Skin is the largest organ of the body. It forms a barrier against the external environment and serves as the body’s first line of defense.
There are hundreds of conditions and of a group of diseases that greatly affects the skin and the signs and symptoms are hardly distinguishable from each other unless you go to a deeper study and research about it or a thorough examination done by a medical expert for accurate diagnosis.
Pemphigus and Pemphigoid are two different skin diseases, but the presenting clinical manifestations quite resemble each other’s. Neither of these is not genetic nor contagious. These are both skin-blistering disease and autoimmune diseases.
Pemphigus
A group of rare autoimmune skin disorders (skin blistering) brought about acantholysis, the disruptions of connections between epidermal cells by antibodies against desmogleins (a type of transmembrane proteins which play a role in desmosomes formation). Desmosomes are specialized cellular structure that prevents shearing of connections between cells by gluing one cell to another.
Pemphigoid
Similar in blister appearance with that of phemphigus but the autoantibody activity is different. In Pemphigoid, no acantholysis takes place.
Pemphigus vs. Pemphigoid – The Comparison
|
Defining
Characteristics
|
Pemphigus
|
Pemphigoid
|
|
Types
|
- Pemphigus foliaceus
- Pemphigus vulgaris
- Paraneoplastic pemphigus
- IgA pemphigus
Sub-type
- Integumentary system condition (hair skin, nails)
|
- Gestational Pemphigoid or Herpes gestationis
- Bullous Pemphigoid
- Mucous membrane Pemphigoid or Cicatricial
|
|
Blister location
|
- Start with a blister in the mouth (intraoral) and can extend to the throat.
- Superficial intraepidermal.
- Mucous membranes of the genitals or perineum.
- Sores on mucosa of the mouth, lips and esophagus.
- Later stage on chest and back and face.
- Lung involvement bronchiolitis, but less frequent (irreversible).
|
- Subepidermal
- No cutaneous involvement.
|
|
Blister Characteristics
|
- Not itchy, but painful.
- Sores are painful (pemphigous pain).
- Very fragile.
- Infection risk is high.
|
- Bullae are intact.
- Less serious because bullae often don’t rupture.
- Infection is less.
|
|
Autoimmune antibody activities
|
Features acantholysis
|
- Does not feature acantholysis
- Auto antibodies lie against the basement of the epidermal membrane (not on epidermal junctions).
|
|
Prevalence
|
The condition can occur at any age, but is more often in middle age (30-60 years old)
|
- In women
- People 60 years old
|
|
Treatment and Management
|
- Early and prompt intervention prevents the disease from becoming more resistant to treatment.
- Treatment is similar for severe burns.
- Prednisone
- Oral steroids in high dosages
may cause intestinal perforations.
- Gastrointestinal system monitoring is vital.
- Oozing sores may come in contact with bed linens and clothes. Use of talcum powder may be helpful.
- Topical steroids (high-potency corticosteroids) for mild cases.
- Severe side effects require close monitoring.
- Intravenous gamma globulin for severe pemphigus is very expensive.
- Plasmaparesis for rebound pemphigus where more auto antibodies increases in numbers aims to reduce circulating antibodies.
- The effect is immediate and is safer.
|
- Drug of choice is corticosteroid for its potent anti-inflammatory effect.
- Steroids can cause immune suppression so it is recommended to use adjuvant drugs
- Dosages of these drugs are titrated when the infection is controlled
- The 3 Distinct phases of immunobullos diseases treatment.
- Control high dosage of therapy targeting to suppress the disease activity and control of further occurrence of new lesions.
- Consolidation continued drug treatment of constant dosage until all lesions totally disappear.
- Maintenance is tapering off medications gradually once most lesions are controlled and totally healed. This is done to reduce risk of side effects
|
*Note:
Familial benign Pemphigus also known as Hailey-Hailey disease is a hereditary skin disease. It doesn’t belong to the Pemphigus group of diseases.