means redness of the skin due to dilatation of blood vessels. This will
lead to hyperemia in a specific area of the skin in response to
endogenous or exogenous factors. Erythema is the first and most
common skin manifestation, which may be localized or generalized.
clinical types of erythema
Localized erythema: this type involves certain areas due to
clinical descriptive names are related to the localized erythema
palmare : is
localized to the palms, which may appear due to liver cirrhosis.
of the intertriginous areas due to excess friction and sweating.
Fig.220b. Erythema intertrigo
pernio: due to
exposure to cold, which affects the acral parts .
occurring in response to any type of heat.
solare: erythema in
response of exposure to sun.
Fig. 220c. Erythema solare
types of erythema may occur in response to certain types of
food,chemicals, drugs, vaccines,stress, gastro-intestinal
disturbances and vasomotor liability.
This type is
generalized involving wide areas of the skin due to systemic
erythema presents with different clinical types.
ERYTHEMA OF THE NEWBORN
toxicum neonatorum is a patchy benign eruption of the newborn that
appears in the first three or four days of life and generally
disappears by the second week.
The cause of
this type of erythema of the newborn is unknown. Different factors
origin : hypersensitivity to milk.
transmitted to the infant via the placenta .
Fig.220d. Erythema due to drug
vaginal secretions of the mother.
response to some components of sebum .
initially appear as erythematous blotchy macules, papules or
pustules mainly on the trunk , face and proximal parts of the limbs.
palms and soles are affected.
may be surmounted by small pustules, 2-4 mm in diameter.
mildest cases these macules fade within a day.
severe cases, urticarial papules arise within the erythematous
areas, on the back and buttocks.
health of the infant is not affected and usually the lesions fade
away without treatment .
erythema of the newborn has to be differentiated from:
miliaria, which clinically simulates toxic erythema.
simplex virus infection .
Incontinentia pigmenti .
pustular pyoderma : The pustules show neutrophilic leukocytes.The
pustules in erythema neonatorum toxicum are filled with eosinophils
which are follicular and perifollicular .
erythematous macules show slight perivascular infiltration with
and supportive measures since no specific treatment is available.
Usually the skin lesion clears within few days .
types of exanthematous diseases manifest with erythema, which is
variable and specific to such diseases. Viral and bacterial diseases
are common cause of erythema in infants and children. Measles,
erythema infectiosum (fifth disease), roseola and scarlet fever are
some types of generalized erythema.
220d,e, Generalized exanthematous reaction
types of generalized erythema were reported in Holland and Germany
in 1958 in children, known as "Margarine disease" . Viral cause from
certain types of diary products was suspected. The disease is
slightly infectious that occurs in epidemics in spring and summer.
appears on the proximal parts of the extremities and sometimes on
the face and spread to cover the entire skin surface.Erythema
appears suddenly and usually without any prodromal symptoms. The
erythematous reaction has different clinical features simulating
erythema multiforme,papular and morbiliform eruption. The skin
lesion on the face has the butterfly appearance and present with
diffuse erythema or grouped tiny papules on an erythematous base.
severe but on the fifth day , erythema begins to regress and
itching becomes less.
This is a
hypersensitivity syndrome due to antigen-antibody reaction, which
may involve skin, mucous membranes and internal organs. Stevens and
Johnson‘s syndrome is an acute and severe clinical type of
Fig. 220 Erythema multiforme
Fig. 221 Erythema multiforme
Fig.223 Erythema multiforme
present with symmetrical bright bluish to dark purple slightly
infiltrated, round macules with tendency to spread peripherally.
clinical types of erythema multiforme may appear, which depend
mainly on the shape and severity of the reaction. Skin lesions may
be macular,papular,nodular, vesicular or bullous forming different
shapes such as the annular, circinate, the iris lesions, purpuric
and urticarial types.
lesion: The central
portion of the lesion usually shows a bluish gray central depression
known as the “iris lesions“ which is usually characteristic to
Fig. 223a. Erythema multiforme
common sites involved are the dorsal aspects of the hands,
fingers and feet.
characterized by symmetrical erythematous papular, urticarial,
bullous or even hemorrhagic reactions appear on the sun-exposed
areas of the skin. Skin lesions have the characteristic iris
membranes are usually involved. This type is usually recurrent and
usually preceded by herpes labilalis before the onset of erythema.
erythematous maculopapules, which spread peripherally forming
polycyclic patches with central clearing. Erythema iris may be
Fig. 223b. Maculopapular erythema
may show hemorrhagic bullae on an erythematous base.
