Anaesthesia, 1984, Volume 39, pages 443455

REVIEW ARTICLE

Anaesthesia and severe skin disease

G . B. S M I T H AND A. J. S H R I B M A N

Summary
A review of the anaesthetic management of severe skin disease is presented. Erythroderma, urticaria
pigmentosa, hereditary angioedema. epidermolysis bullosa, pemphigus, pemphigoid, the Stevens-Johnson
syndrome, Behcet 's syndrome, scleroderma, Ehlers-Danlos syndrome and congenital anhidrotic ectodermal
defect are discussed.

Key words
Skin.
Complications; skin diseases.

The majority of patients who present for surgery

with incidental skin disease cause no specific
problems for the anaesthetist. However, a small
group will be suffering from rare skin disorders
for which specialised management is necessary
to avoid morbidity and mortality. There are
several publications concerning the anaesthetic
management of patients suffering from indi-
vidual skin disorders but there appears to be no
recent collective review of these conditions.
An account is presented of the physiological
functions of the skin, including their modification
by anaesthesia, and the anaesthetic management
of conditions in which the skin disorder directly
affects the conduct of anaesthesia is reviewed.
Systemic disorders in which skin involvement
occurs but has no direct influence on the manage-
ment of anaesthesia are excluded.
Skin physiology and its modification by
anaesthesia
The skin consists of three layers, the epidermis,
dermis and subcutaneous tissue. Hair follicles,
sweat and sebaceous glands, nails, blood vessels,
lymphatics, nerve fibres, collagen, elastin, fibro-
blasts, mast cells, melanocytes and the erector
pili muscles all lie within it. Skin constitutes
approximately 3% of total body weight and has
an oxygen consumption of almost 0.3 m1/100
g/min.
Skin acts as a barrier, temperature regulator,
secretory and sensory organ. It also stores water,
is involved in the production of vitamin D,
secretes sexual attractants and displays emotions.
The skin forms an airtight, elastic, waterproof
envelope surrounding the body's internal organs

G.B. Smith, BM, FFARCS. Senior Registrar, Shackleton Department of Anaesthetics, Southampton General
Hospital, Tremona Road, Southampton SO9 4XY, A.J. Shribman, MB, BS. FFARCS. Senior Registrar,
Department of Anaesthetics, Leicester Royal Infirmary, Leicester.

OOO3-2409/84/050443 + 13 S03.00/0 @ 1984 The Association of Anaesthetists of Gt Britain and Ireland 443

