.