Assessing skin disease in children

7
Assessing skin disease in children Rachel Frost Jenny Hughes Abstract Children frequently present with either a skin rash or skin lesion. It is important to be able to differentiate between well children and those that are systemically ill. A systematic approach with a comprehensive history and examination, along with appropriate investigations, enables clinicians to reach diagnosis. To support this approach a number of exam- ples have been given. Keywords child; diagnosis; differential diagnosis; examination; investi- gation; skin Skin disease is common in children. Primary skin complaints account for up to 24% of first visits to a general paediatric clinic. The presence of any rash or lesion on the skin of a child raises concerns that may range from cosmetic appearance to suspicion of an underlying systemic pathology. Apart from acting as a physical barrier the skin plays a role in temper- ature regulation, fluid balance and activation of the immune system. The aim of this article is to provide a descriptive and diag- nostic framework for clinicians presented with a child with a skin condition. Although the skin is visually very accessible it is a complex organ and the appearance of a rash or lesion is influenced by the location of the pathology within the skin. Reviewing the skin structure aids in understanding of common descriptive terms (Figure 1). Generally, involvement of the epidermis results in altered surface markings, scale, vesicles and crust, whereas involvement of the dermis alone results in normal surface markings. Both epidermis and dermis may be involved, and primary lesions can evolve into secondary lesions. This may be through the natural history of the condition or through scratching or infection which may result in excoriation, scale and crust (Table 1). There are a vast number of different skin conditions and more than 8000 diagnoses are listed in the British Association of Dermatologists’ diagnostic index. Age may be pertinent to help determine likely causes of a skin rash or lesion, for example the well recognized rashes appearing in newborns. However an initial assessment of a child will distinguish between those children that are systemically well with a rash and those that are miserable, give a history of lethargy, anorexia and fever and appear ill on presentation. The likely diagnosis within each group is different. As within any field of medicine, history, examination and investigations are the cornerstone of further assessment, but certain features may be particularly relevant to skin disease. These are outlined below and illustrated in a number of clinical cases, highlighting the relevance of identifying certain features in the history or examination. History Timing of onset, duration and variation of any skin lesion or rash over time should be sought. With viral exanthems, the timing of the onset of the rash in relation to systemic symptoms is crucial to try and define a likely diagnosis as well as an account of the order of progression. For example, in measles there is a prodromal illness 3e4 days before onset of rash with conjunctivitis, runny nose and cough. At the time of presentation there may only be widespread rash but there may be a history of reddish purple macules on the face and shoulders which became confluent and spread downwards. Kopliks spots occur in the mouth for 24e48 h before the rash and may not have been noted. In contrast the rose pink macular rash on the trunk seen in Roseola Infantum appears after 3e5 days of high fever. Associated symptoms should be sought including fever, pain and itch. Itch may be marked in some conditions e.g. scabies, atopic dermatitis and lichen planus. Exposures may contribute to any rash such as drugs, contact with infections, allergens and pets, effect of sunlight and recent travel (particularly if considering insect bites and infections such as Lyme disease). Immunization history should be gained in all children as it may illustrate susceptibility to certain infections. Family history may identify recent illness in other family members e.g. chicken pox or similar symptoms e.g. itch from scabies or flea bites. It may guide diagnosis in inherited genetic disease and help for those conditions where there is a well recognized association e.g. family history of atopy. Quality of life assessment gives insight into the impact of the disease on the child, particularly on sleep, and school atten- dance. For certain conditions this will also provide a clue to the severity of the disorder e.g. eczema. Developmental history is of relevance due to recognition of the association of skin conditions with delay and behavioural prob- lems (particularly if there is a metabolic aetiology) or neurolog- ical symptoms such as seizures. Rachel Frost MBChB MRPCH FRACP is a Paediatric Specialty Doctor at the University Hospital of Wales, Cardiff, UK. Conflict of interest: none. Jenny Hughes MBChB FRCP is a Consultant Dermatologist at the Prince of Wales Hospital, Bridgend, Wales, UK. Conflict of interest: none. SYMPOSIUM: DERMATOLOGY PAEDIATRICS AND CHILD HEALTH 21:3 105 Ó 2010 Elsevier Ltd. All rights reserved.