grouped vesicles surrounded by an erythematous base, which may be
misdiagnosed as herpes lesions.
present with edematous bright bluish red macules or flat-topped
papules, which has the tendency to spread peripherally.
considered as a severe type of erythema multiforme which presents
with skin , mucous membrane and internal manifestations.
may show severe constitutional manifestations, where the severity
and prognosis depend on age, cause of the disease and early
symptoms : fever,
headache, malaise and soreness of the mouth and throat. The syndrome
may present with severe manifestations such as rapid
pulse,weakness,rapid respiration, prostration and joint pains.
may be fatal.
stomatitis is the early manifestation and may be diagnostic. Mucous
membranes show ulceration with bleeding, salivation and
angioedema.This may interfere with drinking or feeding causing more
cachexia to the patients.Conjunctivitis, corneal ulceration,rhinitis
and epistaxis are common mucous membrane manifestations.
Steven’s Johnson syndrome
224b.Steven’s Johnson syndrome
224c.Steven’s Johnson syndrome
lesions present with multiforme, erythematous, macular with an iris,papular,vesicular reaction with large hemorrhagic bullae on an
erythematous base. Rupture of the bullae lead to ulcerating abraded
skin surface, which is susceptible to secondary infections.
hemorrhages are common.
Purpura is a
common manifestation of the severe type.
factors are blamed as a causative of Steven-Johnson syndrome.
types with unknown cause are common.
Some of the
following may cause the syndrome:
Bacterial infections: Diphtheria, Brucellosis, T.B.,Typhoid fever
infections: measles, herpes, small pox vaccination, Asian flu and
infections: Coccidioidomycosis and histoplasmosis.
Protozoal infections: malaria and
Collagen diseases: systemic and discoid lupus
Vaccines: BCG, small pox and poliomyelitis vaccines.
penicillin,sulfonamides, trimethoprine, salicylates, phenobarbitol,
barbiturates, antipyrine and hydrazine.
syndrome may be accompanied with erythema multiforme.
Allergic skin contactants: rhus dermatitis and fire sponges.
Internal malignancy:lymphoma, carcinomas , polycythemia,myeloma
and Hodjkin‘s disease.
infiltrate of the upper dermis.
and edema of the epidermis and dermis.
inflammatory infiltrate around dilated blood vessels.
systemic lupus erythematosus.
depends on the cause and the severity of the clinical
elevatum diutinum is characterized by slightly painful, red,elevated,
round, polygonal nodules, usually arranged in annular shapes. The
most common sites involved are the extensor surfaces of the
extremities and hands.
Fig.224e. Erythema Elevatum
The nodules have smooth surface showing
central depression. The eruption has a chronic course, which may
extend for months.There is no involvement of the mucous membranes
and the general health is no affected.
nodosum is characterized by symmetrical , tender, bright red nodules
of different sizes, most common on the extensor surfaces of the
legs. Mild constitutional symptoms such as fever,malais,myalgia and
arthralgia may precede the appearance of the eruption.The disease is
Fig.224f. Erythema nodosum
nodosum was discussed in the previous chapters).
This type is
due to insect bites such as ticks.The erythematous reaction appears
in rings spreading peripherally with raised edges and pale center.
Fig. 224. Erythema chronicum migrans
This type is
common in children with rheumatic fever and rheumatic endocarditis.
Skin manifestations present with pale red to livid rings of variable
sizes and shapes. The commonest sites involved are thighs,abdomen,
chest and the back.
runs a chronic course, and may begin at any age even in newborn
infants . In neonates, annular erythema may be a sign of maternal
systemic lupus erythematosus. The most common sites are the buttocks,
thighs and upper arms, but any area may be involved. Sometimes
lesions are localized on the extremities but the face is seldom
Fig. 225. Erythema annulare centrifugum
may be solitary or more often multiple that appear mainly on the
buttocks , thighs and upper arms. Small, pink, infiltrated papule
slowly enlarges and forms a ring as the central area flattens and
fades. Individual lesions last for a few days, weeks, or slowly
extend for months, with the appearance of purpura and pigmentation .
the lesion may be irregular to leave arciform segments. The edge may
be quite flat or easily palpable, smooth or show slight scaling.
variable but seldom intense.
means erythema and skin scaling . Erythroderma may be primary or
secondary that occurs in the course of different skin diseases .
desquamativum (Linear‘s disease).
associated with lymphomas, Hodgkin‘s disease, and mycosis
skin exfoliation may occur with:
Fig. 226. Erythroderma (Erythema & skin exfoliation)
complications of erythroderma
sweat retention due to obstruction of sweat gland orifices may lead
to dangerous hypothermia especially in children in the tropics .
wasting: An increase
in metabolic activity provides compensatory increase in body heat
production but at the expense of tissue catabolism
failure: Skin blood
flow, blood volume and cardiac output may all be increased. If these
changes persist, they may lead to cardiovascular failure.
and enteropathy may occur.
