1. Pemphigoid
Autoantibodies attack antigens in dermal-epidermal junction
Bullous pemphigoid:
Affects elderly people
It is itchy
Flexures are often affected
Nikolsky sigh is negative
Usually not fatal but risk factors are:
Old age
The need for high steroid dosage
Low serum albumin level

2. Course:
A self-limiting disease and treatment can often be stopped after 1-2 years
Complications:
Of steroids and immunosuppressives
If untreated, discomfort, and fluid loss
DDx:
Dermatitis herpetiformis
Other bullous diseases

3. Investigations:
Immunofluorescence will show IgG & C3 along the basement membrane
IgG antibodies in serum against basement membrane zone in 70% of patients
Peripheral eosinophilia

4. Treatment:
Potent topical steroids in mild cases
In acute phase, systemic steroids: prednisolone mg/day
Immunesuppressives e.g. azathioprine
Pemphigoid gestationis
It is pemphigoid that occurs with either:
Pregnancy
Hydatiform mole or
Choriocarcinoma
Usually it remits after birth
Treatment is by systemic steroids
Oral contraceptives should be avoided

5. Dermatitis herpetiformis:
Very itchy chronic subepidermal vesicular disease in which the vesicles are arranged into groups similar to those of herpes simplex. For this reason it is called herpetiformis.
Cause:
Gluten-sensitive enteropathy is always present, but it is asymptomatic. It is a mild enteropathy.
There are antibodies against tissue tranglutaminase, reticulin, gliadin, and endomysium which is a component of smooth muscle.

6. Presentation:
Sites particularly affected are: elbows, knees, buttocks and shoulders. Extremely itchy grouped vesicles which soon break due to scratching leaving grouped excoriations.
Course:
The condition lasts for decades unless patients avoid gluten entirely
Gluten-containing diet examples: wheat, barley, rye
Gluten-free diet examples: corn, potatoes, rice, and tapioca
Complications:
Diarrhea, abdominal pain, anemia, and rarely malabsorbtion
DDx:
Scabies
Excoriated eczema
Insect bite
Neurodermatitis

7. Investigations:
Biopsy will show the the histology of subepidermal blister
Direct Immunofluorescence of uninvolved skin shows granular deposits of IgA and usually C3
Serum antibody tests for anti-endomysial antibodies or tissue transglutaminase can help diagnose the enteropathy
Treatment:
Gluten-free diet
Dapsone or sulfapyridine, although both can cause:
severe rashes
haemolytic anaemia (especially in those with glucose-6-phosphate dehydrogenase deficiency)
leucopenia
thrombocytopenia
methaemoglobinaemia and
peripheral neuropathy
Regular blood checks are therefore necessary

8. Inherited bullous disorders:
Epidermolysis bullosa:
Many types. All are characterized by an inherited tendency to develop blisters after minimal trauma
The level of formation of blisters (the level of split) is either intra-epidermal (as in simple epidermolysis bullosa) or subepidermal, which in turn is either in lamina lucida (as in junctional epidermolysis bullosa) or beneath lamina densa (as in dystrophic epidermolysis bullosa)

9. Cause:
Simple epidermolysis bullosa: inherited abnormality in gene responsible for keratin production (keratins 5 and 14). Autosomal dominant
Junctional epidermolysis bullosa: mutations in gene responsible for laminin formation. Autosomal recessive
3. Dystrophic epidermolysis bullosa (DEB): There are many subtypes, all of which probably result from abnormalities of collagen VII, the major structural component of anchoring fibrils.

11. Simple epidermolysis bullosa:
Presentation:
Simple epidermolysis bullosa:
blisters start when baby starts crawling i.e. due to trauma
heal without scars because are superficial
sweating and ill-fitting shoes worsen the condition
nails and mucosae are mildly involved
Treatment:
avoiding trauma
wearing soft well-fitting shoes and using foot powder
large blisters should be picked with a sterile needle and dressed . their roofs should not be removed
local antibiotics may be needed

12. 2-Junctional epidermolysis bullosa:
This rare and often lethal condition is evident at birth
The newborn child has large raw areas and flaccid blisters, which are slow to heal
The peri-oral and peri-anal skin is usually involved
The nails and oral mucous membrane are usually involved
There is no effective systemic treatment.
Hopes for the future include adding the normal gene to epidermal stem cells, and then layering these onto the denuded skin.

13. 3-Dystrophic epidermolysis bullosa (DEB):
Autosomal dominant DEB:
In late infancy
On friction sites (knees elbows and nails)
Heal with scars and milia
Deformed or even lost nails
Mouth not affected
The only treatment is to avoid trauma and to dress the blistered areas

14. ii.Autosomal recessive DEB:
ii.Autosomal recessive DEB:
In infancy
Blisters may fill in blood
Heal with scar which can be severe scar so that nails are lost and webs form between digits
Hands and feet may become useless balls by losing all fingers and toes
Teeth, mouth and upper esophagus are affected (esophageal strictures)
Squamous cell carcinoma may develop
Treatment is unsatisfactory> it is generally as follows:
Minimize trauma
Maintain nutrition
Prevent contractures and web formation
Treat anemia
Treat secondary infection