Short communications
& case reports
The staphylococcal scalded-skin syndrome versus erythema multiforme Ii. Ossoff,
D.M.D.,”
DEPARTMENT
OF
ORAT,
awl
J. L. Giwltcr,
PATHOLOGY,
TUFTS
D.M.D., T’NIVERSITY
MS.,“* SCHOOL
Boston, OF
Mass. DENTBL
MEDICISE
This article reports a case of scalded-skin syndrome in a young child. Characterized by a generalized exfoliative dermatitis resembling scalded skin, the disease may involve the oral mucosa and must be differentiated from Stevens-Johnson syndrome (erythema multiforme). Since the etiology is specific, the disease can be treated with antibiotics with dramatic resulls.
T
he staphylococcal scalded-skin syndrome represents a striking generalized dermatitis characterized by intraepithelial separation which produces large bullae and skin lesions resembling scalded skin.le3 The syndrome is caused by coagulase-positive staphylococci of phage Group II which have been associated with several specific entities.? In newborn infants this exfoliative dermatitis has been called Ritter’s disease. In young children it has been described as toxic epidermal necrolysis (Lyell’s disease). Staphylococcal scarlatiniform eruption and bullous impetigo have a similar common etiology and some overlapping clinical features.’ In some instances toxic epidermal necrolysis in adults is precipitated by drugs and may be considered as erythema multiforme4, 5 Clinically, the scalded-skin syndrome (toxic epidermal necrolysis) in young children is characterized by an abrupt onset of generalized erythema with tenderness of the skin. This is followed by the development of large, flaccid bullae. The Nikolsky sign is usually positive. The epithelium separates in sheets, leaving a red, raw surface resembling scalded skin. All the skin, including the soles and palms, may be involved. Mucous membranes, including those of the oral cavity and pharynx, may be involved. The lips in particular are involved, especially in the later stages of secondary desquamation, and are characterized by flaking and fissuring. The disease progresses, with healing occurring in 10 to 14 days, *Fourth-year ‘*Assistant
126
Medical Professor,
Student, Tufts University School Department of Oral Pathology.
of Medicine.
Volume
Number
Fig. remnant epithelium
Xcalded-skin syndrome
40
1
1. Glovelike desquamation of hand. of bulla at base of thumb. Because are still intact over denuded areas.
Note wrinkling of intraepithelial
of
skin and separation,
rolled margin deeper layers
127
of of
usually with no scarring or complications, although fatal outcomes have been reported.3 Treatment consists of supportive hospital measures, including hydration and a penicillinase-resistant antibiotic. Steroids are contraindicated in children. The following is a case of the scalded-skin syndrome in which the diagnosis of Stevens-Johnson syndrome was also entertained. CASE
REPORT
The patient, a 4-year-old white boy, was in good health until 1 day prior to admission when he fell and struck his neck and chin on a chair, abrading the skin. Later in the day he was irritable, the neck area was noted to be red, and the patient was put to bed. On the day of admission he awoke with redness and soreness of the groin and neck and was seen by his physician, who noted large areas of erythematous skin, with peeling skin on the forehead and exudative pharyngitis and crusted lips. The boy was referred to the Tufts New England Medical Center Hospital for treatment. The patient’s past medical history was noncontributory. He had no known allergies and took no medications. His developmental milestones were normal, and his immunizations were up to date. The family history, social history, and review of systems were all noncontributory. Physical examination revealed au uncomfortable, irritable boy with crusted lips and peeling skin on the face. The temperature was 37.9” C.; respirations, 28; pulse, 120; and blood pressure, 105/75. His height was 103.5 cm. (sixtieth percentile) and his weight was 17.6 kg, (eightieth percentile). Significant physical findings indicated a normocephalic male who had edematous eyelids with peeling of the skin. The cars and nose were without lesions. The lips were cracked, with crusting, and serous material was oozing from them. The mucous membranes were intact, and the tongue was normal. The pharynx was erythematous, with cnlarged tonsils. The teeth were normal in appearance, with no obvious caries. There were multiple erythematous areas denuded of skin (scalded) on the face, neck, axillae,
128
Lips
Or:11 Surg. Julv “I 1975 ’
Ossof and Giunta
Pig. 8. Dried, exfoliated areas of face present erythems, cracking, and angular
and upper fissuring.