444 G.B. Smith and A . J . Shribman
and protects them from trauma. A surface film
of water, lipids, amino acids and polypeptides
derived from sweat and sebaceous glands is
maintained at a pH of 4-6.7 and forms a n
effective antiseptic layer which retards the growth
of micro-organisms. Melanin pigment protects
the skin from ultraviolet radiation.
Skin plays a major role in the control of body
temperature. The skin blood flow determines its
temperature, allowing heat exchange to occur by
radiation, conduction and convection. Heat loss
from the evaporation of sweat is independent of
blood flow.
Basal skin blood flow is approximately 50
m1/100 g tissue/min but may reach 200 m1/100 g
tissuelmin to facilitate heat loss. Increased flow
through specialised arteriovenous shunts and the
loss of precapillary sphincter tone is responsible
for this increase and is controlled by the sym-
pathetic nervous system (SNS).
Heat loss by evaporation uses 0.58 kcal/ml of
water. Insensible water loss is due to diffusion
through the epidermis and accounts for approx-
imately 700 ml in 24 hours. The secretion of the
eccrine sweat glands accounts for the remainder.
These glands are supplied by cholinergic auto-
nomic nerves but will also respond to circulating
adrenaline and noradrenaline. In response to
stimulation they produce a hypotonic solution
containing water, sodium, potassium, glucose,
urea and lactate. Such glands are common over
the trunk, palms and face. The maximum sweat
capacity is 12 htresi24 hours but may reach 3
litres/hour for short periods. Conservation of
heat by pilo-erection is unimportant in humans.
Other secretory functions are performed by
apocrine and sebaceous glands. The former,
situated in the axillae, genital folds and around
the nipples, secrete small quantities of white oily
fluid containing protein and carbohydrate. It is
odourless until decomposed by bacterial action.
Secretion is stimulated by the SNS and circulating
catecholamines.
Sebaceous glands occur throughout the skin
with the exception of the palms and soles. They
secrete sebum, a complex mixture of fatty acids
and cholesterol, which controls moisture loss
from the epidermis and protects the skin from
infection.
Sensation is relayed from the skin by free and
corpuscular nerve endings of many types. None
has an absolute specificity but there is a high
degree of selective sensitivity to different stimuli.
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Anaesthesia may alter the physiology of the
skin in several ways.
The protective role is diminished as the loss of
sensory input resulting from either general or
regional anaesthesia will allow pressure sores,
nerve damage, diathermy burns and other trauma
to go unnoticed by the patient. Invasive tech-
niques may breach the integrity of the skin’s
bacterial defences and, in conjunction with poor
aseptic techniques, may predispose to infection.
Skin blood flow is increased by hypercapnia,
spinal and epidural anaesthesia, vasodilator
drugs and the loss of central vasomotor control.
The subsequent heat loss will cause the body
temperature to fall in cold surroundings because
the anaesthetised patient is unable to compensate
by shivering and increasing the metabolic rate.
Conversely, in a warm environment, the patient
tends to absorb heat through the dilated skin
vessels.
The use of anticholinergic drugs or spinal and
extradural anaesthetic techniques reduce sweat-
ing and may lead to hyperthermia. The inter-
ference with thermoregulation is exaggerated in
children because of the high ratio of surface area
to body volume.
The effects of anaesthesia on the skin’s other
physiological functions are unimportant.
Erythroderma
Erythroderma describes a condition in which
there is severe generalised scaling and erythema
of the skin. The commoner causes are psoriasis,
exfoliative dermatitis, drug reactions and the
reticuloses in which the widespread inflammation
may cause disturbances in the cardiovascular.
thermoregulatory and metabolic systems of the
body.
Total skin blood flow is approximately 1 litre/
min at 37°C; however, in erythroderma, this may
increase to 5 litre/min, reaching as much as 10
litre/min in the presence of pyrexia.’ Several
authors have described the resulting high output
cardiac This may be exacerbated by
iron or folate deficiency anaemia4 due to in-
creased skin cell turnover, hypoalbuminaemia
and hypercatabolism. Thus, non-urgent surgery
should be avoided in patients with such dis-
turbances but, if emergency anaesthesia is
required, the following points should be con-
sidered.
The hyperdynamic circulation may cause an
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Anaesthesia and severe skin disease 445

alteration in the speed of onset of both inhala-

tional and intravenous anaesthetic agents.
Hypervolaemia, hypoalbuminaemia and a reduc-
tion in renal blood flow' may be responsible for
alterations in the kinetics of drug distribution
and excretion. Surgical blood loss may be
increased because of the tachycardia and raised
venous pressure. Blood and fluid replacement
must be given with care to avoid further
embarrassment to the cardiovascular system.
Patients often have subnormal temperatures
due to the inability to vasoconstrict6,' and this
may be exacerbated by a cold operating environ-
ment. The use of space or water blankets to
protect against hypothermia requires careful
monitoring of skin and core temperature as
patients with erythroderma are unable to sweat
and may have a raised metabolic rate.3
Concurrent steroid therapy should be managed
appropriately.
Urticaria pigmen tosa
Urticaria pigmentosa is characterised by multiple
discrete rust-coloured maculopapular lesions
containing increased numbers of mast cells which
redden, swell and blister when rubbed (Fig. 1).
It is usually associated with mast cell infiltration
of the liver, bone and spleen and is then called
mastocytosis. Mast cell degranulation may occur
on exposure to trauma, temperature extremes,
biological polymers, alcohol, bacterial toxins and
drugs. The release of histamine, heparin and
other vasoactive substances may cause general
malaise, pruritus, headache, flushing, tachy-
cardia, syncope and cardiovascular collapse.
Systemic deposits of mast cells may cause
osteoporosis, marrow suppression and spleno-
megaly. Clotting factors may be reduced due to

