Hedwig Weiss - Occupational Therapy in Epidermolysis Bullosa: A Holistic Concept for Intervention from Infancy to Adult

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Hedwig Wei and Florian Prinz Occupational Therapy in Epidermolysis Bullosa A Holistic Concept for Intervention from Infancy to Adult 10.1007/978-3-7091-1139-0_1 Springer Wien New York 2013

1. Epidermolysis Bullosa (EB) the Condition

Hedwig Wei 1 and Florian Prinz 1

(1)

University Clinic for Physical Medicine and Rehabilitation, State Hospital Salzburg, Salzburg, Austria

1.1

1.2

1.3

1.4

1.5

1.6

Abstract

Blister; primary skin eruption; a cavity filled with liquid raised above the normal skin level; caused by a simple separation between the layers of the skin, usually only one chamber; at least 5mm; differentiation is made between the localisation and the cause: subcorneal bulla (below the cornea), intra-epidermal bulla (in the epidermis), sub-epidermal bulla (between the epidermis and the corium) and acantholytic bulla (in the epidermis due to the breakup of the cell structures); bulla inflammatoris (inflammatory blister due to toxic noxa, inflammatory, allergic reactions, etc.), bulla mechanica (due to mechanical injury in epidermolysis), bulla actinica (due to exposure to the sun, e.g. hydroa vacciniforme), bulla spontanea (e.g. pemphigoid). Compare with efflorescence (Pschyrembel 2012, p. 328).

1.1 Definition

1.1.1 Bulla (lat.) f:

Blister; primary skin eruption; a cavity filled with liquid raised above the normal skin level; caused by a simple separation between the layers of the skin, usually only one chamber; at least 5mm; differentiation is made between the localisation and the cause: subcorneal bulla (below the cornea), intra-epidermal bulla (in the epidermis), sub-epidermal bulla (between the epidermis and the corium) and acantholytic bulla (in the epidermis due to the breakup of the cell structures); bulla inflammatoris (inflammatory blister due to toxic noxa, inflammatory, allergic reactions, etc.), bulla mechanica (due to mechanical injury in epidermolysis), bulla actinica (due to exposure to the sun, e.g. hydroa vacciniforme), bulla spontanea (e.g. pemphigoid). Compare with efflorescence (Pschyrembel 2012, p. 328).

1.1.2 Epidermolysis (; ; lys-*) f:

Separation and blister development in the dermoepidermal zones (e.g.e. bullosa acquisita, forms of e. bullosa hereditaria and bullous pemphigoid) or intra-epidermal (e.g. pemphigoid vulgaris) (Pschyrembel 2012, p. 597).

1.1.3 Epidermolysis bullosa (EB):

EB is a hereditary skin condition in which the skin of the affected person is liable to blistering. The skin can be as sensitive as the wing of a butterfly.

In this rare congenital condition there is a tendency for the skin to blister on account of minor injury. Sometimes, pressure or mild friction alone is sufficient to cause blistering. Depending on the form of the condition, this blistering can also affect the mucous membranes, especially in the mouth and the oesophagus.

The cause of this fragile skin and mucous membranes is a mutation in certain genes. The mutations are in the genes of the structural proteins of the dermoepidermal basement membrane zone (subepithelial). Normally, these structural proteins hold the layers of the skin together, but in this case they are deficient or defective in their function. Depending on the form, number and localisation, etc., there can be a very wide spectrum of possible clinical forms, which can vary from single blisters on the soles of the feet or palms of the hand to a generalisation over the whole body and the development of serious systemic complications. The clinical picture, progress, prognosis and course as well as the treatment can vary considerably. A causal treatment does not exist at present (cf. Laimer et al. 2008; Netzwerk EB 2009).

1.2 Pathology of EB

In principal, the classification of the three main forms of hereditary EB is done according to the level of blister formation in the skin.

• Epidermolysis bullosa simplex (EBS) with fissure formation in the basal keratinocyte layer
• Junctional EB (JEB) with separation in the lamina lucida along the basement membrane
• Dystrophic EB (DEB) with dermolytic coherency dissociation below the lamina densa of the basement membrane (cf. Laimer et al. 2008; Netzwerk EB 2009)

The Blistering of EB
Basement membrane zone	Localisation of the blister
Fig. 1.1 Site of the blistering of EB (Waldhr)

In each of these main types there are many subdivisions (see p. 69).

Apart from the hereditary forms there is also an acquired form: EB acquisita. This is an autoimmune condition which is extremely rare (cf. Laimer et al. 2008).

1.2.1 EB Simplex (EBS)

EB simplex is the most common form of the condition. It is almost completely autosomal dominant inherited. The progressive form is usually mild; in childhood there is usually more blistering later, but the tendency remains lifelong. The healing of the blisters which develop intra-epidermally is normally without scarring or skin atrophy. Blistering happens mostly on the exposed areas, such as hands, feet, elbows, knees, etc., and is usually more during the summer months. Nails, mouth, mucous membrane and teeth are not affected, as can happen in other forms of EB (cf. Netzwerk EB 2009; EB Info World 2012).

Fig. 1.2

a Infant with EBS (Hametner). b Hand of a child with EBS (Hametner)

1.2.2 Junctional EB (JEB)

This form of the condition is inherited autosomal recessively. Here there is a separation along the lamina lucida of the basement membrane, so that there is no cohesion between the epidermis and the dermis.

Depending on the severity of JEB, the progress can vary between minimal blistering and life-threatening limitations of skin function. Usually, the blisters heal without scarring, but due to secondary infections scarring can occur, and this may lead to atrophy of the skin in further progress of the condition. Typically, this skin atrophy develops on hands, elbows, feet and knees, and there may be dystrophic changes to the nails. Anomalies of the teeth, alopecia and pigmented (birthmark) may also occur (cf. Netzwerk EB 2009; EB Info World 2012).

Fig. 1.3

a Blisters on the leg of a child with JEB (Hametner) b Infant with JEB (Hametner)

1.2.3 Dystrophic EB (DEB)

This form of the condition is mostly inherited autosomal recessively. Usually there is scarring because the blistering is below the lamina densa, where the blood vessels and nerves are. Because of this the blisters can be very deep, contain blood and are painful. The blistering occurs as a result of injury, but it appears that sometimes it may be spontaneous. The clinical signs may vary considerably.

Scarring, milia (small white cysts) and nail dystrophy, and/or loss of nails are typical in this form of the condition. Usually, a flexor contracture of the fingers and a restriction of the abduction of the thumb develop.

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