ARTICLE
Staphylococcal scalded skin syndrome (SSSS) is usually observed in neonates
or children, and is extremely rare in adults [1]. The prognosis and risk
factors for SSSS in adults differ from those in children [1]. Most adult
cases of SSSS involve severe complications that decrease immunity or renal
function [1], therefore, in contrast to infant cases, the mortality rate
is high in adult cases. Nineteen of 32 reported cases with SSSS in adults
died soon after diagnosis, despite appropriate antibiotic therapy [1].
In Japan, the reports of SSSS caused by methicillin-resistant Staphylococcus
aureus (MRSA) have increased, especially in adults [2, 3]. We report
a fatal case of SSSS in an adult patient with prosthetic valve endocarditis
(PVE) caused by MRSA.
Case report
A 67-year-old man was referred to the division of dermatology of Yamagata
Prefectural Nihon-kai Hospital with erosion and erythema on his face,
neck, axilla, abdomen and genitalia on August 24, 1998. Right hemiparesis
due to a cerebral hemorrhage had developed two years before the episode.
The patient had undergone coronary artery bypass grafting and aortic valve
replacement bioprosthetic valve for treatment of angina pectoris and aortic
regurgitation on July 16, 1998. Post-operative administration of cefazoline
(4 g daily) was performed for 7 days. High fever, asthma-like attacks,
leukocytosis and increased serum C reacting protein (CRP) level in laboratory
findings were observed on the 9th postoperative day and arterial blood
culture obtained at that time was negative. Mediastinitis or PVE was suspected
and intravenous application of vancomycin hydrochloride (2 g daily) was
started because symptoms were observed just after cefazoline administration.
High fever decreased and abnormal laboratory findings were normalized
after the administration of vancomycin hydrochloride for 10 days. On the
35th postoperative day (August 19, 1998), high fever and an increased
CRP level recurred. Diffuse erythema with erosion on the face, axillar
and inguinal regions appeared four days later (August 23, 1998). Dermatological
examination at that time revealed diffuse erythema on the face, especially
in the perioral region, and erythema with giant desquamation on the neck,
axilla, genitalia, chest and abdomen. Leukocytosis (12,410/ml), thrombocytopenia
(80,000/ml) and increased serum CRP (14.29 mg/dl) and lactate dehydrogenase
(595 U/l) levels were also observed, but renal functions were normal.
We suspected him of having SSSS due to bacterial endocarditis, and we
began administering 2 g of vancomycin hydrochloride daily. The patient,
however, died of cardiac rupture caused by myocardial necrosis three days
after the start of the therapy. Postmortem arterial and venous blood culture
were positive for MRSA, although bacterial culture of erosive skin and
pharynx were negative for MRSA. Autopsy was performed immediately after
death. A white coating was observed around the aortic valve, which usually
indicates PVE. Cardiac rupture at the site of vegetation was also seen.
MRSA was cultured from the white coating and from venous blood. Dermatological
findings at autopsy showed extensive exfoliation over the entire body
surface and erosion on the axilla, neck, chest and abdomen (Fig.
1). Histopathological findings of a skin specimen revealed separation
in the supra-granular layer of the epidermis with a few infiltrating cells
in the dermis (Fig. 2).
The strain of MRSA isolated from the venous blood of the patient produce
exfoliative toxin (ET)-B only and not ET-A, enterotoxins A, B, C, D, E,
or toxic shock syndrome toxin-1, on assay of supernatant from patient
cultures using latex agglutination kits (EXT-RPLA; Denka Seiken Co. Ltd.;
Gosen, Niigata, Japan) [4].
Discussion
SSSS is a severe general infection caused by ET-producing Staphylococcus
aureus. For many years, SSSS and toxic epidermal necrolysis (TEN)
were confused as the same disease. Melish and Glasgow [5], however, made
a distinction between the two. Histologically, SSSS shows intra-epidermal
cleavage at the level of the granular layer and relatively little damage
to the underlying epidermal cells, while TEN shows a cleavage plane affecting
almost the full thickness of the epidermis accompanied by considerable
necrosis of the epidermal cells [6]. The diagnosis of SSSS is based on
three criteria [7]: 1) the clinical pattern of erythroderma, desquamation
or bulla formation; 2) isolation of exfoliative toxin-producing S.
aureus from the patient; 3) histological demonstration of intraepidermal
cleavage through the stratum granulosum. Our patient satisfied these criteria.
Most cases of SSSS occur in infants, and SSSS in adults was first described
in 1972 by Levine and Norden [8]. SSSS is rarely observed in adults probably
because adults are better able to metabolize and excrete ET [9], and have
lower sensitivity to ET of Staphylococcus aureus [10]. Therefore,
most adult cases of SSSS involve severe complications that decrease immunity
or renal function [1, 11]. Two major risk factors for SSSS in adults have
been identified: renal failure and immunosuppression caused by malignant
diseases, HIV infection or administration of agents such as corticosteroids,
non-steroidal anti-inflammatory drugs or immunosuppressive drugs [1].
However, SSSS can occur in healthy adults without any risk factors [1].
Therefore, in contrast to infant cases, the mortality rate in adults is
high. Nineteen of 32 reported adults with SSSS died soon after diagnosis,
despite appropriate antibiotic therapy [1]. In our case, there was no
obvious risk factor except for postoperative state.
The port of entry for staphylococcal infection
or the initial focus of infection is often obvious in SSSS in adults:
catheterization, abcesses, septic arthritis, and infection of arteriovenous
shunts [1]. We consider that SSSS of our case was caused by MRSA infection
of the prosthetic valve because other sites including lesional skin and
pharynx were negative for Staphylococcus aureus. However, to our
knowledge, there are no other reported cases caused by PVE. Only two other
cases complicated with endocarditis have been reported [12, 13]. PVE often
causes perivalvular leakage, prolapse of prosthetic valves and severe
heart failure. Therefore, PVE is often diagnosed in the early stages and
therapy is performed immediately. In our patient, the clinical symptoms
of PVE were atypical. Therefore, we ruled out PVE in the early stage and
administered an inadequate dose of vancomycin hydrochloride. We speculate
that the inadequate dose of vancomycin hydrochloride caused persistent
but relatively mild and non fatal endocarditis due to ETB-positive MRSA;
therefore, SSSS occurred before the patient died of PVE. In Japan, reports
of SSSS caused by MRSA have increased markedly in the past several years
[2, 3]. We should note that PVE caused by MRSA has the possibility for
focus of infection in SSSS of adults.
Article accepted on 31/8/00
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