Extensive scaling leads to protein and iron loss beside impaired
absorption and utilization, causing edema and iron deficiency anemia
. Serum B12 tend to be low, mainly due to increased utilization by
the hyperplastic epidermis.
and water loss: The
barrier efficiency of the skin in psoriatic erythroderma is
certainly impaired. The chief effect is the increase in water loss
by diffusion since evaporation of which also contributes to heat
output tends to drop and if water intake is inadequate for any
reason, dehydration results.
may be suspected in an infant with erythroderma that has persisted
from birth or shortly after birth.
thrive and diarrhea.
and/or hepatospleenomegaly will help to establish the diagnosis.
ichthyosiform erythroderma .
atopic eczema .
a common vascular skin reaction characterized by the appearance of
wheals that are transient and recurrent. These are erythematous,
elevated skin swelling surrounded by a halo and accompanied by
severe itching or stinging sensation.
may be accompanied by systemic manifestations such as asthma,
abdominal cramps, joint pain and severe laryngeal edema .
common in children and has different clinical picture that may
appear with different shapes , and varies from the simple wheal to
the severe bullous type .
Urticaria(Drug reaction ,Amoxycillin)
hemorrhagic edema (purpura en cocarde) occurs in very young children
and is on the borderline between urticaria and vasculitis.
lesions show vascular dilatation. The reaction in urticaria is
related to different factors; histamine (mediator for the common
urticaria), kinins (mediator for angioedema), serotenin ,
prostaglandin, anaphphylatoxin and acetylcholine release .
different factors affecting children are the same as that of adults
with a tendency to be more acute in young ages .
is classified according to its etiology into immunological or non-immunological
Fig. 227. Urticaria
Fig. 228. Urticaria
Fig. 229. Angioneurotic edema
Fig. 230. Urticaria
immunologic type of urticaria may be an IgE dependent.
factors that can cause immunologic urticaria are :
Food due to
a specific antigen .
factors due to sunlight , heat , water , and pressure urticaria.
This type is
due to substances that have direct effect causing degranulation of
factors that can cause non-immunologic urticaria are:
containing histamine such as fish .
physical factors .
presenting with chronic urticaria should be thoroughly investigated.
This doesn‘t mean to do immediately every possible investigation
to diagnose or exclude every possible underlying cause .
a careful interrogation of the patient or the child‘s mother may
be very helpful to reach the diagnosis of the triggering factors.
relation to food especially food with additives, colored or
preserved foods should be considered .
taken by the child such as antibiotics or antipyretics as
salicylates should be thoroughly investigated.
may appear with other diseases such as systemic diseases or hormonal
ants , mosquitoes may cause severe reaction especially in sensitive
Fig. 231. Papular urticaria, Purpuric lesion
Fig. 232. Papular urticaria (Insect Bite)
state of the child is important where some cases of urticaria
are due to stress and psychological trauma .
of chronic recurrent urticaria is not always successful in detecting
the offending causative factors .
tests may be considered such as:
blood picture: Blood count, including eosinophils count and ESR.
protein electrophoresis .
function tests .
any suspected infections .
function tests .
B surface antigen .
Complement screen , including C1 esterase inhibitor .
analysis for detecting any parasites .
x-ray for the nasal sinuses to detect any septic focus .
Provocation tests with Tartrazine and anti candida may be of value
in older children.
are different types of urticaria :
agents such as cold, heat and sun may precipitate this type of
urticaria may be transmitted as an autosomal dominant .
lesions present with wheals that appear after exposure to cold . The
condition begins after birth and may persist throughout life .
of cold urticaria accompanies collagen diseases such as lupus
erythematosus, dysproteinaemia or haemoglobinuria.