The urticarias
Urticaria results from a transient increase in
capillary permeability causing focal oedema of
the dermis. Urticaria1 wheals are mediated by
many vasoactive substances but histamine
release is responsible for most of the common
urticarias. About 80% of cases are idiopathic and
in approximately half of these angioedema of the
face may occur. A small number may also develop
abdominal symptoms which may mimic an acute
abdomen. Exposure to heat, cold, pressure, drugs
or insects is responsible for the majority of other
presentations. However, two rare variants are
urticaria pigmentosa and angioedema.
The anaesthetic management of patients suf-
fering from the common urticarias is based on
the avoidance of trigger factors. The following
general points should be noted. It would seem
prudent to avoid all histamine-releasing drugs
(see below). Deep pressure urticaria may occur,
particularly on the buttocks and feet, so that
extra protection of these areas is needed.
Tourniquets should be avoided if possible.
Cold urticaria is precipitated by a cold en-
vironment or direct contact with a cold substance.
Care should be taken to warm laryngoscope
blades and any intravenous fluids. Warming
blankets should be considered. Immune urticaria
may follow exposure to drugs such as penicillin Fig. 1. The multiple discrete rust coloured maculo-
and drugs known to cause allergy should be papular lesions of urticaria pigmentosa contain in-
avoided. creased numbers of mast cells.
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446 G.B. Smith and A . J . Shribman

the combined effects of hepatic fibrosis and

malabsorption of fat soluble vitamins secondary
to intestinal lesions.8 Rarely, endogenous heparin
may cause a prolonged prothrombin time9 and
this can be reversed with protamine sulphate.1°
Pulmonary infiltrations may occur but d o not
usually affect lung function., I
There are several reports of anaesthesia'O. 1 2 - l 3
in patients with mastocytosis including one fatal
case. l O The extent of skin lesions and the presence
of bruising, intra-oral lesions and pathological
fractures should be noted. Tests should include
a clotting screen and a full blood count to detect
marrow suppression. Premedicant drugs which
may release histamine such as and
atropine12 should be avoided, but diazepam has
proved safe. ' 2. ' Prophylactic antihistamines
have been suggested.* The pre-operative use of
disodium cromoglycate, successful in the treat-
ment of chronic mastocytosisg*l 4 would seem
reasonable but has yet to be confirmed. Steroids
are rarely beneficial I O but should be considered
in severe cases.8*9
Anaesthetic agents known to release histamine
such as morphine derivatives, tubocurarine,
gallamine and atropine should be a v ~ i d e d . ~ .
The following would appear to be safe; metho- Fig. 2. Acute swelling of the eyes and lips in a child
hexitone, pethidine, diazepam, suxamethonium with hereditary angioneurotic oedema. Similar changes
and the inhalational agents. 2 , 3* 5 * Mucosal may occur as a result of chronic urticaria or allergic
lesions may make intubation hazardous. During drug reactions.
anaesthesia, urticarial triggers such as cold, heat
and friction should be avoided. Transfusion
reactions are usually serious; however, if blood upper airway obstruction, is common in the
transfusion is essential, the blood should be immediate family of known sufferers.I Oedema
warmed. It is suggested that regional anaesthesia of both the larynx and pharynx has led to death
is superior to general anaesthesia,8 but there in 15-33% of cases. l a The pathological condition
is one report of a severe urticarial reaction is due to a deficiency of C,-esterase inhibitor.lg
following a Bier's block.' Hypotension resulting Cl-esterase is the enzyme responsible for the
from histamine release should respond to alpha- activation of the first component of the comple-
and beta-sympathomimetic agents, especially ment cascade. A small group of sufferers may
adrenaline. possess normal levels of a biologically inactive
C,-esterase inhibitor. This real or relative
deficiency leads to an uncontrolled activation of
Hereditary angioedema
the complement cascade with the formation of
Angioedema describes swelling of the eyes, lips kinins and vasoactive peptides. Attacks are often
and mouth secondary to chronic urticaria, an precipitated by trauma which may initiate
allergic drug reaction or an inherited defect. fibrinolysis by activation of the Hageman factor
Hereditary angioneurotic oedema (HAO) is and promote the conversion of plasminogen to
transmitted as an autosomal dominant trait. It plasrnin.2O Plasmin is a potent activator of C1
is characterised by episodic acute swelling of the and the subsequent initiation of the complement
eyes, lips and mouth and, occasionally, the skin, cascade will lead to increased vascular per-
subcutaneous tissues and bowel (Fig. 2). A meability and tissue oedema.18
history of sudden death, usually secondary to There are several reports of anaesthesia for
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Anaesthesia and severe skin disease 441

