Bollous Diseases

8/13/2019 Bollous Diseases

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BOLLOUS DISEASES

- Any diasease marked by eruptions of blisters, or bullae filled with fluid,on the skin or mucous membranes

- Results from the actions of the immune system attacking the healthycells in the body (autoimmune disorder) by producingautoantibodies.In the skin, there are proteins that attach epidermal

cells to each other and proteins that attach the epidermis to the dermis.These proteins are the glue that keeps the skin intact.When theseproteins are damaged by autoantibodies,the cells separate from eachother and a blister appears

- Result in the appearance of blisters or the accumulation of fluidbneneath two layers of skin that results in raised bumps

- No cure but can be controlled with treatment

- Occurs more in women especially women of childbearing age


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Types of Bollous Disorders

A. Pemphigus- Derived from the Greek word pemophix meaning bubble or blister- Blistering and raw sores inside the mouth or on the skin- May present or worsten during pregnancy (1st and 2nd trimesters)- Menstruation triggers or cause relapses- Risk factors : pregnancy, frequent handling of certain spices,

repeated minor thermal burns and cosmetic proceduresMain Types:

1. Pemphigus vulgaris- occurs in 40-60, Jewish or Miditerranean descent- affects inside of the mouth/ oral sores in 50-70% of affected

people spreading to the upper part of the troat making eatingvery difficult- 70% of pemphigus cases- application of firm pressure on normal looking skin produces a

tear or sore (Nikolskys sign)- does not spread to any internal organs


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2. Pemphigus vegetans- a localized form of pemphigus vulgaris in which there is a

localized vegetating papillomatous response. The eroded areas do notheal like usual but form papillomatous growth and vegetation.


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3. Pemphigus foliaceus

- characterized by crusty sores that often begin on the scalp, and may

move to the chest, back, and face.- sores are superficial and often itchy, usually not painful

- mouth sores are significantly absent

- the protein is found only on the top dry layer of the skin


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4. Paraneoplastic pemphigus

- painful sore appear in the mouth, lips, esophagus associated with

underlying cancer- usually results in fatal destruction of lung tissue (bronchiolitisobliterans)

- rarest and the most severe type often diagnosed before the tumoritself

- irreversible but can be improved completely removing or treating thetumor


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Complications

1. Systemic infection

2. Fluid losses through the skin

Treatment (Aim: decrease blister formation,prevent infections, promotehealing of blisters and erosions )

1. Oral corticosteroids eg. Prednisone2. Immunosuppressants

- quiet the immune attack so that blistering stops and sores can heal

3. Plasmapheresis (reduce antibodies in the blood)

Diagnosis

1. Visual examination of skin lesions

2. Skin biopsy

3. Direct immunoflourescence examination


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Two Major Forms of Pemphigoid1. Bullous Pemphigoid

- age of onset: 65-75 y.o.; 14 cases/million/year- a blistering disease of the skin caused by autoantibodies directedagainst skin proteins that connect the epidermis to the dermis or in thebasement membrane (connects the lower skin layer, dermis, to the upperskin layer, epidermis) attracting cells of inflammation causing largeblisters, itching and pain- Precipitating factors: exposure to ultraviolet light and radiation therapy,

drugs(furosemide,ibuprofen,NSAIDs,captopril,penicillamine,antibiotics)- blisters heal without scarring- affects the skin of the lower abdomen, groin, flexor surfaces of thehands and feet- classified as type II hypersensitivity reaction- Sn/Sx: early lesions appear urticarial (hives) and tense bullae (multiple

blisters) eventually erupt, break open and form ulcers or open sores,itching, rashes, mouth sores, bleeding gums- cause is not known but may be related to immune system disorders,certain diseases, or use of some medications- condition goes away within 6 years


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2. Mucous membrane pemphigoid (MMP,Cicatricial pemphigoid, benignmucosal pemphigoid, benign mucous membrane pemphigoid, ocular

pemphigus, scarring pemphigoid)- a blistering disease that can arise on any mucous membrane surfaceor subepithelial areas characterized by erosive skin lesions that resultsin scarring of at least some sites of involvement including the nose,mouth,eyes,esophagus, larynx, urethra, and anal mucosa

- target ages 60-80 y.o.- needs prompt treatment to prevent scarring and spontaneousimprovements and remissions are rare

- (+) for: Nikolskys sign, IgG, circulating auto-antibodies to BP-1antigen


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Signs and Symptoms

Site Features

Eye sensation of grittiness or pain

conjunctivitis

lesions form, eorde and heal to leave scar tissue

may lead to impaired vision or blindness

Mouth blisters form first in the gums near the teethpalate,tongue, lips, buccal mucosa, floor of the mouthand throat may be affected

painful and difficult to eat

lesions ocurring in the throat (esophagus, trachea,

larynx) and can become life threateningSkin blisters on the skin develop in 25-30% cases

may be itchy

bleeding may occur if traumatized


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Site FeaturesNose nosebleeds after blowing the nose

crusting causing discomfortGenitals painful blisters and erosions on the clitoris,labia,shaft ofthe penis, perianal area