This is a
common type of urticaria, where whealing appears after exposure to
cold and can be relieved after warming of the skin . Mucous membrane
of the mouth , pharynx and gastrointestinal tract may be involved.
having this type of urticaria should have much care and should be
warned not to be exposed suddenly to cold or swimming in cold water
or having a cold shower, where these may cause syncope and even
cold urticaria is by applying an ice on the skin, this will
provoke an urticarial lesion .
lesions appear due to histamine release after sweating initiated by
heat or after bathing with hot water .
lesion appears few minuets after exposure to sunlight . This
condition may be associated with the delayed type of
photosensitivity and polymorphous light eruption .
urticaria is a common type, characterized by linear and with sharp
edged wheals, appearing after scratching of the skin by a blunt
object. This is associated with increase in the circulating IgE.
stroking of the skin gives rise to the triple response of Lewis with
a wheal and ‘flare‘. This may appear in 25-50% of normal people.
Fig. 233. Dermographic urticaria
This type of
urticaria can be provoked by pressure of tight clothes or after
stroking the skin, where few seconds later a wheal with a
surrounding erythematous flare corresponds to the affected sites.
This may be
accompanied by itching . Scratching also leads to the appearance of
linear raised erythematous streaks .
food and drugs should be eliminated .
(Atarax) is considered specific in the treatment of such type of
generation antihistamine; Cetrizine (Zyrtec) is also of value.
steroids are not always recommended .
parental steroids are temporarily effective and rarely indicated in
infants and children .
such as Cimetidine may be tried in chronic and resistant cases .
(Periactin): May be given to older children and not to young age.
This drug may be effective especially if combined with hydroxazine.
combination of two antihistamines of the different group may be
necessary in older children .
Tricyclic antidepressants, such as doxepin, have
the capability to block both H1 and H2 receptors. Leukotriene
receptor antagonists and oral beta-adrenergic agents are reported to
be helpful as combination therapy with antihistamines.
Low-dose thyroid hormone for patients who
have positive antithyroid antibodies .
colchicine, dapsone, and COX 2 inhibitors have also been used in
some cases not responding to traditional treatment.
This type of
urticaria is herido-familial dominantly transmitted due to absence
of C1 esterase inhibitor, which normally inhibits the activity of
the first component of the complement .
manifestations begin early in childhood and are characterized by
recurrent attacks of swelling of the subcutaneous tissue, lips,
mouth and eyes. The condition may be precipitated by injury where
non-itchy, firm and painful lesions appear.
symptoms: severe abdominal colic due to gastro-intestinal tract
involvement may be a manifestation in some cases .
tract :may be involved and in severe cases it may cause asphyxia in
infants and children .
herido-familial urticaria depends on :
typical urticarial lesions .
subcutaneous tissue .
colic due to gastro-intestinal involvement .
C1 esterase inhibitor,C2 and C4 complement components.
sometimes disappointing in such cases, where the mortality rate is
considered high . Fresh plasma replacement of esterase inhibitor may
have some effect .
acid and Danazol ( weak androgen which inhibits pituitary
gonadotrophin ) both proved to be effective prophylactic
pigmentosa is an uncommon dermatoses that usually develops during
the first year of life . The condition is characterized by
urticarial lesions due to histamine release from excess mast cells
in the skin and may be associated with systemic manifestations.
early in infancy usually in the first year of life . The clinical
picture varies from scanty urticarial lesions and pigmented
flat macules with irregular edges that appear after scratching
of the skin .
can be provoked mechanically by stroking normal skin.
Fig. 234. Juvenile urticaria
Fig. 235. Juvenile urticaria
Fig. 236 urticaria
urticarial lesions with beaded edges due to accumulation of
mast cells under the skin may appear . Blisters may develop
due to separation of the dermoepidermal junction .
pigmentosa may be accompanied by systemic manifestations such
as abdominal colic , vomiting , tachycardia and flushing due
to excess histamine release .
regression of the infantile form may take place or may persist
to the adult life presenting with flat-pigmented macules .
Demonstration the presence of mast cells can
appear by rubbing the urticarial patch , where the rubbed
area becomes reddened, swollen and itchy. This is known as
Darier sign, and confirms the presence of mastocytosis.
may be the continuation to the infantile type or arise in the adult
age . The lesions are in the form of itchy macules around 4mm in
diameter, lightly pigmented appearing after rubbing or scratching of
the skin surface.
manifestation of the adult type is more than in the juvenile type .
spleen may be enlarged due to diffuse mastocytosis and the bones may
show osteoporosis and sclerosis .
Fig. 237. Adult urticaria pigmentosa
should be warned not to rub the body vigorously especially during
and after bathing with hot water, where this may activate mast cells
in the skin and respiratory tract which may lead to flushing ,
hypotension, bronchospasm and even sudden death .
H1 blockers and cyproheptadine may give some relief .
claimed to have some effect in alleviating symptoms in urticaria
manifestations can be treated accordingly .