patients suffering from HA0.2'-27 Fatal airway

obstruction may result from an acute attack and
dental surgery is associated with particular risk.26
The anaesthetic management is based on the
prevention of acute attacks for which steroids
and antihistamines are ineffective but cinnari-
zine, androgens,2',28 epsilon aminocaproic
acid,ZZ.Z3.29-31 and tranexamic acid'8.20.3'have
all been used with some success but may be
unreliable. In theory, the treatment of H A 0
should involve replacement of the deficient C,
esterase inhibitor; however, purified concentrates
are expensive and have a short half-life.32
Fortunately, fresh frozen plasma (FFP) is effec-
tive in both the prophylaxis and treatment of
HA0.17.24.26.27.33 Two units of FFP given in
the immediate pre-operative period will restore
the C,-esterase inhibitor to a safe level for
between 1 and 4 days.24,27.33FFP will also
control acute attacks of angioedema within 45 Fig. 3. Epidermolysis bullosa is characterised by
minutes of infusion.33 Some author^^',^^ have frictional separation of the epidermis. Bullae and
scarring are common on the hands and feet.
expressed concern that F F P also supplies the
complement fractions necessary to fuel an acute
attack; however, this does not seem to be a
problem.26.27J33 Subcutaneous adrenaline, 1 mg/ frictional separation of the epidermis by a split
hour, has been of use in threatened airway at the lower epidermis or at the demo-epidermal
o b s t r ~ c t i o n ;however
~~ the intravenous route junction (Fig. 3). Of the many forms, epidermo-
may be preferable. lysis bullosa dystrophica (EBD) is the one of
Despite adequate prophylaxis, the anaesthetist anaesthetic importance.
should still meticulously avoid trauma to the EBD is transmitted as an autosomal recessive
larynx and trachea. Tracheal intubation and disease and has an incidence of approximately
throat packs should be avoided, if possible. 1:300000 birth^.^^,^^ Its onset is from birth or
Facilities for emergency tracheostomy should be early infancy and it is characterised by extensive
available as the passage of even a small tracheal bullous and dystrophic lesions. The bullae, which
tube may be impossible during an acute attack. may develop spontaneously or at the sites of
During the postoperative period, patients should friction or trauma, are large and flaccid, ofien
be monitored in a well staffed recovery area or become infected and may be haemorrhagic.
intensive care unit. These heal by scar formation which may cause
severe deformity such as pseudosyndactyly.
Abnormalities of the nails, conjunctiva, hair and
Bullous diseases
teeth may occur. Lesions within the mucous
Blistering is caused by either impaired adherence membranes of the mouth, pharynx and oeso-
between the layers of the skin or mucous phagus may lead to feeding difficulties, fixation
membranes, or excessive disruptive forces applied of the tongue to the floor of the mouth and
to them. The blistering diseases which may cause oesophageal stricture. Death often occurs before
anaesthetic morbidity or mortality are epi- the age of 20 but in survivors the condition may
dermoloysis bullosa. pemphigus, pemphigoid improve with time.
and the Stevens-Johnson syndrome. Epidermolysis bullosa lethalis is the other reces-
sive form of epidermolysis bullosa, and survival
Epidermo1.vsis bullosa
is rare beyond 3 months. There are two dominant
forms with normal life expectancy in which bullae
Epidermcl. ' hullosa is the name given to a are usually confined to the hands and feet.
group of inherited disorders characterised by Healing occurs without scar formation, the