chest
with
seborrheic
type
of
flaking.
elbows, groin, and knees. Bullae were present on the shoulders. The Nikolsky sign was positive on intact skin. The lungs were clear to percussion and auscultation. The heart had a regular rhythm, with no murmurs or gallops. The mucosa of the penis and rectum was intact. The remainder of the physical examination was within normal limits. Admission laboratory findings recorded a hemoglobin/hematocrit of 13.8/42; white blood count, 13,600; sodium, 140; potassium, 4.2 ; chloride, 102 ; carbon dioxide, 25; erythrocyte sedimentation rate, 0.5/2; and a blood urea nitrogen of 10. Urinalysis revealed a specific gravity of 1.031; sugar, negative; protein, trace; Ph, 6; and white blood cells, O-1. Cultures of the nose, bullae, skin, and urine were all negative for coagulase-positive Btaphylococc~~s aureas. The admission diagnosis was toxic epidermal necrolysis (staphylococcal scalded-skin syndrome) with a secondary diagnosis to rule out Stevens-Johnson syndrome. Because of discomfort, the patient was unable to tolerate orally administered liquids, and intravenous fluid therapy was begun on admission. Oxacillin, 200 mg. per kilogram per day, was given intravenously. During the first 3 days of hospitalization several areas of apparently uninvolved skin became erythematous and were then sloughed, while older areas of involvement showed drying, crusting, and improvement. On the third hospital day, the patient began to tolerate liquids given orally. Consrqucntly, the intravenous fluid therapy was discontinued and the Oxacillin \vas given orally. On the fourth hospital day (Figs. 1 and 2), the patient sloughed a glovelike portion of the left hand as the lower arms became involved with blisterC. On the fifth hospital day the patient was afebrile ing. His temperature spiked to 39.2” and doing well. He was discharged on the seventh hospital day on a regimen of Oxacillin, 250 mg. orally every 6 hours, for 5 days. Recovery VRS uneventful.
DISCUSSION
The case is considered to be one of the staphylococcal scalded-skin syndrome (toxic epidermal necrolysis) despite the negative cultures for coagulase-positive staphylococci. Features leading to this conclusion are the history of the abraded skin, the abrupt onset of the symptoms, the striking appearance of the skin and its resemblance to scalded skin, the positive Nikolsky sign, the spiking of temperature during sloughing, and the response to antibiotic therapy. In this disease the generalized exfoliative dermatitis is the striking feature. The characteristics of the bullae and the appearance of the denuded areas help to
Volume Number
40 1
Scalded-skin
syndrome
129
separate the disease from erythema multiforme. In the scalded-skin syndrome the oral mucosal involvement is not the predominant feature, whereas in erythema multiforme the appearance of the oral mucous membranes, particularly the lips, is a striking feature. In erythema multiforme the lips invariably are involved with bullae. They subsequently become swollen, hemorrhagic, and severely crusted as the bullae rupture, By comparison, in the scalded-skin syndrome the lip involvement is minimal, and erythema, cracking, and fissuring appear to be the salient features. In part, this can be explained by the histopathology of these two diseases. In the scalded-skin syndrome the pathologic change is in the upper layers of epithelium, producing an intraepithelial splitting in and about the granulosal cell layer.2l 6 There is little inflammation in the dermis. Thus, the Nikolsky sign is positive and the desquamation leaves a reddened area. In erythema multiforme, the pathologic change is generally a coagulation degeneration of the epithelium with subepithelial separation and extensive dermal inflammation. The bullae are more turgid. The Nikolsky sign is not a constant feature, since all layers of epithelium are involved. The separation