Gollhausen R, Kligman AM. Human assay for identifying substances,
which induce non-allergic contact urticaria: the NICU-test.
Contact Derm 1985; 13: 98-106.
Groot AK, Gerkens F. Contact urticaria from a chemical textile
finish. Contact Derm 1989; 20: 63-4.
L. Recurrent urticaria: clinical investigation of 330 patients. Br
J Dermatol 1981; 104: 369-81.
Michaelsson G, Juhlin L. Urticaria induced by preservatives and
dye additives in food and drugs. Br J Dermatol 1973; 88: 525-32.
WE. Possible relevance of changes in mast cells and neutrophils to
perpetuation of chronic urticaria. In: Champion
Greaves MW, Kobza Black A et al., eds. The Urticarias. Edinburgh/
New York: Churchill Livingstone, 1985: 70-85.
Black A, Greaves MW, Champion RH et al. Urticaria. Br J Dermatol
1991; 124: 100-8.
Lagunoff D. The mechanism of histamine release from mast cells.
Biochem Pharmacol 1972; 21: 1889-96.
MacDonald SM, Lichtenstein LM, Proud LM et al. Studies of IgE-dependent
histamine releasing factors: heterogeneity of IgE. J Immunol 1987;
RP Champion RH. Urticaria. London: W.B. Saunders, 1974.
WE Possible relevance of changes in mast cells and neutrophils to
perpetuation of chronic urticaria. In: Champion RH, Greaves MW,
Black AK, Pye RJ, eds. The Urticarias. Edinburgh: Churchill-Livingstone,
C. IgE immune complexes in food allergy; significance,
pathogenicity and clinical consideration. Clin Allergy 1987; 17:
DJ, Kobza-Black A, Barrow SE et al. Prostaglandin D2 and histamine
release in cold urticaria. J Allergy Clin Immunol 1986; 78:
Michaelsson G, Juhlin L. Urticaria induced by preservatives and
dye additives in food and drugs. Br J Dermatol 1973; 88: 525-32
Thompson HL, Burbelo PD, Segui-Real B et al. Laminin promotes mast
cell attachment. J Immunol 1989; 143: 2323-7.
Spiller R, Knox JM. Histopathology of erythema toxicum neonatorum.
Dermatol 1960; 82: 586-9.
Harris R, Schick B. Erythema neonatorum. Am J Dis Child 1956; 92:
Yadav V. Etiology of toxic erythema. Am J Dis Child 1963; 106:
HL, Cothran F. Erythema toxicum neonatorum present at birth. Am J
Dis Child 1962; 103: 617-19.
RA, Gavin MP, Keefe M. Severe toxic erythema caused by diltiazem.
Br Med J 1988; 296: 1071.
T, Jansen CT. Erythroderma: a follow-up of 50 cases. J Am Acad
Dermatol 1983; 8: 836-40.
JJ, Mali JWH. The influence of erythroderma on the body. Arch
Dermatol 1957; 75: 573.
DWS, Spencer M-J, Tidman MJ. Papulo-erythroderma - clinical and
ultrastructural features. Clin Exp Dermatol 1990; 15: 105-6.13.
B. Chemistry and storage function of mast cell granules. J Invest
Dermatol 1978; 71: 76-80.
Briffa D, Eady RAJ, James MP et al. Photochemotherapy (PUVA) in
the treatment of urticaria pigmentosa. Br J Dermatol 1983; 109:
Poynard T, Nataf C, Messing B et al. Secretory diarrhea and
prostaglandin D2 overproduction in systemic mastocytosis. New Engl
J Med 1982; 307: 186 .(Letter).
AD, Herriot R, Davidson RJL et al. Adult mastocytosis, an
immunophenotypic and flow cytometric investigation. Br J Dermatol
1990; 122: 737-44.
Rasmussen JE. Xanthelasmoidea: an unusual case of urticaria
pigmentosa. Arch Dermatol 1976; 112: 1270-1.
MC, Beaulieu G, Birt AR. Histamine liberation by codeine and
Polymyxin B in urticaria pigmentosa. Arch Dermatol 1962; 86:
NA, Austen KF, Wasserman SI. Oral disodium cromoglycate in the
treatment of systemic mastocytosis. New Engl J Med 1979; 301:
Crotty RQ. Erythroderma desquamativum
(Leiner‘s disease). Arch Dermatol
1955; 71: 587-90.
Tandt W, Eggermont E, Bourgeois N. Erythroderma desquamativum.
Acta Paediatr Belg 1967; 21: 433-50.