448 G.B. Smith und A . J . Shribmun
general health is good and the disease tends to
improve after puberty.
Anaesthesia may be required in patients with
EBD for correction of pseudosyndactyly or
oesophageal stricture, removal of scar tissue,
dental treatment or incidental surgery.37 The
risk of bullous formation during surgery should
be reduced by various modifications of standard
anaesthetic techniques.
At the pre-operative visit the extent of the
disease should be assessed. Epidermolysis bullosa
may be associated with malnutrition, anaemia,
electrolyte imbalance, renal failure and
a m y l o i d ~ s i s3 .8 ~ ~Patients
~ may be receiving
steroids. A good rapport with the patient will
lessen the chance of struggling at induction or
recovery, and consequent blistering.38.3 9
Intramuscular premedication should be
avoided because bullae may form a t the site of
injection. Oral premedication is suitable; how-
ever, some authors prefer no premedication so
that patients are able to transfer themselves on
to the operating table, thus avoiding trauma.40
Theatre and recovery room staff, porters and
other personnel must be aware of the dangers of
trauma to the ~ a t i e n t . ~Adhesive ~,~ strapping
or diathermy pads, electrocardiogram (ECG)
electrodes and precordial stethoscopes may all
cause skin Padding beneath a blood
pressure cuff is essential to reduce the shearing
forces on the arm. Intra-arterial monitoring may
be justified for longer p r 0 c e d ~ r e s . j ~Non-
adhesive ECG plates or intradermal needle
electrodes are suitable. Bland, sterile ointment
should be used instead of tapes to protect the
eyes. The sheets beneath the patient should be
free from creases, and foam padding should
protect the heels and elbows. Intubation, suction
and other instrumentation of the oropharyx must
be gentle and kept to the minimum. Bleeding
from ruptured mucosal bullae may be treated
with sponges soaked in 1 in 200000 adrenaline.44
Several techniques of anaesthesia for patients
with EBD have been epidural or
spinal anaesthesia may be justified as long as the
skin on the back is free from lesions or
i n f e ~ t i o n . ~Infiltration
' anaesthesia is contra-
indicated because of the likelihood of skin
~ l o ~ g h . ~Ketamine . ~ ~ . has
~ ~been
* ~ used
~ on
several occasion^.^^^^^^^^ It has the advantages
of a rapid smooth induction and maintenance of
laryngeal reflexes with the avoidance of face-
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masks and oral airways. Ketamine can be given
intramuscularly if scar tissue makes intravenous
injection impossible.
If inhalational anaesthesia is required, airway
management may be difficult because of scarring
and fixation of the tongue.41 Facemasks covered
with soft cotton wadding or muslin and
lubricated with 0.5% hydrocortisone cream and
gel have been used.46 Petty and G ~ n t h e have r~~
described a technique in which anaesthetic gases
are passed into a polythene hood enclosing the
patient's head. The hood was loosely tied around
the neck and kept distended by altering the fresh
gas flow and size of exhalation port. It was
applied after a gas induction using a high gas
flow with the mask held just above the face.
Expired carbon dioxide was measured through-
out the procedure. The hood could easily be
removed in the event of airway obstruction.
Intubation may be unavoidable in certain types
of surgery or because of airway difficulties.
Disuse atrophy of the patient's muscles and
extensive tissue injury could possibly cause a
hyperkalaemic response to ~ u x a m e t h o n i u m . ~ ~ . ~ "
Fasciculations, following suxamethonium, may
cause limb movements with resultant skin
damage. Paralysis should be profound to aid
atraumatic intubation; however, there may be an
unpredictable sensitivity to non-depolarising
muscle relaxants.42 At laryngoscopy care should
be taken t o avoid damage to severely carious
teeth, which is a feature of EBD. A Macintosh
laryngoscope blade is preferred to the Magill
blade which may traumatise the posterior surface
of the epiglottis resulting in supraglottic oedema
and o b s t r ~ c t i o n . ~ ~
The tracheal tube selected should be smaller
than usual to minimise contact with the laryngeal
inlet. It should be tied with a soft flannel bandage.
The surgeon should be prepared to perform a
tracheostomy if airway obstruction occurs.
It was believed that there was a close associa-
tion between EBD and porphyria. It now appears
that patients with presumed EBD affecting only
the skin have porphyria cutanea tarda (PCT)
which may be latent without porphyrinuria.
Diagnosis is therefore difficult. Barbiturates and
other enzyme inducing drugs should be avoided
in these patients. As PCT only manifests itself in
skin exposed to sunlight, the presence of mucosal
lesions excludes this d i a g n o s i ~ . ~ "
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Anaesthesia and severe skin disease 449

