Chapter 16 Liz Gormley‐Fleming This chapter provides an overview of the anatomy and physiology of the skin. The chapter introduces the reader to altered pathophysiology and the impact this may have on the child, young person and family. The condition of the skin can often be the first sign of an underlying health issue. The skin is the largest organ in the human body. It has several vital functions that are essential to sustain life. The skin, also known as the integumentary system, is a complex organ that is essential for human survival due to it physiological functions. It undergoes significant changes from birth to adulthood, such as thickening of the dermis and increased activity of the sebaceous glands. The most dynamic changes occur within the first 3 months of life (Hoeger & Enzmann, 2002). At birth the skin of a term baby is developed to cope with extra‐uterine life. As a system, the skin has contributions from basic germ layers: the ectoderm and the mesoderm. The ectoderm forms the surface epidermis and the associated glands while the mesoderm forms the underlying connective tissue of the dermis and subcutaneous layer (Chamley et al., 2005). It is also populated with melanocytes and sensory nerve endings. These different tissues perform many specific functions: thermoregulation, synthesis of vitamin D, excretion, and immunity. Frequently referred to as the largest organ in the body, the skin covers all of the body’s external surfaces and is approximately 10% of the body mass. By adulthood the skin will be almost 2 square meters. The ratio of skin surface to body weight is highest at birth and this will decline progressively during infancy. At birth, the surface area is nearly three times greater than that of an older child, whereas at 37 weeks’ gestation or less, it is proportionally five times greater than that of a term baby (Wong, 1999). The skin is the first line of defence against the environment. Skin disorders of childhood vary greatly in both symptoms and severity. They can be permanent or temporary, painful or painless, minor to life‐limiting. They are frequently associated with viral or bacterial infections, for example, slapped cheek syndrome, meningococcal septicaemia. Some of the more common conditions are identified in Table 16.1. Empirical evidence has demonstrated that loss of skin integrity in infants and children is most commonly attributed to wounds secondary to congenital conditions, thermal injury, extravasation injury, epidermal stripping and pressure ulcerations (McCullough & Kloth, 2010). Table 16.1 Skin conditions of childhood Disorders of the skin are normally described by the type of lesions that appear on the skin, the shape of the lesion, the colour and the configuration. Noting the type, shape, location and colour of the lesion is an essential component of obtaining a history of the rash or skin condition. Therefore it is important to be able to describe accurately the details of any rash an infant or child presents with, for example, a generalised, macular erythematous rash, hot to touch. Lesions may be described as either primary or secondary. Epidermolysis bullosa (EB) is an inherited disorder that occurs in 1 : 17 000 children in the UK. It is a group of skin‐blistering conditions and it is characterised by blisters, skin breakdown, pain, deformity, infection that can lead to secondary complications and an increased risk of squamous cell carcinoma (Watson, 2016). The epithelial lining of other organs may also be affected and blister as a result of minimal trauma. A lifelong condition, the child with EB will require lifetime healthcare input. Epidermolysis bullosa is generally an inherited autosomal dominant disorder in that it relies on only having one affected parent for transmission. If a parent is a carrier, there is a 50% chance of having a child from each pregnancy who will inherit EB. Males and females are affected equally. It may also be autosomal recessive so each parent would carry the affected gene and the risk of having a child born with EB would be 25% for each pregnancy. It may also occur as a new disorder with no parent carrying an affected gene by genetic mutation. Diagnosis is by immunohistochemistry (Tenedini et al., 2015). A definitive diagnosis will require a skin biopsy, possibly imaging and endoscopy if there is thought to be involvement of the gastrointestinal tract (Bruckner‐Tuderman et al., 2014). This is essential even in the presence of a complete history and assessment in order to prescribe the correct plan of treatment. Blood sampling for genetics may be obtained. Amniocentesis may be performed for prenatal diagnosis as early as the 10th week of gestation (National Institute of Arthritis and Musculoskeletal and Skin Diseases [NIAMS], 2013). There are various types of epidermolysis bullosa ranging in severity from mild to life‐limiting. Currently there are more than 30 types of EB so categorisation is ongoing as research develops in this area. International consensus was agreed