an uncommon auto-immune bullous disease which is rare in children.
Two varieties of childhood pemphigus have been described, mainly;
pemphigus vulgaris and pemphigus folacious.
drugs can induce pemphigus - like reaction.
vulgaris is a rare relapsing disease affecting skin and mucous
is characterized by flaccid bullae, which appear on normal or
erythematous skin surface.
may appear in waves, grouped or annular and are filled with
serosanguinous, sero-prulent or hemorrhagic exudates.
Fig. 283:1-8: Different clinical types of Pemphigus Vulgaris
Fig. 283 Pemphigus vulgaris
Fig. 283. Pemphigus vulgaris (Flaccid Bullae)
Pemphigus (Nicoklysk's signe +ve)
Fig. 283-2 Pemphigus vulgaris(infected
Fig.283-4 Pemphigus-Subungual bullae
Fig. 283-5 Pemphigus vulgaris
Fig.283-2 Pemphigus of the
scalp and cicatricial alopecia
Fig. 286 d,e,f - Pemhigus vulgharis
sign is characteristic for pemphigus. This test can be performed by
drawing of the finger with firm pressure over the surface of the
skin covering the bulla which is apparently normal , shows that the
epidermis slides off much like a piece of wet tissue paper.
involved are usually the areas subjected to pressure or rubbing and
the mucous membrane of the mouth and larynx
the beginning are minimal such as mild itching or burning of the
rupture on the skin and mucous membrane leaving denuded eroded
bacterial infection is common where healing takes longer time.
Healing usually leaves residual hyperpigmentation but without
vulgaris is unusual in pregnancy.
cases were shown to be transmitted via the placenta. Affected
infants may have cutaneous and/or mucosal erosions or bullae, where
others were stillborn.
immunofluorescence shows positive result in skin biopsies from
all affected infants.
IgG pemphigus antibodies have been found in the majority.
sign is positive in pemphigus vulgaris
vegetans is characterized by hypertrophic vegetations formed after
rupture of the bullae mainly on the intertriginous areas.
affect first the oral cavity and then spread to cover extensive
areas of the skin. Secondary bacterial infection is common which may
lead to constitutional symptoms, malodorous smell of the mucous
membrane, and skin lesions.
Diagnosis of Pemphigus
‘Pemphigus syphiliticus‘: occurs in some babies. The bullae
most commonly occur on red infiltrated base on the palms and
soles. Their serous contents contain abundant active treponemas.
impetigo: The bullae of staphylococcal impetigo (pemphigus
neonatorum) erupt on normal skin. The causative bacteria can be
detected and the response to antibiotics.
Fig. 284. Mucosal pemphigus
Fig. 285. Pemphigus vegetans
folacious is a rare bullous disease involving an extensive area of
the skin, usually in the form of exfoliative dermatitis.
begin as small vesicles that gradually exfoliate and become crusted
covering wide areas of the skin. The condition at this stage is not
easily differentiated from exfoliative dermatitis. The lesions of
pemphigus folacious have symmetrical distribution.
Fig.285b. Pemphigus folacious
Fig. 285c. Pemphigus folacious
minimal but secondary bacterial infections are common.
hair usually fall.
pemphigus has the same cutaneous manifestations of pemphigus
folacious and the difference depends on age and time of onset. This type
begins in younger age groups and is accompanied by endocrine
begin on the face (butterfly area) and chest in the form of
erythematous scaly, crusted lesions, simulating lesions of
seborrheic dermatitis and lupus erythematosus.
Fig. 285b.Pemphigus erythematosus
is the degeneration of the intercellular bridges
eosinophilic microabscesses with intra-epidermal cleavage.
shows mild inflammatory infiltration by lymphocytes, eosinophils and
steroids are considered the main line in treatment of pemphigus.