Transcript of Assessing skin disease in children

SYMPOSIUM: DERMATOLOGY

Assessing skin disease inchildrenRachel Frost

Jenny Hughes

AbstractChildren frequently present with either a skin rash or skin lesion. It is

important to be able to differentiate between well children and those

that are systemically ill. A systematic approach with a comprehensive

history and examination, along with appropriate investigations, enables

clinicians to reach diagnosis. To support this approach a number of exam-

ples have been given.

Keywords child; diagnosis; differential diagnosis; examination; investi-

gation; skin

Skin disease is common in children. Primary skin complaints

account for up to 24% of first visits to a general paediatric

clinic. The presence of any rash or lesion on the skin of a child

raises concerns that may range from cosmetic appearance

to suspicion of an underlying systemic pathology. Apart from

acting as a physical barrier the skin plays a role in temper-

ature regulation, fluid balance and activation of the immune

system.

The aim of this article is to provide a descriptive and diag-

nostic framework for clinicians presented with a child with a skin

condition. Although the skin is visually very accessible it is

a complex organ and the appearance of a rash or lesion is

influenced by the location of the pathology within the skin.

Reviewing the skin structure aids in understanding of common

descriptive terms (Figure 1).

Generally, involvement of the epidermis results in altered

surface markings, scale, vesicles and crust, whereas involvement

of the dermis alone results in normal surface markings. Both

epidermis and dermis may be involved, and primary lesions can

evolve into secondary lesions. This may be through the natural

history of the condition or through scratching or infection which

may result in excoriation, scale and crust (Table 1).

There are a vast number of different skin conditions and more

than 8000 diagnoses are listed in the British Association of

Dermatologists’ diagnostic index. Age may be pertinent to help

determine likely causes of a skin rash or lesion, for example the

well recognized rashes appearing in newborns. However an

Rachel Frost MBChB MRPCH FRACP is a Paediatric Specialty Doctor at the

University Hospital of Wales, Cardiff, UK. Conflict of interest: none.

Jenny Hughes MBChB FRCP is a Consultant Dermatologist at the Prince of

Wales Hospital, Bridgend, Wales, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 21:3 105

initial assessment of a child will distinguish between those

children that are systemically well with a rash and those that are

miserable, give a history of lethargy, anorexia and fever and

appear ill on presentation. The likely diagnosis within each group

is different.

As within any field of medicine, history, examination and

investigations are the cornerstone of further assessment, but

certain features may be particularly relevant to skin disease.

These are outlined below and illustrated in a number of clinical

cases, highlighting the relevance of identifying certain features in

the history or examination.

History

Timing of onset, duration and variation of any skin lesion or rash

over time should be sought. With viral exanthems, the timing of

the onset of the rash in relation to systemic symptoms is crucial

to try and define a likely diagnosis as well as an account of the

order of progression. For example, in measles there is

a prodromal illness 3e4 days before onset of rash with

conjunctivitis, runny nose and cough. At the time of presentation

there may only be widespread rash but there may be a history of

reddish purple macules on the face and shoulders which became

confluent and spread downwards. Kopliks spots occur in the

mouth for 24e48 h before the rash and may not have been noted.

In contrast the rose pink macular rash on the trunk seen in

Roseola Infantum appears after 3e5 days of high fever.

Associated symptoms should be sought including fever, pain

and itch. Itch may be marked in some conditions e.g. scabies,

atopic dermatitis and lichen planus.

Exposures may contribute to any rash such as drugs, contact

with infections, allergens and pets, effect of sunlight and recent

travel (particularly if considering insect bites and infections such

as Lyme disease).

Immunization history should be gained in all children as it may

illustrate susceptibility to certain infections.

Family history may identify recent illness in other family

members e.g. chicken pox or similar symptoms e.g. itch from

scabies or flea bites. It may guide diagnosis in inherited genetic

disease and help for those conditions where there is a well

recognized association e.g. family history of atopy.

Quality of life assessment gives insight into the impact of the

disease on the child, particularly on sleep, and school atten-

dance. For certain conditions this will also provide a clue to the

severity of the disorder e.g. eczema.

Developmental history is of relevance due to recognition of the

association of skin conditions with delay and behavioural prob-

lems (particularly if there is a metabolic aetiology) or neurolog-

ical symptoms such as seizures.

� 2010 Elsevier Ltd. All rights reserved.

