Pemphigus.
Pemphigus is a disease that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older. It tends to be a long-lasting (chronic) condition, and some types can be life-threatening without treatment.
Etiology
Autoimmune process that cause acantholysis into the epidermis. Antibodies against desmosomes below the epidermis attack and degrade the desmosomes. This process leads to acantholysis and formation of bubbles filled with exudate.
Types:
There are two main types of this condition. What symptoms you have will depend on what type you have.
- I. PEMPHIGUS VULGARIS– This is the most common form. It affects the moist parts of your body, like your mouth and genitals. Adults between ages 30 and 60 are most likely to get it. The first sign of a problem will typically be blisters in your mouth that peel easily. You may find it hard to swallow or eat.
- II. PEMPHIGUS FOLIACEUS - These are crusty blisters that tend to form on your chest, back, and shoulders. They don’t hurt, but they do itch.
Risk factors:
- Viral infections
- Medications
- Radiation
Clinical presentation
2 principal localization:
- Oral Localization:
- Tissues around lips
- Buccal mucosa
- Present in half of the patients and may present before the skin lesion
- Very painful
- Difficult to speak and eat
Oral lesions:
- Briefly lasting bullae
- Easily bleeding and erosive
- Excessive salivation
- Halitosis
- Skin Localization:
- May not be present in old patients
- Most frequent location at body folds, face and scalp
Skin lesions:
- Present as loose bubbles of different sizes with clear or hemorrhagic contents
- Bullae rupture easily and expose bright red painful erosions
- Once dry, dark/brown hemorrhagic crust are formed
Diagnosis
Phenomena:
- Nikolsky sign- when press onto the skin close to the active lesion we obtain exfoliation of epidermis
- Asboe- Hansen sign- when pressure is put on top of the bulla it expands
- Histological examination
- Skin biopsy
Treatment
- Local oral treatment with antiseptic solutions, antibiotic creams, antifungal creams, topical corticosteroids
- Systemic treatment with corticosteroids and cytostatics
- In severe cases combination therapy of corticosteroids with cytostatics