of epithelium leaves an ulcer which crusts on the skin. Experimental studies have shown that penicillinase-resistant antibiotics are the treatment of choice for the scalded-skin syndrome and produce a dramatic effect. Steroid therapy, which may be beneficial in cases of erythema multiforme, is contraindicated and may have an adverse effect. Drug-induced cases of toxic epidermal necrolysis in adults offer a challenge in diagnosis, because these cases may represent erythema multiforme and are sometimes fatal.3 The scalded-skin syndrome should be considered in the differential diagnosis of vesiculobullous diseases. The generalized exfoliative dermatitis with a scalded appearance, a positive Nikolsky sign, and dramatic improvement with antibiotics help to differentiate this entity from erythema multiforme. REFERENCES
Scalded-Skin Syndrome : The Expanded 1. Me&h, M. E., and Glasgow, L. A.: Staphyloeoccal Clinical Syndrome, J. Pediatr. 78: 958967, 1971. 2. Melish, M. E., and Gl’asgow, L. A.: The Staphylococeal Scalded-Skin Syndrome, N. Engl. J. Med. 282: 1114-1119, 1970. 3. Bailey, G., and Rosenbaum, J. M.: Toxic Epidermal Necrolysis, J. A. M. A. 191: 979-982, 1965. 4. Zak, F. G, Fellner, M. J., and Geller, A. J. : Toxic Epidermal Necrolysis (Lyell) : The Scalded-Skm Syndrome, Am. J. Med. 37: 140-146, 1964. 5. Wheeler, C. E.: Erythemas. In Cecil, R. L., and Loeb, R. F.: Textbook of Medicine, ed. 12, Philadelphia, 1967, W. B. Saunders Company, p. 478. 6. Barrett, H. L.: Pediatrics, ed. 15, New York, 1972, The Meredith Corporation, p. 1776. Reprint
requests
to:
Dr. John Giunta Department of Oral Pathology Tufts University School of Dental One Kneeland St. Boston, Mass. 02111
Medicine
& case reports
The staphylococcal scalded-skin syndrome versus erythema multiforme Ii. Ossoff,
D.M.D.,”
DEPARTMENT
OF
ORAT,
awl
J. L. Giwltcr,
PATHOLOGY,
TUFTS
D.M.D., T’NIVERSITY
MS.,“* SCHOOL
Boston, OF
Mass. DENTBL
MEDICISE
This article reports a case of scalded-skin syndrome in a young child. Characterized by a generalized exfoliative dermatitis resembling scalded skin, the disease may involve the oral mucosa and must be differentiated from Stevens-Johnson syndrome (erythema multiforme). Since the etiology is specific, the disease can be treated with antibiotics with dramatic resulls.
T
he staphylococcal scalded-skin syndrome represents a striking generalized dermatitis characterized by intraepithelial separation which produces large bullae and skin lesions resembling scalded skin.le3 The syndrome is caused by coagulase-positive staphylococci of phage Group II which have been associated with several specific entities.? In newborn infants this exfoliative dermatitis has been called Ritter’s disease. In young children it has been described as toxic epidermal necrolysis (Lyell’s disease). Staphylococcal scarlatiniform eruption and bullous impetigo have a similar common etiology and some overlapping clinical features.’ In some instances toxic epidermal necrolysis in adults is precipitated by drugs and may be considered as erythema multiforme4, 5 Clinically, the scalded-skin syndrome (toxic epidermal necrolysis) in young children is characterized by an abrupt onset of generalized erythema with tenderness of the skin. This is followed by the development of large, flaccid bullae. The Nikolsky sign is usually positive. The epithelium separates in sheets, leaving a red, raw surface resembling scalded skin. All the skin, including the soles and palms, may be involved. Mucous membranes, including those of the oral cavity and pharynx, may be involved. The lips in particular are involved, especially in the later stages of secondary desquamation, and are characterized by flaking and fissuring. The disease progresses, with healing occurring in 10 to 14 days, *Fourth-year ‘*Assistant
126
Medical Professor,
Student, Tufts University School Department of Oral Pathology.
of Medicine.