Figure 1

Descriptive terms

Term Appearance Example

Macule Flat, circumscribed skin discolouration. Not raised or depressed Freckle, flat naevus

Patch Macule more than 1 cm Port wine stain, vitiligo, cafe au lait patch

Papule Circumscribed, elevated non-vesicular, non-pustular,

less than 1 cm

Molluscum, lichen planus

Plaque Broad elevated disc shape more than 1 cm Psoriasis

Naevus sebaceous

Nodule Circumscribed, elevated, solid

Involves dermis, may extend into subcutis

Neurofibroma

Pilomatricoma

Nodular scabies

Wheal Local, superficial, transient oedema Urticaria

Vesicle Sharply circumscribed, elevated, fluid filled Herpes simplex virus, pompholyx

Bulla Vesicle more than 1 cm Bullous pemphigoid

Epidermolysis bullosa

Pustule Circumscribed lesion containing pus Folliculitis, acne

Erythema Redness due to increased skin perfusion

Erythroderma Severe inflammation of the skin with more than 90% of body surface

involved

Causes include eczema, pustular psoriasis and

toxic shock syndrome

Crust Collection of debris, dried serum and blood Impetigo

Erosion Partial focal loss of epidermis; heals without scarring Bullous impetigo

Ulcer Full thickness, focal loss of epidermis and dermis;

heals with scarring

Trauma

Scale Thick stratum corneum, either hyperproliferation or increased cohesion of

keratinocytes

Tinea corporis

Table 1

SYMPOSIUM: DERMATOLOGY

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Examination

Firstly a good light source is necessary. Examining all the skin,

hair, nails and oral mucosa can provide valuable information.

Accurate observation of a rash with associated features can limit

the likely number of causes. Assessment of skin disease in chil-

dren involves more than just examination of the skin. General

growth provides a base line assessment and may be impaired in

chronic disease or underlying genetic disorders. Examination for

lymphadenopathy is helpful in viral exanthems and chronic

infections. Hepato-splenomegaly may be relevant in some

metabolic conditions. Equally important is the appreciation that

the disease process identified in the skin may be manifested

elsewhere in the body e.g. vasculitis affecting the kidney or

gastrointestinal tract.

What can be seen? Most paediatricians are adept at deciding if

a rash is blanching or non-blanching. It is valuable to observe if

a rash is raised (papules/nodules/plaques), if there are epidermal

changes, if there are pustules, vesicles or bullae (the fragility of

which can be a reflection of the level of the split in the

epidermis). Colour may vary due to a variety of causes including

vasodilatation, bleeding into the skin (purpura), or pigment from

melanocytes. It is important to note that erythema may some-

times be more difficult to see in pigmented skin.

Where can it be seen? (site). Some rashes are more likely to be

seen on the face e.g. malar rash of systemic lupus eryth-

ematosus, whilst others characteristically occur on the trunk e.g.

pityriasis rosea. The Koebner phenomenon refers to the devel-

opment of typical lesions at the site of minor trauma and is

characteristically seen in psoriasis, lichen planus and planar

warts.

What is the distribution? Some diseases are more likely to

follow a symmetrical pattern, others may be more likely to occur

on the extremities, some lesions follow a clear dermatomal

pattern and areas of sparing may be relevant for example absence

of rash in an area not exposed to sunlight.

How are the lesions arranged? It is helpful to describe the

relationship of individual lesions to those nearby e.g. grouped,

linear, arcuate.

How much of the skin is involved (extent)? This becomes

particularly important if a child is erythrodermic and in danger of

skin failure.

Touching the skin can give further information and in some

cases can be diagnostic e.g. rubbing of a mastocytoma that can

result in urticaria and flare as a result of histamine release

(Darier’s sign), or the easy separation of the epidermis from the

dermis on lateral pressure (Nikolsky’s sign) noted characteristi-

cally in Toxic Epidermal Necrolysis.

Examination of the hair may not only yield an infestation

responsible for a patch of eczema on the neck but will hide the

scalp that may reveal for example psoriasis or a tinea infection.

PAEDIATRICS AND CHILD HEALTH 21:3 107

Hair itself may also be a clue to the underlying disease for

example sparse hair in ectodermal dysplasia.

Examination of the nails may identify a number of changes

associated with various conditions e.g. pitting seen in psoriasis

and alopecia areata or identify changes in the nail folds e.g.

telangiectasia seen in dermatomyositis.