B. Epidermolysis Bullosa Acquisita

- Chronic, uncommon, sunepidermal blistering disease of the skin andmucous membranes

- onset: 50 y.o.- Inherited or as a result of a random mutation ina gene that occurred

during the formation of an egg and sperm cell

- Where and when blisters develop depend on the type of epidermolysisbullosa


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Symptoms include:

Alopecia

Blisters around the eyes and nose

Blisters in and aroundthe mouth and throat, casuing feedingproblems or swallowing difficulty

Blisters on the skin as a result of minor injkury or temperature change

Blistering that is present at birthDental problems such as tooth decay

Hoarse cry, cough, or other breathing problems

Milia (tiny white bumps or pimples)

Nail loss or deformed nails


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Diagnosis

- Appearance of the skin

- Genetic testing

- Skin biopsy

- Special microscopic tests of skin samples

- Blood test for anemia

- Culture to check for bacterial infection when wounds are healingpoorly

- Upper endoscopy or an upper GI series if there are swallowing orfeeding difficulties

- Observation of growth curves in children

- Testing of limb range of motion with contractures


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Treatment

Goal: prevent blisters from forming and complications

Interventions: Avoid skin damage (trauma) and hot environments

Proper skin care, regular whirlpool therapy and antibioticointments, bandaging or dressing

Oral steroids for short periods of time, medication for candida ifapplicable

Good dental; hygiene, regular dental visits

Proper nutrition: extra calories and protein, avoid eating hard orbrittle foods such as pretzels, nuts, and chips. Eat soft foods.

Work with a PT to keep full range of motion in the joints andminimize contractures

Skin grafting for denuded or ulcerated areas of the skin Other surgeries: dilation of the esophagus with stricture, repair of

hand deformities, removal of any squamous cell carcinoma thatdevelops

Gene Therapy


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PrognosisContractures or deformities (eg. Fingers, elbows, knees)

Feeding and swallowing difficultiesFused fingers and toesRestricted mobility from scarringSecondary infection is common

ComplicationsAnemiaDeathEsophageal strictureEye disorders including blindnessInfection including sepsis

Loss of function in the hands and feetPeriodontal diseaseMuscular dystrophySevere malnutritionSquamous cell skin cancer


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PreventionGenetic counsellingChorionic villi sampling during pregnancy to test fetus (8-10th week ofpregnancy)

Wear padding around injury-prone areas such as elbows, knees, ankles,buttocks to prevent skin damage and blistering.

Avoid contact sports.IF UNDER STEROIDS FOR MORE THAN 1 MONTH, TAKE CALCIUM

AND Vit DSUPPLEMENTS TO PREVENT OSTEOPOROSIS.

C. Dermatitis Herpetiformis and IgA Mediated Disorders (Duhrings disease)- a chronic bullous skin disorder that occurs with patients with glutensensitivity and not related to herpes virus- blisters occur on the buttocks and back causing severe itching

- caused by reduced deposits of IgA and complement and alsoautoantibodies to reticulin,gliadin, and gluten- lasting remission due to gluten-free diet- onset: 20 y.o. and older; hereditary- unknown cause but linked to gluten sensitivity (celiac disease) in thesmall bowel


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Symptoms:

Extremely itchy red bumps or blisters (subepidermal and rounded

lateral borders)

Rash is usually the same size and shape on both sides

Some patients may have scratch marks instead of blisters

Three Stages of Rashes:1st Stage: slight discoloration of the skin at the site where the lesions

appear

2nd Stage: skinlesions transform into obvious vesicles and papules thatare likely to occur in groups

3rd Stage: healing of the lesionscharacterized by change in skin colorturning lighter or darker than the rest of the body. Crust may form dueto intense itching and scratching.


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Diagnosis

Skin biopsy

Biopsy of the intestines

Blood test for IgA antibodies

(+) lymphocytes, eosinophils, neutrophils in affected areas

Treatment

Dapsone (drug of choice: antibiotic, reduces itching in 2-3 days)Other choices: colchicine, tetracycline,sulfamethoxypyridazine

Strict gluten-free diet (lifelong treatment)

Immunosuppressive medications

PrognosisWell controlled with treatment but may cause risk of intestinal cancerif without treatment


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Complications

Thyroid disease

Cancer of the intestine

Prevention

None. Avoid foods containing gluten


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Bollous Diseases