multiple, erythematous, pruritic maculopapular rashes that started in the dorsal aspect of both hands.

progressed to fluid filled skin lesions approximately ½ cm that was were gradually enlarging, rupturing
and forming new fluid filled blisters involving the upper arms, lower extremities, trunk and head (scalp
and forehead).

skin rashes were noted to be intensely pruritic during hot weather.

(multiple, pruritic maculopapular lesions that gradually became vesicles and bulla) was noted now
affecting the whole body with sparing of the face, soles and palms. There was also noted drying and
crusting of the nails.

multiple pruritic maculopapularlesions that eventually became vesicles and bulla which eventually
ruptured that started again from hands and lower extremities which progressed to the trunks. There
were noted sparing of the face and oral mucosa.

recurrence of similar skin lesion starting from the hands and eventually progressed to trunk. These again
started in the hands and lower extremities and progressed to the trunk, scalp, foreheadand with
involvement of oral mucosa and nails.

Work in the field and has direct exposure to humid, dry weather; and allergens

(+) Allergy to eggs, seafoods, chicken, ‘bagoong’

Head. (+) multiple, erythematous, pruritic maculopapularrashes topped with scales and crusting in the
scalp Eyes. (+) purulent eye discharge, (+) excoriations and crusting in the eyelids

(+) erosions on the buccalmucosa and tongue

(+) Multiple, ill to well defined, round tense, flaccid vesicles and bulla on the trunk, upper extremities,
lower extremities (+) Multiple, ill to well defined plaques topped with crusts, scales and areas of erosion
with erythematous base of trunk, upper extremities and lower extremities (+) dry and crusted nail tips,
both upper and lower extremities (+) Nicholsky sign

Subjective Data Objective Data ● 45-year old male ● Skin lesions that are pruritic, fluid filled that
eventually ruptured located in the upper extremities, lower extremities, trunk, scalp, forehead ● Lesion
involving the tongue and nails ● History of hypersensitivity/allergy to food ● Presence of Multiple, ill to
well defined, round tense vesicles and bulla on the trunk, upper extremities, lower extremities ●
Presence of Multiple, ill to well defined plaques topped with crusts, scales and areas of erosion with
erythematous base of trunk, upper extremities and lower extremities ● Mucosal involvement (tongue)

occur largely in the

newborn and in children younger than 5 years, and rarely in older individuals
no staphylococci can be grown
from the bullae of the staphylococcal scalded-skin syndrome, in contrast to the those
of
bullous impetigo. In staphylococcal scalded-skin syndrome, the staphylococci are
present
at a distant focus, often a purulent conjunctivitis, rhinitis, or pharyngitis or rarely a
cutaneous infection or a septicemia. The bullae are caused by a staphylococcal
exotoxin,
called exfoliatin.

no staphylococci can be grown

from the bullae of the staphylococcal scalded-skin syndrome
inically, patients with SSSS develop a tender, erythematous, scarlatiniform eruption
followed by the development of flaccid, easily ruptured bullae involving the
periorificial face, neck, axillae, trunk, and groins with sparing of the mucous
membranes [10]. The bullae typically exhibit a positive Nikolsky sign, leaving
behind moist, red erosions. Desquamation of the skin occurring in sheet- or ribbon-
like fashion follows

SSSS is characterized histologically by subcorneal splitting within the stratum

granulosum [11]. The blister roof is comprised of stratum corneum and few
granular keratinocytes. Rare acantholytic cells and sparse neutrophils may be
present within the intact lesion. The superficial dermis typically contains a sparse
mixed inflammatory infiltrate, in contrast to bullous impetigo and pemphigus
foliaceus, wherein the infiltrate is typically heavier. In addition, the subcorneal
blister of bullous impetigo typically contains a heavier neutrophilic infiltrate as well
as multiple Gram-positive cocci [11]. The diagnosis of SSSS is typically rendered
based on the clinical, histologic, and microbiologic data. Systemic and topical
antibiotics targeting Gram-positive cocci in addition to appropriate wound care of
erosions are the mainstay of therapy.

