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Lection 5

Blistering (bullous) dermatoses

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Etiology and pathogenesis


The group of vesicular and pemphigus dermatoses includes different diseases on the basis of etiology and pathogenesis (pemphigus, Duhrings dermatosis, simple vesicular lichens, herpes zoster, exudative multimorphic erythema). There are different etiopathogenic theories, in particular, the viral theory but it is not completely proved. Recently autoimmune processes are considered to be of great importance in the pathogenesis: discovery of antibodies to intercellular substance in the skin, in liquid of the vesicles and in blood serum. In immunofluorescence, in intercellular space of stratum spinosum of epidermis immunoglobulin G is found only in the 2 patients with pemphigus.

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Peculiarities of the course of pemphigus


The morphologic basis of development of vesicles in this disease is acantholysis destruction of protoplasmic process of the cells of the epidermis stratum spinosum, weakness of intercellular contacts and accumulation of exudate in this layer, due to which inter epidermal vesicle is formed. Acantholysis underlies Nikolskys phenomenon: by pulling the covering of the vesicle, the epidermis exfoliates outside the boundary of the vesicle on the healthy skin. During severe scratching of the healthy skin between the vesicles the exfoliation of the epidermis takes place. Nikolskys symptom is more expressed in the stage of pemphigus exacerbation. The vesicles appear suddenly on the healthy skin or mucosa, then open and form erosions, or their exudate gets covered by crust. The disease has a chronic character, continuous progress and in the absence of treatment soon results in lethal outcome.

Classification of pemphigus
pemphigus vulgaris (common), pemphigus vegetans, pemphigus foliaceus (exfoliative) seborrheal pemphigus.
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Pemphigus vulgaris.
Pemphigus vulgaris is a rare autoimmune disease that is characterised by blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. It is the most common subtype of pemphigus, accounting for 70% of all pemphigus cases worldwide although it is extremely rare in New Zealand (about one case per million). Discovery of Tzanck acantholytic cells in the smear from the erosion, discovery of immunoglobulin G during immunofluorescence in the intercellular space of the stratum spinosum. 5

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Pemphigus vulgaris.

Pemphigus vulgaris is an autoimmune blistering disease, which basically means that an individual's immune system starts reacting against his or her own tissue. The building block cells of the epidermis are called keratinocytes. These cells are cemented together at special sticky spots called desmosomes. In pemphigus vulgaris immunoglobulin type G (IgG) autoantibodies bind to a protein called desmoglein 3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid, the blister. 6

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Pemphigus vulgaris. Clinical features.


Most patients first present with lesions on the mucous membranes such as the mouth and genitals. Several months' later blisters on the skin may develop or in some cases mucosal lesions are the only manifestation of the disease. The most common mucosal area affected is the inside of the mouth but others include the conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus.

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Pemphigus vulgaris. Clinical features.


Common features of oral mucosal lesions include: 50-70% of patients get oral lesions blistering superficial and often appears as erosions widespread involvement in the mouth painful and slow to heal may spread to the larynx causing hoarseness when talking may make it difficult to eat or drink Skin lesions appear as thin walled flaccid blisters filled with clear fluid that easily rupture causing painful erosions. 8

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Pemphigus foliaceus
The pemphigus family are rare autoimmune blistering diseases affecting skin and/or mucous membranes. Pemphigus foliaceus (PF) is a rare relatively benign form of pemphigus. In New Zealand pemphigus foliaceus is more often encountered than its more serious relative pemphigus vulgaris, although worldwide pemphigus vulgaris is more common. Pemphigus foliaceus is characterised by blistering lesions on otherwise healthy-looking skin. Blisters tend to form when the skin is rubbed (Nikolsky sign).

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Causes of PF
Pemphigus foliaceus is an autoimmune disease, which basically means that an individual's immune systems starts reacting against his or her own tissue. The building block cells of the epidermis are called keratinocytes. These cells are cemented together at special sticky spots called desmosomes. In pemphigus foliaceus autoantibodies bind to a protein called desmoglein-1, which is found in desmosomes in the keratinocytes near the top of the epidermis.