Examination of the mouth. The oral mucosa may help define

a likely cause of a rash, whilst the absence of lesions in the mouth

may indicate that a desquamating condition is more likely to be

Staphylococcal Scalded Skin than Toxic Epidermal Necrolysis.

Investigations

Usually there is a limited requirement for investigations.

Skin swabs can be helpful to identify infections and guide

antibiotic sensitivities.

Skin scrapings and nail clippings can help identify fungal

infections.

Woods light is anultraviolet light sourceused to look for certain fungi

and corynebacterium (which cause erythrasma). Unfortunately, the

fungi currently commonly responsible for tinea capitis do not fluo-

resce.Woods light also emits purple light in the visible spectrum. The

purple light is absorbed by melanin and therefore hypopigmented

patches of vitiligo and ash leaf macules can appear more prominent.

Patch testing can be helpful where contact allergy (type IV) is

suspected.

Skin prick testing or measurement of specific IgE for Type I,

IgE mediated, allergy may be of benefit where there is a clear

history of an associated urticarial response but is not useful

routinely in chronic urticaria or eczema.

Skin biopsy is occasionally required to make a diagnosis with

the aid of histopathology and immunoflourescence. Tissue may

be sent for microbiological culture, helpful to identify unusual

infections particularly in immunosuppressed patients (Table 2).

Summary

A child presentingwith a rashmay cause concern for any doctor not

familiar with dermatological conditions. Children who are unwell

will need timely intervention and consideration of the need for

antibiotics or antivirals or stopping treatments which may be

implicated in the aetiology of the rash. In addition there may be a

need to attend to the issues of skin failure, including fluid balance

and thermoregulation. Conversely, well children can often be

treated symptomatically and referred for definitive diagnosis. At all

times the clinicianmust remain vigilant to an atypical patternwhich

may indicatemore significant pathology e.g. histiocytosis as a cause

of persistent nappy rash. Generally, a systematic approach with an

initial visual assessment to facilitate a detailed but targeted history,

followed by a thorough examination, will allow the clinician to

define the problem and respond appropriately. A

� 2010 Elsevier Ltd. All rights reserved.

Examples of common skin complaints in children

Disease Picture Key points in history Key points in

examination

(i) Children appearing well on presentation

Guttae psoriasis

Sore throat, either

concurrent or preceding

the eruption

(Streptococcal infection is

implicated in the

aetiology). Often there will

be a family history of

psoriasis and certain

drugs, including

antimalarials, may be

implicated. May be family

history of psoriasis.

Scattered,

erythematous, well

demarcated plaques.

Scaling should be

apparent, although may

be less marked in the

early stages. Located

particularly over the

trunk and proximal

limbs. May extend onto

the face, ears and scalp.

Pityriasis rosea

Characteristically, there is

a history of a herald patch

prior to the general

eruption which occurs in

crops every 2e3 days for a

period of approximately

10 days (sometimes

several weeks). Classically

there are no symptoms but

occasionally it is noted to

be itchy.

The herald patch is a well

defined red oval, usually

2e5 cm in diameter,

covered with fine scale

towards the edge but not

at the margin. The

general eruption consists

of pink papules that

spread out to produce

oval macules 1e3 cm in

size, with a characteristic

collarette of scale.

Distribution is truncal

and proximal limbs

(sometimes referred to as

T shirt and shorts).

Granuloma

annulare

Rash may have evolved

over previous weeks.

Largely asymptomatic,

may be mildly pruritic.

Small papules or

nodules, lesions may be

multiple, commonly

associated with extensor

surfaces of lower legs,

feet, fingers and hands

but other areas of the

body may be involved.

As the rash evolves

nodules are apparent in

an annular

configuration. The

overlying epidermis is

usually intact, however it

may be slightly red or

hyperpigmented.

The lack of epidermal

changes helps to

clinically distinguish this

condition from tinea

corporis.

(continued on next page)

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Table 2 (continued)

Urticaria Clear temporal

relationship may identify

cause (consider potential

physical causes e.g. cold/

pressure/sunlight/water/

exercise as well as recent

viral illness and food

allergens). Discriminate

between the duration of

the process (which may be

ongoing for days or

weeks) and the duration of

individual lesions, which

should last only 24 h. Itch

rather than pain is the

main symptom.

Pink, raised areas of

skin, with no surface

change, may be pale

centrally, often with a

surrounding red flare.

Individual lesions should

resolve within 24 h,

leaving the skin with a

normal appearance.