Prednisolone orally in a dose of 3-6 mg/kgm daily. This dose may be
increased to 5-8mgm/kgm/day, if the lesions are not controlled by
the smaller doses. Tapering of the dose when there is control to
lesions and the patient is maintained later on lower doses.
and azathioprine in older age groups, were used as an adjuvant
therapy, but serious toxic side effects limited their use in
treatment of pemphigus.
drying lotions such as potassium permanganate 1: 8000 can be used
and antibacterial cream as Muperacin if there is secondary bacterial
topical mild or fluorinated steroid preparations can be used and
tried first, before the oral steroids in localized lesions, are
FAMILIAL CHRONIC PEMPHIGUS
familial chronic pemphigus is a rare hereditary disease unrelated to
pemphigus vulgaris although they have the same histopathological
develop mainly in areas exposed to friction, such as the sides of
the neck, axillae and groin, but less commonly involve the scalp or
are characterized by recurrent eruptions of flaccid vesicles with
clear or turbid fluid on normal or erythematous skin. Lesions extend
peripherally and the center may heal or show soft, flat, moist
remissions occur in cold weather and most patients find that heat
and sweating aggravate the condition.
membrane lesions are un common.
vegetans of the crural areas.
disease characteristically responds to antibiotics locally and
preparations can be
given when fungal lesions are detected.
combination with antibacterial or anti fungal topically (Decoderm
trivalent, Lotriderm, Kenacomb) may cause clearance of the skin
steroids are rarely indicated in contrast to pemphigus, where
systemic steroids are considered the main line of treatment.
This is an
auto-immune blistering disease which is rare in childhood. Bullous
pemphigoid is characterized by accumulation of IgG antibodies at the
dermo-epidermal junction in a linear band.
herpetiformis in contrast shows by immunophlorescence IgA antibodies
accumulation at the dermoepidermal junction. This can differentiate
dermatitis herpetiformis from bullous pemphigoides.
clinical varieties of bullous pemphigoid:
and non-cicatricial pemphigoid.
Fig. 286.a,b,c. Bullous
of bullous pemphigoides are associated with malignancy.
localized types: appear as a few itchy lesions of different
sizes in the form of blisters on an erythematous base and filled
with clear or hemorrhagic fluid.
sites involved are the palms and soles which may be misdiagnosed
membrane of the mouth and conjunctiva may be involved leading to
scarring after healing.
disseminated types: involve extensive areas of the body mainly
on the abdomen and inner sides of the limbs.
lines applied for pemphigus. Corticosteroids and
immunosuppressive drugs may be an effective treatment.
PEMHYGOID OF CHILDHOOD
bullous disease of childhood is rare. Different nominations were
given for this disease such as:
pemphigoid of childhood, juvenile dermatitis herpetiformis, juvenile
pemphigoid and childhood bullous dermatitis herpetiformis.
blisters develop, which may become very extensive, involving usually
the genitalia, buttocks and inner thighs. Most patients have the
lesions on the scalp, peri-oral area and to a lesser extent on the
bullae predominate but are occasionally haemorrhagic, and usually
arise on previously normal skin. Only rarely the lesions manifest
with wheals and papules.
may cluster around an older lesion forming a so-called ‘cluster of
jewels‘. Healing is rapid with hyperpigmentation but generally
and bullae are moderately pruritic, predominantly over the pelvic
and peri-oral regions.
The eyes are
often sore or gritty and rarely there is conjunctival scarring.
impetigo may resemble the initial lesions of childhood bullous
pemphigoid, but its short duration and response to antibiotics can
help in the differentiation.
bullosa: are often
present at birth and the lesions when heal leave scarring of the
skin and mucous membrane.
pigmenti and Acrodermatitis enteropathica may
cause confusion, but the onset of bullae within the first few weeks
of life in the former and the mucous membrane lesions of the latter
should help to differentiate both diseases.
rarely affects the face or genital region and usually has short
pemphigus is rare in childhood and the diagnosis depends on the
clinical picture, histological and immunological manifestations.
may give a similar clinical picture, but the deposition of IgG and
C3 at the basement membrane zone is usually diagnostic.
herpetiformis is a pruritic blistering skin disease, occasionally
seen in children. The disease has an immune origin and may be
accompanied by coeliac disease. Severe emotional stress may act as a
as excoriated pruritic erythematous papules, urticarial wheals or a
grouped small vesicles or bullae.
appear on the extensor aspects of the limbs, especially the knees,
below the elbows, axilla, trunk, shoulders, face and scalp.
stage the lesion may present with pigmentation and grouped scars.
are not uncommon.
pigmentation of the sites of the skin lesions occurs in half of the
Fig. 287. Dermatitis herpetiformis
Fig. 288. Dermatitis herpetiformis
Fig. 289. Dermatitis herpetiformis
herpetiformis may be associated with gastro-intestinal enteropathy
presenting with a clinical picture of coeliac disease.
dermatitis herpetiformis often involves the genitalia, sacral,
axillary folds, buttocks and extensor surface of the limbs. In
children the disease is usually asymptomatic, more common in male
children and curiously the disease in young age groups does not
respond to sulfapyridine and other medications that are effective in
herpetiformis may begin in childhood as papulovesicular or bullous
lesions that may extend to the adulthool which show exacerbation and
herpetiformis by immuno-phlorescence shows IgA antibodies
accumulation at the dermo-epidermal junction.
symetrical distribution, grouping of lesions, severe pruritus,
configuration with different shapes and response to sulphapyridine
and dapsone can differentiate the disease.