Fig. 4. Bullous formation on the chest of a woman with pemphigus vulgaris. Similar
lesions may co-exist in the mouth.

Fig. 5. A large bulla on the forearm of a woman with pemphigoid.

Pemphigus vulgaris and pemphigoid

Pemphigus is characterised by bullous formation
in the skin and mucous membranes, particularly
of the mouth, conjunctiva and genitalia (Fig. 4).
There is impaired epidermal cell adhesion and an
associated immune reaction to the intercellular
cement. Pemphigus may be associated with
myasthenia gravis and thymomas. Eruptions
may occur spontaneously or may be precipitated
by trauma, stress, allergies, drug reactions or
infection.52 Mortality is greater than 90% with-
out treatment, with death occurring within 1 year
of onset. Steroids have dramatically improved
survival.
Pemphigoid, a disease of the elderly, differs
from pemphigus in that the blisters are sub-
epidermal and tend to heal (Fig. 5). The condition
is self-limiting and the mortality is lower.
Both diseases closely resemble the oral
manifestations of EBD and the anaesthetic
management is the same.47 The use of a slow,
intravenous infusion of ketamine and diazepam,
thus avoiding the need for an oral airway or
tracheal tube, has been d e s ~ r i b e d . ~ '

450 G.B. Smith and A.J. Shribman
Stevens-Johnson syndrome
The Stevens-Johnson syndromes4 is an extreme
form of the spectrum of disease known as
erythema multiforme, in which there is extensive
acute inflammation of the skin and mucous
membranes. Its incidence is unknown although
it is more common in children,ss and mortality
is between 3 and 18%.56,s7Viruses, bacteria,
irradiation and a wide variety of drugs may be
causal factors.ss*s 6 * s 8
The skin is inflamed with macules, papules,
vesicles and bullae. Secondary infection causes
sloughing. Lesions may occur throughout the
body with involvement of the eyes, kidneys and
gut. Ulceration within the respiratory system is
common and may result in purulent sputum,sg
pleural effusions and bullae which may progress
to bronchopleural f i s t ~ l a e . ~Atrial fibrillation
and myocarditis may be present. A high tempera-
ture is common.
The anaesthetic management of Stevens-
Johnson syndrome is similar to that of epi-
dermolysis bullosa, although it is not classified
as a blistering disease. Ketamine has been used
successf~lly.~~ Intermittent positive pressure
ventilation may be hazardous in the presence of
visceral pleural bullae. Intravenous hydro-
cortisone may be necessary if patients are taking
oral corticosteroids.
Behcet’s syndrome
BehGet’s syndrome is a mucocutaneous disorder
Fig. 6. Waxy, non pitting oedema of scleroderma. The lack of veins is demonstrated.
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characterised by recurrent aphthous ulceration
of the oral mucosa and external genitalia,
associated with iritis. Scarring in the mouth is
uncommon but may produce difficulties at
intubatiom60
Scleroderma
Scleroderma may be either localised to the skin,
as in morphoea, or may result in progressive
fibrous deposition throughout the body. This
latter form, also known as progressive systemic
sclerosis, concerns the anaesthetist.
The onset is usually between the third and fifth
decade, being three times more common in
women.61 Dermal fibrosis of the hands, arms,
face and upper chest causes a waxy, non-pitting
oedema (Fig. 6). The face is pinched, the mouth
tight and restricted opening may lead to poor
dental hygiene (Fig. 7).8
Raynaud’s phenomenon is common and the
ability to sweat may be reduced. Chest wall
involvement may cause respiratory failure.62The
association of calcinosis cutis, Raynaud’s phen-
omenon, sclerodactyly and multiple telangiectasia
of the skin, lips, oral mucosa and gut is known
as the CRST syndrome.61
Cardiac involvement occurs in 80% of cases
and may result in cardiomegaly, pericarditis,
pericardial effusions, conduction defects and
dysrhythmias. However, of these, 19% will only
show an abnormal rhythm during continuous
twenty-four hour ECG monitoring.63
Pulmonary fibrosis leads to decreased compli-
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Anaesthesia and severe skin disease 45 1