May be associated with

angio-oedema.

Scabies Intense itch which is often

present in other family

members (although may

be denied). Very young

babies do not scratch and

may just be miserable and

not feeding well. More

severe infestation with

crusted scabies is

associated with children

with Downs Syndrome as

well as those with

significant developmental

problems and

immunosuppression.

Linear burrows

characteristically found

in the web spaces,

instep and wrist. May

involve trunk and scalp.

Papules and nodules

accompany the

development of

hypersensitivity to the

mite and are located

around the axilla,

abdomen, thighs and

genital areas. They are

intensely itchy and may

persist for weeks after

the scabies mite has

been eradicated.

Secondary lesions with

infection and extensive

eczematization may

occur. Generalized

lymphadenopathy may

be present. Examine

other family members.

PAEDIATRICS AND CHILD HEALTH 21:3 109 � 2010 Elsevier Ltd. All rights reserved.

Table 2 (continued )

Kerion

Hair loss with mild redness

and scaling of scalp.

Possible treatment with

topical antifungal without

improvement. Other family

members may be involved.

Raised boggy plaque on

scalp, studded with

pustules. Large painful

occipital, post-auricular

and preauricular

adenopathy help to

distinguish from

seborrhoeic dermatitis

or bacterial folliculitis.

Erythema

multiforme

May be low grade fever

and malaise and previous

viral infection. Commonly

associated with herpes

simplex virus.

Target pattern on distal

extremities well

recognized. Can be

macular, papular or

urticarial.

(ii) Children appearing unwell on presentation

Staphylococcal

Scalded

Skin Syndrome

Irritable child, usually

under 5 years with fever.

Skin usually tender before

blistering.

May be apparent

infection localized to

conjunctivae, nose,

perioral region,

perineum or umbilicus

(sometimes occult

infection). Tender red

skin develops large

superficial fragile

blisters that rupture

easily. Blistering is

mediated by toxin and

can occur anywhere on

body but oral mucosa

spared.

(continued on next page)

PAEDIATRICS AND CHILD HEALTH 21:3 110 � 2010 Elsevier Ltd. All rights reserved.

Table 2 (continued)

Toxic Epidermal

Necrolysis

Child unwell for several

days with high fever. Drug

history important as often

implicated. Skin is tender.

Macules become confluent

and form flaccid bullae.

Tendency to start at the

head.

Child may be

hypotensive and

shocked. Sheet like

erosions involving more

than 30% of body.

Involvement of eyes,

mouth and genital

mucous membranes.

Impetigo

Severe forms associated

with fever, eczematous

skin more vulnerable to

infection.

Small blisters containing

pus easily rupture and at

the time of presentation

the dried crust of the

exudates is most

apparent. Involvement

of mucous membranes is

rare. Circular bullae that

rupture very easily are

seen in bullous

impetigo.

Herpes simplex

virus

Often acute deterioration

in a child with known

atopic dermatitis who is

unwell with a fever,

increased itch and

irritability. There may be a

history of cold sores or

herpetic whitlow in family

members. (The latter may

have initially been thought

to be localized

staphylococcal infection.)

2e3 mm vesicles,

punched out vesicles,

erosions and crusting.

Table 2

Practice points

C Initial assessment of children should identify those systemically

unwell and in need of resuscitation and immediate treatment.

C For diagnosis there is no substitute for detailed history and

thorough examination (including hair, nails and oral mucosa).

C Identification of the lesions seen and use of correct dermatolog-

ical terms are essential to formulate accurate differential diag-

noses and to communicate clearly and safely with colleagues.

SYMPOSIUM: DERMATOLOGY

FURTHER READING

Bernard A. Cohen MD. Pediatric Dermatology, 2nd Edn. Mosby

International Ltd, 1999.

Harper J, Oranje A, Prose N, eds. Textbook of pediatric dermatology.

Massachusetts: Blackwell Publishing, 2000.

Lewis-Jones Sue, ed. Paediatric dermatology (Oxford Specialist Hand-

books in Paediatrics). Oxford University Press, 2010.

Paller Amy S, Anthony J, Mancini MD. Hurwitz clinical pediatric derma-

tology: a textbook of skin disorders of childhood and adolescence.

Elsevier Health Sciences, 2005.

PAEDIATRICS AND CHILD HEALTH 21:3 111 � 2010 Elsevier Ltd. All rights reserved.