Volume
Number
Fig. remnant epithelium
Xcalded-skin syndrome
40
1
1. Glovelike desquamation of hand. of bulla at base of thumb. Because are still intact over denuded areas.
Note wrinkling of intraepithelial
of
skin and separation,
rolled margin deeper layers
127
of of
usually with no scarring or complications, although fatal outcomes have been reported.3 Treatment consists of supportive hospital measures, including hydration and a penicillinase-resistant antibiotic. Steroids are contraindicated in children. The following is a case of the scalded-skin syndrome in which the diagnosis of Stevens-Johnson syndrome was also entertained. CASE
REPORT
The patient, a 4-year-old white boy, was in good health until 1 day prior to admission when he fell and struck his neck and chin on a chair, abrading the skin. Later in the day he was irritable, the neck area was noted to be red, and the patient was put to bed. On the day of admission he awoke with redness and soreness of the groin and neck and was seen by his physician, who noted large areas of erythematous skin, with peeling skin on the forehead and exudative pharyngitis and crusted lips. The boy was referred to the Tufts New England Medical Center Hospital for treatment. The patient’s past medical history was noncontributory. He had no known allergies and took no medications. His developmental milestones were normal, and his immunizations were up to date. The family history, social history, and review of systems were all noncontributory. Physical examination revealed au uncomfortable, irritable boy with crusted lips and peeling skin on the face. The temperature was 37.9” C.; respirations, 28; pulse, 120; and blood pressure, 105/75. His height was 103.5 cm. (sixtieth percentile) and his weight was 17.6 kg, (eightieth percentile). Significant physical findings indicated a normocephalic male who had edematous eyelids with peeling of the skin. The cars and nose were without lesions. The lips were cracked, with crusting, and serous material was oozing from them. The mucous membranes were intact, and the tongue was normal. The pharynx was erythematous, with cnlarged tonsils. The teeth were normal in appearance, with no obvious caries. There were multiple erythematous areas denuded of skin (scalded) on the face, neck, axillae,
128
Lips
Or:11 Surg. Julv “I 1975 ’
Ossof and Giunta
Pig. 8. Dried, exfoliated areas of face present erythems, cracking, and angular
and upper fissuring.
chest
with
seborrheic
type
of
flaking.
elbows, groin, and knees. Bullae were present on the shoulders. The Nikolsky sign was positive on intact skin. The lungs were clear to percussion and auscultation. The heart had a regular rhythm, with no murmurs or gallops. The mucosa of the penis and rectum was intact. The remainder of the physical examination was within normal limits. Admission laboratory findings recorded a hemoglobin/hematocrit of 13.8/42; white blood count, 13,600; sodium, 140; potassium, 4.2 ; chloride, 102 ; carbon dioxide, 25; erythrocyte sedimentation rate, 0.5/2; and a blood urea nitrogen of 10. Urinalysis revealed a specific gravity of 1.031; sugar, negative; protein, trace; Ph, 6; and white blood cells, O-1. Cultures of the nose, bullae, skin, and urine were all negative for coagulase-positive Btaphylococc~~s aureas. The admission diagnosis was toxic epidermal necrolysis (staphylococcal scalded-skin syndrome) with a secondary diagnosis to rule out Stevens-Johnson syndrome. Because of discomfort, the patient was unable to tolerate orally administered liquids, and intravenous fluid therapy was begun on admission. Oxacillin, 200 mg. per kilogram per day, was given intravenously. During the first 3 days of hospitalization several areas of apparently uninvolved skin became erythematous and were then sloughed, while older areas of involvement showed drying, crusting, and improvement. On the third hospital day, the patient began to tolerate liquids given orally. Consrqucntly, the intravenous fluid therapy was discontinued and the Oxacillin \vas given orally. On the fourth hospital day (Figs. 1 and 2), the patient sloughed a glovelike portion of the left hand as the lower arms became involved with blisterC. On the fifth hospital day the patient was afebrile ing. His temperature spiked to 39.2” and doing well. He was discharged on the seventh hospital day on a regimen of Oxacillin, 250 mg. orally every 6 hours, for 5 days. Recovery VRS uneventful.