and a fibrosed temporomandibular joint. Sel-

lick's manoeuvre may not be effective if the
oesophagus is fibrosed. Pretreatment with
antacids or H, receptor antagonists, emptying
the stomach or a head up induction are alterna-
tive aids. Intubation, as always, should be gentle
since the angle of the mouth may tear easily.
Muscle relaxants should not be given until the
larynx has been viewed.64
In the operating theatre the patient should be
kept warm to avoid precipitating Raynaud's
phenomenon. Radial artery sampling is unwise.
Although peripheral neuropathies may be
associated with scleroderma, local anaesthesia is
a safe alternative to general anaesthesia. There
are several reports of prolonged sensory block
following conduction anaesthesia, one sciatic
nerve block lasting sixteen h o ~ r s . ~All ~ + ~ ~
patients eventually recovered their normal sensa-
tion. The reduced skin blood flow may be the
cause of this prolonged action.

Ehlers-Danlos syndromes

Fig. 7. Scleroderma showing the characteristic pinched The Ehlers-Danlos syndromes are a group of
mouth and loss of skin creases on the forehead. disorders in which there is a genetic defect in
collagen synthesis. Inheritance may be autosomal
or X-linked, dominant or recessive.6L The fea-
ance and vital capacity. Gas transfer is impaired tures may range from the subclinical to the
and pulmonary hypertension may develop.* severe.
Gastro-intestinal complications include dys- The skin is thin, fragile and easily torn. Joints
phagia, gastric acid reflux, perforation, obstruc- are hyperextensible and dislocations are common
tion and malabsorption of the fat soluble (Fig. 8). Blood vessel fragility and poor support-
vitamins. Renal failure is common and may lead ing stroma lead to bruising, and at operation
to malignant hypertension.** bleeding may be profuse despite a normal clotting
Before operation. the ability to open the mouth screen. Aortic aneurysms, mitral regurgitation,
and the presence of carious teeth and mucosal
telangiectasia should be assessed. Routine in-
vestigations should include a full blood count,
urea and electrolytes, chest X-ray, ECG and
clotting screen. Lung function tests, steroid cover
and pretreatment with vitamin K may be neces-
sary.
Venous access may be difficult. Intravenous
induction of anaesthesia may provoke a painful,
cyanotic reaction in the fingers, similar to
Raynaud's phenomenon, possibly due to retro-
grade flow in the constricted veins.64 A gaseous
induction is preferable if no large veins can be
found. If acid reflux is a feature, protection of
the lungs must be considered. A rapid sequence
induction is not practicable since tracheal intuba- Fig. 8. Ehlers-Danlos syndrome is characterised by
tion may be difficult due to poor mouth opening hyperextensiblejoints.