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Causes of PF
The result is the surface keratinocytes separate from each other, and are replaced by fluid: the blister. Because the blister is very close to the surface of the skin the blisters rupture easily. In most cases the autoantibodies are immunoglobulin type G (IgG) but in IgA PF the autoantibodies are type A (IgA). PF is sometimes provoked by sun exposure. Endemic PF occurs in South America, where it is commonly known as Fogo Selvagem. It appears to be set off by a virus transmitted by an insect bite. Penicillamine, nifedipine, captopril or nonsteroidal anti-inflammatory drugs most often provoke drug-induced PF. If the drug is stopped, there is a 50% chance the PF will clear up. 11 Lection 5Lection 5

PF. Clinical features


Pemphigus foliaceus is confined to the skin and there is little or no involvement of mucous membranes. This is in contrast to pemphigus vulgaris where there may be extensive mucous membrane involvement (mouth, eyelids etc). The patient is usually otherwise in good health. Small fluid-filled blisters first form on the trunk. Because they form in the upper layers of the epidermis they rupture very easily and only erosions may be seen. On the face, scalp and upper trunk the lesions are often scaly and crusty on a red and inflamed base. A burning sensation or localised pain may be felt. 12

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Pemphigus vegetans
Erosions in the skin folds may develop into vegetative lesions which are granular and crusty looking (known as pemphigus vegetans). At the beginning of its development this form of pemphigus is clinically similar to pemphigus vulgaris and often starts with the appearance of lesions on the oral mucosa. From the very onset of the disease, however, attention is drawn to the tendency of the bullae to be localized around the natural orifices, the navel and in the region of the large skin folds (axillary, inguinofemoral, intergluteal, under the mammary glands, behind the ears).

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Pemphigus vegetans
Papulomatous growths secreting a considerable amount of exudate are formed later in places of the ruptured bullae against the background of an eroded surface covered with a dirty film. The lesions tend to coalesce and form large vegetative surfaces at places with purulent necrotic disintegration. Nikolsky's sign is often positive. The dermatosis is accompanied with pain and a sensation of burning. Active movements are difficult because of sharp pain.

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Pemphigus seborrhoicus or erythematosus (Senear-Usher syndrome)


Pemphigus seborrhoicus belongs to the group of true pemphigus because the possibility of its development into the foliaceus or vulgaris variant has been authentically proved. In most cases, how-ever, the disease is benign, though of a long duration. The lesions usually first appear on the face and less frequently on the scalp, chest and back. Closely arranged greasy crusts are formed against an erythematous background and simulate the picture of cicatrizing erythematosis on the face. Moist eroded surfaces are exposed when the crusts are removed. Acantholytic cells are seen in impression smears from these surfaces. The bullae are often formed unnoticeably and the crusts seem to be primary lesions. In other cases vesicles covered with stratified yellowish crusts are formed on the trunk and limbs, in sites characteristic of seborrhea. Lesions rarely occur on the mucous membranes, but if this happens, they are a bad prognostic sign. 15

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Dermatitis herpetiformis
An uncommon skin condition with the peak incidence around 30-40 years of age. Male is more common than female. In western countries, the disease usually associates with coeliac disease. In Hong Kong, since coeliac disease is extremely rare, this association is not usually observed. Lesions are composed of excoriated and scabbed intensely itchy papules. The sites of predilection are extensors of 4 limbs, buttocks and face. Intact vesicles seldom seen since they are ruptured by self scratching. Lesions heal with no scarring.

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Differential sign Primary elements Character of lesions Erosion Localization of the vesicle Character of the elements Localization of elements Infection of the mouth cavity Nicolskys symptom Itch Course Prognosis Tzanck acantholytic cells Reaction with iodine Eosinophilia Therapeutic effect

Acantholytic pemphigus Vesicles on the healthy skin Flaccid, thin cover soon opens Often extensive, without the tendency towards epithelization Intraepidermal Monomorphic Unsystematic In most patients the disease often starts in the mouth cavity Positive Rare Continuos course Serious, in severe cases may be lethal Discovered Absent Not high in blood; rare in vesicles Absent

Dermatitis herpetiformis (Duhrings disease) Vesicles, bulla, macula, papule, pustule (polymorphism) The cover is dense, exists for 7-10 days Often less; soon heals Subepidermal Polymorphic Geometric, i.e. grouped Very rare; does not start in the mouth cavity. Negative Usually intense Relapsing More favourable, rare transformation into pemphigus Not discovered Causes intensification Frequent in blood (20-30%) Nearly always high in vesicle Marked in many cases

Multiform exudative erythema


Multiform exudative erythema is an acute skin disease, which is characterized by polymorphism of lesions, tendency towards relapsing and seasonal development. Lection 5Lection 5 18

MEE. Course.
Two forms of MEE are differentiated: idiopathic, during which the etiologic factor is not known, and symptomatic, connected with a defined reason (medicaments, sometimes vaccines, presence of focal infection, acute infectious diseases) The characteristic lesions for MEE are sharply limited maculae and edematous compressed papules; tendency towards peripheral growth and towards fusion into plaques. Pay attention to two-coloured lesions (pinkish-red in the centre; bluish-violet, cyanotic on the periphery) and impression in the centre of plaques. Simultaneously with the maculae and papules, blisters and even vesicles and bullae are often formed: true polylmorphism. Such multiform eruption and its exudative character is the reason for the name of this disease.