Edema of the
tips of dermal papillae and infiltration with neutrophils and
of the tips of the papillae.
of the epidermis and the confluence of several dermal tips produce
an effective medication. Much care should be considered when using
this medication due to the possibilities of the different side
of patients is very important during treatment due to possibility of
drug toxicity as hemolytic anemia and methaemoglobinaemia.
Erythema elevatum diutinum.
Bullous diseases (Pemphigoid, mucous membrane Pemphigoid).
Chronic bullous disease of childhood.
Bullous eruption of systemic lupus erythematosus.
Subcorneal pustular dermatosis.
Collagen diseases as Rheumatoid arthritis.
dose as a starting dose is: 50-100 mgm. /day. The dose may be
increased to 400 mgm /day according to the patients response then a
maintenance dose of 25 mgm/ week can be recommended.
patients with dermatitis herpetiformis can be controlled on lower
Dapsone is a big problem but overall the drug has probably fewer
long-term side effects than do corticosteroids or Sulphapyridine.
commonest side effects of Dapsone are:
This is also
common and is responsible for the bluish lips.
blood checks of hemoglobin and reticulocytes but also including
white cells and platelets should therefore be undertaken in all
patients for the first few months after starting Dapsone.
is in general less effective than Dapsone and, in doses, which are
effective, tends to cause more side effects, especially marrow
suppression. The usual dose is 0.5 g twice or three times daily.
1-2 gm /day also improves some cases of dermatitis herpetiformis.
diet: to improve
coeliac disease may be tried when there is no response to Dapsone,
which has an effect mainly on the skin rash.
Corticosteroids and ACTH:
are used for cases not responding to other lines of treatment.
Antihistamines and topical steroids.
pustular dermatoses is not related to infection where
could not be cultured from fresh lesions, meanwhile IgA and IgG
immunoglobulins have been detected.
pustular dermatosis is affecting mainly old women. In younger
patients the general health is not affected but myeloma may be seen
in old age groups.
most common sites involved are the flexor surfaces of the limbs,
axilla, submammary areas and the groin.
has a chronic benign, relapsing course and the pustular eruption has
the distinctive histology of a subcorneal bulla containing
of subcorneal pustular dermatosis are characterized by:
grouped vesicles that soon become pustular having different shapes;
annular, gyrate or spread peripherally with a serpiginous edges.
rupture leaving superficial leafy scales and faint brown-pigmented
may appear in successive waves in the same areas after healing.
easily distinguished by the presence of pathogenic organisms and the
response of the lesions to antibiotics.
differentiated by the clinical, immunological features and its
response to Dapsone and Sulphapyridine.
immunoglobulins can be detected in dermatitis herpetiformis,
characteristically both in the region of the blister and in normal
May show an
intra-epidermal bulla without acantholysis and may even respond to
Dapsone, but has characteristic direct immune-fluorescence.
by the clinical pattern and the positive direct immuno-fluorescence,
but repeated biopsies may be needed to obtain the typical spongiotic
psoriasis : may
resemble subcorneal pustular dermatosis very closely, either of the
acute von Zumbusch type with small pustules, or the spreading
natural history of the disease, the response to Dapsone and the fact
that the subcorneal pustule sits on the surface of the epidermis
rather than being an integral part of it serves to distinguish the
benign bullous disease of childhood can produce a subcorneal
pustule, as can a number of other disorders.
distribution, symmetry, tendency to iris formation and bulla
formation are characteristic.
generalized pustular eruption following an upper respiratory
infection described as a pustular bacterid occasionally shows
Subcorneal Pustular Dermatosis.
hygiene to the abraded skin surfaces. Drying lotions such as
potassium permanganate 1: 9000.
cream may have some effect.
steroids are usually not helpful.
the drug of choice that can give good results without relapsing
after stopping treatment as in dermatitis herpetiformis. Dapsone
dose is 50-150 mgm / day for adults. The dose is usually monitored
according to weight, age and the severity of the disease.
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