452 G.B. Smith and A.J. Shribman
pneumothorax and bowel perforation may
occur.6 s
Adequate blood must be crossmatched even
for minor procedures. Platelet count and clotting
screen should be checked pre-operatively. Severe
bruising may result from intramuscular pre-
medication.
In the anaesthetic room venous access may be
difficult. Internal jugular or subclavian vein
cannulation is inadvisable because of the risk of
bleeding. If a cut down is needed the skin should
be approximated with tape, because stitches tend
to cut out.61Tracheal intubation should be gentle
to avoid causing bleeding in the oropharynx.
Intermittent positive pressure ventilation carries
the danger of causing a pneumothorax and is
best avoided. In theatre the delicate skin must be
protected with adequate padding.
Spinal and extradural anaesthesia are tradi-
tionally contraindicated in patients who tend to
bruise but may be the best compromise after
careful selection of the patients.69
Congenital anhidrotic ectodennal defect
Congenital anhidrotic ectodermal defect is a rare,
sex-linked, recessive disorder characterised by
partial or complete absence of sweat glands and
other epidermal appendages.6' Female carriers
may be mildly affected. The reduced number of
sweat glands leads to extreme dryness of the skin
and prevents heat loss via evaporation, especially
in children.
Salivary glands may show a marked inflam-
matory reaction. Mucous glands in the upper
respiratory tract may be absent, predisposing to
respiratory tract infections.
Frontal bossing, nasal deformities and mal-
formations of the maxilla and mandible may
occur and some or all of the teeth may be absent.
Regional techniques are preferable because
airway maintenance and intubation may be
difficult. If general anaesthesia is indicated, gases
should be humidified.
The eyes should be protected to prevent
corneal drying as tear production may be
reduced. Hyperpyrexia is a problem encountered
mainly in infants and adequate monitoring of
temperature with facilities for cooling, such as
fans and icebags should be present in theatre.
The risk of postoperative chest infections is
high and physiotherapy is recommended.
13652044, 1984, 5, Downloaded from https://associationofanaesthetists-publications.onlinelibrary.wiley.com/doi/10.1111/j.1365-2044.1984.tb07313.x by CochraneChina, Wiley Online Library on [30/01/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Steroid therapy in skin disease
Systemic steroid therapy is uncommon in skin
diseases. However, topical steroids may be used
for their anti-inflammatory activity, their effect
on immune reactions and to reduce DNA
synthesis and cell division. A large number of
topical steroid preparations exist and are avail-
able as ointments, creams, gels or lotions. The
rate of steroid release is highest from ointment
based preparations.
The degree of systemic absorption of topical
steroids depends on the concentration of the
agent, the area of diseased skin, the length of
time of application of the drug and the presence
or absence of occlusive polythene dressings.
The application of large quantities of potent
topical steroids under occlusive dressings for
several weeks may allow sufficient steroid to be
absorbed to cause hypothalamic-pituitary-
adrenal axis (HPAA) In prac-
tice, topical steroids are not prescribed in this
fashion and evidence exists that HPAA suppres-
sion is not a long-term p r ~ b l e m . ~We* , ~have
~
been unable to find any evidence that topical
steroid application has been responsible for
hypotension or cardiovascular collapse during
anaesthesia.
However, topical steroids may be responsible
for skin atrophy, probably as a result of suppres-
sion of mitosis of epidermal cells. It is important
that anaesthetists protect the fragile skin of such
patients and limit the use of strapping and
adhesive electrodes.
General principles of anaesthesia for severe skin
disease
The following points should always be con-
sidered. The anaesthetist should have a thorough
knowledge of the skin disease concerned and any
potential anaesthetic problems. There should
be a complete pre-operative assessment with
particular reference to the extent of skin lesions
and any systemic involvement. Awareness of
current drug therapy is important, particularly
regarding steroid administration. Monitoring
may be difficult. Trauma to skin and mucous
membranes should be avoided. The variety of
problems associated with the skin conditions
discussed make it difficult to suggest general
guidelines for the anaesthetic management of
individual diseases.

Acknowledgments
The authors would like to thank Miss A. Maggs
for secretarial assistance, Dr S. Wright and the
Department of Dermatology, Bristol Royal
Infirmary for supplying the illustrations, and Dr
S. Willatts for advice and constructive criticism
during the preparation of this manuscript.
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