Hematogenous spread of the toxins produces fever and

widespread erythema of the skin that can be tender and quickly forms
thin-walled, fluid-containing bullae that rupture, leaving a moist base of
skin. Gentle friction applied to the skin produces Nikolsky sign, a
sloughing of superficial sheets of skin (see Figure 13-
2). Desquamation eventually follows.
SSSS usually occurs in younger children but can rarely develop in
adults.
It is most common in young children but may occur in adults who have
renal insufficiency or are immunocompromised. 
Very rarely, SSSS infection occurs in adults, but the chances in immunocompromised and renal failure
patients’

in SSSS S. aureus is present at a primary distant site such as the

nose, mouth, conjunctiva, umbilicus, or circumcision site. Fresh skin
lesions are therefore sterile and blister fluid is culture negative.
Staphylococcal scalded skin syndrome is manifested as a sudden
onset of fever and tender, blanchable erythema.
It starts on the central part of the face, neck, and intertriginous areas
and rapidly generalizes.
The palms, soles, and mucous membranes are spared. Flaccid
blisters occur within 1 to 2 days and soon exfoliate in large sheets,
with superficially denuded skin remaining 

Patients with SSSS manifested with an onset of fever and develop erythematous,
scarlatiniform eruption followed by the development of flaccid, easily ruptured
bullae involving the face, neck, axillae, trunk, and groins. The bullae typically
exhibit a positive Nikolsky sign. Conjunctivitis and eyelid crusting is common.
However, there is typically no mucosal involvement in Staphylococcal Scalded Skin Syndrome. Skin
blisters (bullae) and skin erosions give negative result in culture test, because there are triggered
by toxin and not direct bacterial infection.

Occur largely in the newborn and in children younger than 5 years, and rarely in
older individuals. May occur in adults who have renal insufficiency or are
immunocompromised. 

n general, skin blisters give negative result in culture test, but S. aureus develops from some prominent
site of infection as umbilicus, conjunctiva, breast, surgical wound, nasopharynx, blood. 
ollowing desquamation, affected areas appear as painful, shiny,
denuded patches

Fever
(+) erosions on the buccal
Flaccid vesicles and bulla on erythematous base of mucosa and tongue
the face and  trunk
well defined plaques 
Positive Nikolsky sign
Purulent eye discharge
Crusting on eyelids

Positive Staphylococcus aureus growth on Wound

GS/CS

flaccid bullae that usually arise

on an erythematous base. Erythema, oozing, and crusting are present.
Oral lesions do not occur.
Nikolsky sign is
positive,
pruritus

small flaccid bullae with crusting and scaling

scaly papules or superficial flaccid bullae on normal erythematous skin
the bullae readily rupture, resulting in moist erosions and corn flake like crusts
lesions are usually demarcated as opposed to the extending erosions of
phemphigus vulgaris
face scalp and upper trunk

Histopathology

The earliest recognized change may be either eosinophilic spongiosis, or more

commonly, spongiosis in the lower epidermis. Acantholysis leads first to the
formation of clefts, and then to blisters in a predominantly suprabasal location,
although intraepithelial separation may occasionally be higher in the stratum
spinosum. The basal keratinocytes, although separated from one another
through the loss of attachment to each other, remain firmly attached to the dermis
like a row of tombstones lining the blister base. The blister roof consists of the
remaining intact squamous epithelium. Within the blister cavity, there are
acantholytic keratinocytes that have rounded, condensed cytoplasm about an
enlarged nucleus with peripherally palisaded chromatin and enlarged nucleoli.
These may reside singularly or in clusters. They may be recognized cytologically in
a Tzanck preparation, which is a smear taken from the underside of the roof and
from the base of an early, freshly opened bulla. Acantholysis may extend into
adnexal structures. There is little inflammation in the early phase of blister formation.
If present, it is usually a sparse, lymphocytic perivascular infiltrate accompanied by
dermal edema. If, however, eosinophilic spongiosis is apparent, numerous
eosinophils may infiltrate the dermis, and as the lesions age, a mixed inflammatory
cell reaction consisting of neutrophils, lymphocytes, macrophages, and eosinophils
may develop. Because of the instability of the blister roof, erosion and ulceration
may occur. Older blisters may also have several layers of keratinocytes at the blister
base because of keratinocyte migration and proliferation, and there may be
considerable downward growth of epidermal strands, giving rise to so called villi.

Immunofluorescence Testing
DIF testing is a very reliable and sensitive diagnostic test for pemphigus vulgaris, in
that it demonstrates IgG in the squamous intercellular substance in 80% to 95% of
cases, including early cases and those with very few lesions, and in up to 100% of
cases with active disease. It remains positive, often for many years after the disease
has subsided. Indirect testing is less specific than the direct test. Circulating IgG
antibodies in patients with pemphigus vulgaris react with desmoglein 3, a
desmosomal protein, resulting in release of plasminogen activator and activation of
plasmin. This proteolytic enzyme acts on the intercellular substance and may be the
primary mechanism of dyshesion.

Pemphigus vulgaris, direct immunofluorescence, medium power. There is cell

surface (intercellular) IgG deposition. C3 deposition is also frequently seen.

Pemphigus vulgaris, indirect immunofluorescence, high power. Using monkey

esophagus as a substrate there is prominent cell surface (intercellular) staining with
IgG.