in 2008 on the currently used names. Classification is dependent on the layer of skin at which blistering occurs (Fig. 16.1), as follows: Figure 16.1 Cross‐section of the skin and primary sites of blister formation. Epidermolysis bullosa simplex (EBS) is a generalised form and is usually present at birth with blisters occurring primarily on the hands and feet. Blistering may be more widespread in subtypes of EBS. The cells of the epidermis are normally supported by keratins, which give the cells their shape and support. The keratin, usually type 5, type 14, or rarely plectin, is absent or present in insufficient amounts. In the presence of friction the cells rupture causing fluid to leak, which forms a blister. Finger and toenails may be absent. Blisters may occur inside the mouth. There may be thickening of the skin on the palms of the hands and soles of the feet. Anaemia may be present. In general the blisters are small but plentiful and approximately 2 cm in size. EBS accounts for 70% of cases (British Association of Dermatologists [BAD], 2015). Junctional epidermolysis bullosa (JEB) is usually severe and the child will have large blisters on its face, trunk and legs. It can be life‐threatening because of the risk of infection and fluid loss. Blisters may also be present in the upper airway, oesophagus, lower intestinal system and urogenital system. This may lead to life‐threatening episodes. Absent finger and toenails may be in evidence and the skin may have a very thin appearance (atrophic scarring). Alopecia may occur following blistering of the scalp. The child will have delayed growth secondary to malnutrition. Anaemia is common. Involvement of soft tissues of the nose and mouth will be identified. JEB accounts for 5% of all cases, (BAD, 2015). Dystrophic epidermolysis bullosa (DEB) occurs in approximately 25% of cases (BAD, 2015). This may be either the autosomal dominant or recessive forms of DEB. In the dominant and mild recessive forms, blisters will be present on the hands, feet, elbows, and knees. There will be milia present on the trunk and limbs. The oesophagus is usually affected. Nails will be present but misshapen. In severe DEB there will be blisters over large areas of the body surface. In addition, there will be loss of nails, pruritus, scarring, anaemia and delayed growth. There may be inflammation of the eyes and corneal erosion. The gastrointestinal tract and the oral cavity are likely to be affected. Early loss of permanent teeth occurs. Pseudosyndactyly – fusion of the fingers and toes – will affect most suffers of this type of DEB. Squamous cell carcinoma has been reported in young people with recessive DEB (NIAMS, 2013). Kindler syndrome is a rare type of EB. In addition to blistering, there are changes to the appearance of the skin – poikiloderma (breakdown of the skin) – and involvement of the gastrointestinal tract and the eyes. Hyperkeratosis of the soles of the feet and the palms of the hands will occur. Kindler syndrome is usually diagnosed by the age of 1 year, with the presence of blisters on the hands and feet being the initial cause of concern. By the age of 5 years, the skin will be considerably thinner and more wrinkled than that of an unaffected child. It occurs as a result of a defect in the gene KIND1 (also called the FERMT1 gene) and is an autosomal recessive disorder (Genetic Reference, 2016). Children with Kindler syndrome are more susceptible to skin damage from sunlight so treatment plans and education on safety in the sun need to be provided. Cytolysis, which is the destruction of cells by either rupture or disintegration, occurs. This causes blisters to form in the epidermis or basement membrane. It is the result of a genetic mutation, which affects the protein at the epidermal–dermal junction. In EBS, cytolysis leads to blister formation in the basal or spinous layers of the epidermis. The keratinocytes have abnormal keratin filaments and they may also be structured inadequately. In JEB, there is separation of the epidermis from the basal lamina. The protein structures, hemidesmosomes, which are thread‐like fibres, are absent or reduced; thus the epidermis is not firmly anchored to the base membrane. As a result of this, the tissues separate and blistering occurs in the upper area of the base membrane, the lamina lucida. In cases of DEB, the basal lamina and the epidermis are attached but a blister cavity forms below the lamina densa of the dermo–epidermal junction (Nettina, 2010). The hemidesmosomes (anchoring fibrils) are abnormal, reduced in number, or completely absent. As he is pyrexial the paediatrician arranges for Harry to have blood drawn for a full blood count and C‐reactive protein level; the results are shown in Table 16.3. Table 16.3 Harry’s blood test results A holistic, systematic assessment is required based on the individual’s needs. Care will be planned and implemented in accordance with findings. In addition to treating any infections, the aim of care should be to prevent any