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Herpes simplex
There are five types of herpesvirus. Herpes simplex1 (HSV-1) and herpes simplex-2 (HSV-2, the most common type) are considered sexually transmitted diseases. Herpesvirus can be transmitted from a mother to her developing fetus, which may impair neurological development and can be fatal. Lection 5Lection 5 20

Herpes simplex

HSV-1 causes cold sores and fever blisters on the mouth and has been found in genital lesions as well. HSV-2, commonly called genital herpes, causes internal and external genital sores and blisters.
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Herpes simplex
Herpes is a lifelong, incurable disease but can be managed with antiviral drugs and safer sex practices. The virus resides deep in nerve cells and it may never produce symptomatic disease or may actively recur throughout a person's lifetime. The latent nature of the virus contributes to its insidious spread. Lection 5Lection 5 22

Herpes simplex
Small (2-5 mm), fluid-filled lesions on the genitals, buttocks, anus, and adjacent areas are the telltale sign of infection. They are painful and often occur in clusters. They can also develop inside the vagina, on the cervix (where they cause inflammation), or in the rectum. Lesions break open and ooze for a few days before crusting over and healing. 23

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Herpes simplex
The first symptoms (primary infection) can last 3 weeks and are usually severe. Symptoms disappear as the virus retreats to the nerve cells near the sacral region of the spine and becomes latent until reactivated by a trigger. The virus then travels down the nerve cell to the skin, where new sores erupt. Many people experience itching, tingling, or heightened sensitivity and Recurrent herpes simplex labialis tenderness in the area of the original infection a few hours before the sores appear. Some experience pain in the buttocks or knees.

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Herpes simplex
Recurrences can be triggered by: Minor trauma. Other infections including minor, upper respiratory tract infections. Ultraviolet radiation (sun). Conditions affecting the nerves of the face. Operations on the face nerves. Dental surgery. Herpes simplex on buttock with Dermabrasion or laser scarring from previous episodes resurfacing. Menstrual cycle (flare-ups may occur before the monthly period). 25

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Herpes zoster
Herpes Zoster is a painful blistering rash caused by reactivation of Chickenpox virus. During this widespread infection, which usually occurs in childhood, virus is seeded to nerve cells in the spinal cord, usually of nerves that supply sensation to the skin. The virus remains in a resting phase in these nerve cells for years before it is reactivated and grows down the nerves to the skin to produce HZ. This can occur in childhood but is much more common in adults, especially the elderly.

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Herpes zoster
The first sign of shingles is usually pain, which may be severe, in the areas of one or more sensory nerves, often where they emerge from the spine. The pain may be just in one spot or it may spread out. The patient usually feels quite unwell with fever and headache. The lymph nodes draining the affected area are often enlarged and tender. Within one to three days of the onset of pain, a blistering rash appears in the painful area of skin. Sometimes, especially in children, HZ is painless.

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Herpes zoster
It starts as a crop of closely-grouped red bumps in a continuous band on the area of skin supplied by one, occasionally two, and rarely more neighbouring spinal nerves. New lesions continue to appear for several days, each blistering or becoming pustular then crusting over. HZ occasionaly causes blisters inside the mouth or ears, and can also affect the genital area. The pain and general symptoms subside gradually as the eruption disappears. In uncomplicated cases recovery is complete in 2-3 weeks in children and young adults, and 3 to 4 weeks in older patients.

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Herpes zoster
Herpes zoster ophthalmicus Occasionally pain is not followed by the eruption - HZ "sine affecting the trigeminal eruptione". The chest nerve (thoracic), neck (cervical), forehead (the forehead) and lumbar/sacral sensory nerve supply regions (ophthalmic)

are most commonly affected at all ages but the frequency of ophthalmic shingles increases with age. Rarely the eruption may affect both sides of the body. In elderly and undernourished patients the blisters are deeper. Healing may take many weeks and be followed by scarring. Muscle weakness arises in about one in twenty patients because the muscle nerves are affected as well as the sensory nerves. Facial nerve palsy is the most common result. There is a 50% chance of complete recovery and in time some improvement can be expected in nearly all cases. 29

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