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DERMATOLOGICA SINICA 32 (2014) 113114
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Coexisting staphylococcal scalded skin syndrome and acute generalizedexanthematous pustulosis
Figure 1 (A) Perioral exudation and crusting with radial fissures; (B) pinhead-sizedpustules with underlying erythematous base and desquamation.
Staphylococcal scalded skin syndrome (SSSS) is caused by epi-dermolytic toxins produced by Staphylococcus aureus and charac-terized by blistering and superficial desquamation.1 Acutegeneralized exanthematous pustulosis (AGEP) is most often causedby drugs and is characterized by the rapid occurrence of many ster-ile, nonfollicular pustules, usually arising on an edematous ery-thema and often accompanied by leukocytosis and fever.2 Wepresent a case of a 3-year boywith initial SSSS, who then developedAGEP due to erythromycin.
A 3-year-old boy presented with fever, sore throat, desquama-tion in the neck, and progressive malaise. His family physicianthus prescribed erythromycin and ibuprofen suspension. However,pustular lesions erupted on his neck the day after the first intake ofmedication, and grew to involve the other major skin folds thefollowing day. The child grew increasingly lethargic and com-plained of periorbital pain. He was brought to our emergencydepartment and was admitted on suspicion of SSSS.
On physical examination, the childs temperature was 38.2Cand he appeared acutely ill. The white blood cell count was20,900/mL. The patient had periorbital erythema that was tenderon palpation, and perioral exudation and crusting, with radial fis-sures around the mouth (Figure 1A), but no lesions on the oral mu-cosa. Desquamation as well as pinhead-sized pustules overlying theerythematous base was observed around the neck, axilla, and thegroin region (Figure 1B). Bacterial cultures of a pustule weredone, which were later found to be negative. A skin biopsy wastaken from his neck. The specimen demonstrated two histologicalpatterns: the left side of the specimen contained a subcorneal cleftand the right side showed intraepidermal pustules (Figure 2A). Thesubcorneal cleft, under higher magnification, showed acantholysiswith minimal inflammation, which is consistent with SSSS(Figure 2B). On the other side of the specimen, the intraepidermalspongiform pustules were infiltrated by abundant neutrophils inthe papillary dermis, findings consistent with AGEP (Figure 2C).Thus, the biopsy showed two distinct disease processes, SSSS andAGEP, in accordance to history and clinical presentation.
The patient was started on intravenous oxacillin 500 mg q6hand clindamycin 150 mg q8h, and antibiotic treatment continuedthroughout hospitalization (8 days). The intermittent feverresolved on Day 6; desquamation on the trunk improved bydischarge on Day 8. The patient was discharged on cloxacillin250 mg q8h and clindamycin 150 mg q8h for the remainder ofthe course. There has been no recurrence to date.
1027-8117/$ see front matter Copyright 2013, Taiwanese Dermatological Associatiohttp://dx.doi.org/10.1016/j.dsi.2013.04.004
The clinical and pathologic features of the case point to an initialSSSS with subsequent development of AGEP, according to thecriteria proposed by Roujeau et al.3 Tracing back the medical his-tory, ibuprofen had been previously administered and no adverseevents had occurred. Erythromycin was thus considered the culpritdrug for AGEP.
This is an unusual case of SSSS coexisting with AGEP. The exfo-liative phase of SSSS is often heralded by perioral exudation andcrusting with large radial fissures, but does not involve the mucosalmembranes, as occurred in our patient. There is usually no historyof drug exposure. The most reliable diagnostic tool remains a skinbiopsy.1 Our biopsy specimen showed a large subcorneal cleft for-mation, with acantholysis in the superficial epidermis and no trace
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Figure 2 (A) Biopsy specimen shows a subcorneal cleft on the left and intraepidermalpustules on the right; (B) a subcorneal cleft with acantholysis with minimal inflamma-tion; (C) intraepidermal spongiform pustules with abundant neutrophils in the papil-lary dermis. (Hematoxylin and eosin; A, 20; B and C, 200.)
Correspondence / Dermatologica Sinica 32 (2014) 113114114
of neutrophils in the bullae; a finding consistent with SSSS but un-explainable by AGEP.
There arefive criteria forAGEP: (1) sudden eruptionof numerous,small (