further trauma and blistering to the skin by any of the care interventions. The pain score will indicate the type of analgesia required. This will need to be reassessed on a regular basis and the appropriate analgesia administered as required. Opiates are frequently used to control pain and prior to change of dressings. Appropriate monitoring will need to be considered to ensure the safety of the child. There is a very high probability that a secondary infection is present so antibiotics will need to be prescribed. These may be oral, usually a penicillin‐based antibiotic or clarithromycin if there is a known allergy to penicillin (BNFC, n.d.), or topical antibiotics may be prescribed. The choice of oral or topical antibiotics will be determined by the clinical assessment. A swab of the infected area is required for culture and sensitivity. This will identify the organism and indicate if the antibiotics prescribed are suitable. Hydration is important when there is a loss of skin integrity and fluid loss from blisters. Oral fluids should be encouraged. A petroleum‐based product should be used to protect the lips. If oral hydration is not possible, intravenous fluids will be required. Utmost care is required when inserting a cannula; use of a tourniquet is not recommended. Adhesive dressing must not be used to secure the cannula once in situ. Because of the presence of blisters in the mouth and oesophagus, oral intake may be insufficient. A naso‐gastric tube should not be used. The presence of a naso‐gastric tube will cause additional blistering both on insertion and while in situ. The dietician is an integral member of the care team, and will usually suggest high calorific and protein‐fortified drinks and food. These will replace the protein lost from the fluid that drains from the blisters. Nutritional supplements may also be prescribed, such as minerals and vitamins. The dietician can also advise on foods and fluids that will prevent and assist with constipation. The objective of care is to reduce pain and prevent discomfort, excessive loss of body fluid, and infection, and to promote healing. If not lanced, blisters will continue to enlarge (Abercrombie et al., 2008). Apply very gentle pressure with soft gauze to the blister. A sterile hypodermic needle 14 or 16 gauge is inserted at the lowest point of the blister, and passed through it to create an exit point. Fluid will then drain from the blister. Heavy drainage from the blisters can irritate the skin further so it is important that this is absorbed and removed. If the roof of the blister is intact then leave it exposed. The needle should not be resheathed prior to disposal. Parents/carers are generally educated to pierce their child’s blisters in the home environment. Daily cleaning of the skin is important. The skin should be patted, not rubbed. Using antiseptic washes is thought to help reduce the risk of contracting skin infections (BAD, 2015). Adding salt to bath water is also beneficial in reducing pain, infection and odour (Thomas, 2010). The amount of salt in the water has not been determined and is not of significance. Clothing should be soft, loose and made of natural fibres. Overheating should be avoided. Shoes should be well fitting and made of natural material. Dressings must be non‐adherent (silicone‐based) and cut to size. Hydrogel dressing will assist with cooling blistered areas. Dressings should be secured using a tubular bandage or silicone‐based tape. Dressings must be changed when strike‐through occurs. If silicone tape is in situ it should be rolled back. An appropriate dressing removal spray should be used or the dressing should be soaked off in a bath. Fingers and toes should be dressed individually (Denyer & Murrell, 2010).
Disorders of the skin
Aim
Introduction
Congenital/present at birth
Infections
Acquired/trauma
Epidermolysis bullosa
Cellulitis
Surgery
Haemangioma
Herpes simplex
Burns
Eczema
Rubeola (measles)
Epidermal stripping
Café au lait spots
Verrucas
Pressure ulcerations
Port wine stains
Necrotising fasciitis
Blunt force trauma
Mongolia blue spots
Impetigo
Nappy rash
Lymphatic malformations e.g., cystic hygroma
Molluscum contagiosum
Melanoma
Albinism
Pilonidal sinus
Acne
Vitiligo
Shingles
Urticaria
Ringworm
Friction
Chicken pox
Sunburn
Type of lesion
Primary lesions
Secondary lesions
Colour
Shape
Arrangement
Epidermolysis bullosa
Diagnosis
Types of epidermolysis bullosa
Epidermolysis bullosa simplex
Junctional epidermolysis bullosa
Dystrophic epidermolysis bullosa
Kindler syndrome
Pathophysiology
Test
Harry’s results
Normal range
White blood cells (WBC)
16.4
4.5–13.5 WBC × 109/L
Neutrophils
9 × 109/L
1.5–8 × 109/L
Lymphocytes
8.4 × 109/L
1.5–7.0 × 109/L
Red blood cells (RBC)
4.8 × 1012/L
4.0–5.2 × 1012/L
Haemoglobin (Hb)
11.1 g/100 mL
11.5–15.0 g/100 mL
Platelets
240 × 109/L
150–450 × 109/L
C‐reactive protein
25 mg/L
<5 mg/L
Care and management
Pain
Infection
Hydration and nutrition
Blisters and skin care
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