DISCUSSION
The case is considered to be one of the staphylococcal scalded-skin syndrome (toxic epidermal necrolysis) despite the negative cultures for coagulase-positive staphylococci. Features leading to this conclusion are the history of the abraded skin, the abrupt onset of the symptoms, the striking appearance of the skin and its resemblance to scalded skin, the positive Nikolsky sign, the spiking of temperature during sloughing, and the response to antibiotic therapy. In this disease the generalized exfoliative dermatitis is the striking feature. The characteristics of the bullae and the appearance of the denuded areas help to
Volume Number
40 1
Scalded-skin
syndrome
129
separate the disease from erythema multiforme. In the scalded-skin syndrome the oral mucosal involvement is not the predominant feature, whereas in erythema multiforme the appearance of the oral mucous membranes, particularly the lips, is a striking feature. In erythema multiforme the lips invariably are involved with bullae. They subsequently become swollen, hemorrhagic, and severely crusted as the bullae rupture, By comparison, in the scalded-skin syndrome the lip involvement is minimal, and erythema, cracking, and fissuring appear to be the salient features. In part, this can be explained by the histopathology of these two diseases. In the scalded-skin syndrome the pathologic change is in the upper layers of epithelium, producing an intraepithelial splitting in and about the granulosal cell layer.2l 6 There is little inflammation in the dermis. Thus, the Nikolsky sign is positive and the desquamation leaves a reddened area. In erythema multiforme, the pathologic change is generally a coagulation degeneration of the epithelium with subepithelial separation and extensive dermal inflammation. The bullae are more turgid. The Nikolsky sign is not a constant feature, since all layers of epithelium are involved. The separation of epithelium leaves an ulcer which crusts on the skin. Experimental studies have shown that penicillinase-resistant antibiotics are the treatment of choice for the scalded-skin syndrome and produce a dramatic effect. Steroid therapy, which may be beneficial in cases of erythema multiforme, is contraindicated and may have an adverse effect. Drug-induced cases of toxic epidermal necrolysis in adults offer a challenge in diagnosis, because these cases may represent erythema multiforme and are sometimes fatal.3 The scalded-skin syndrome should be considered in the differential diagnosis of vesiculobullous diseases. The generalized exfoliative dermatitis with a scalded appearance, a positive Nikolsky sign, and dramatic improvement with antibiotics help to differentiate this entity from erythema multiforme. REFERENCES
Scalded-Skin Syndrome : The Expanded 1. Me&h, M. E., and Glasgow, L. A.: Staphyloeoccal Clinical Syndrome, J. Pediatr. 78: 958967, 1971. 2. Melish, M. E., and Gl’asgow, L. A.: The Staphylococeal Scalded-Skin Syndrome, N. Engl. J. Med. 282: 1114-1119, 1970. 3. Bailey, G., and Rosenbaum, J. M.: Toxic Epidermal Necrolysis, J. A. M. A. 191: 979-982, 1965. 4. Zak, F. G, Fellner, M. J., and Geller, A. J. : Toxic Epidermal Necrolysis (Lyell) : The Scalded-Skm Syndrome, Am. J. Med. 37: 140-146, 1964. 5. Wheeler, C. E.: Erythemas. In Cecil, R. L., and Loeb, R. F.: Textbook of Medicine, ed. 12, Philadelphia, 1967, W. B. Saunders Company, p. 478. 6. Barrett, H. L.: Pediatrics, ed. 15, New York, 1972, The Meredith Corporation, p. 1776. Reprint
requests
to:
Dr. John Giunta Department of Oral Pathology Tufts University School of Dental One Kneeland St. Boston, Mass. 02111
Medicine
