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Neonatal Sweet's Syndrome Associated with Rectovestibular Fistula with Normal Anus.

Shinozuka J, Tomiyama H, Tanaka S, Tahara J, Awaguni H, Makino S, Maruyama R, Imashuku S - Pediatr Rep (2015)

Bottom Line: Rectovestibular fistula was confirmed after the signs of inflammation subsided and the rash disappeared.In the literature, we found another case of neonatal Sweet's syndrome associated with rectovestibular fistula in a Japanese female neonate.The perineal region should be screened for anomalies following diagnosis of Sweet's syndrome in neonates.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatrics, Uji-Tokushukai Medical Center , Uji, Kyoto, Japan.

ABSTRACT
Sweet's syndrome, characterized by fever and a painful erythematous rash with a dermal neutrophilic infiltrate, develops primarily due to paraneoplastic phenomena in adults. Sweet's syndrome is very rare in neonates. We report a Japanese female neonate (age <2 months), who developed Sweet's syndrome with episodes of perineal infection in association with congenital rectovestibular fistula with normal anus. Sweet's syndrome was diagnosed basing on clinical features and histopathology of biopsied skin tissues. Rectovestibular fistula was confirmed after the signs of inflammation subsided and the rash disappeared. In the literature, we found another case of neonatal Sweet's syndrome associated with rectovestibular fistula in a Japanese female neonate. The perineal region should be screened for anomalies following diagnosis of Sweet's syndrome in neonates.

No MeSH data available.


Related in: MedlinePlus

Biopsied specimen of skin shows a confluent neutrophilic infiltrate in the dermis and epidermis.
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fig002: Biopsied specimen of skin shows a confluent neutrophilic infiltrate in the dermis and epidermis.

Mentions: A 1.5-month-old female Japanese child was hospitalized due to fever and rash in 2014. Initial presentation included papules resembling varicella on the scalp. Birth history was uneventful. Upon admission, the patient (4.9 kg in weight, 48 cm in height) was febrile (37.7°C), and had a heart rate of 144 beats/min. She had signs of an upper respiratory infection, including cough and rhinorrhea. Papules were scattered on the face, neck, and trunk (Figure 1). On admission, she had leukocytosis associated with high C-reactive protein (CRP). Liver and renal functions were within normal limits (Table 1). We first suspected varicella lesions or pyoderma gangrenosum as infectious diseases and Langerhans cell histiocytosis as neoplastic disease; however, on the second day of hospitalization, at the dermatology clinic, Sweet’s syndrome was highly suspected due to symptoms such as fever, neutrophilic leukocytosis, painfully looking erythematous plaques as well as nodules, and a skin biopsy was performed (Figure 2). Furthermore, to rule out neoplastic leukocytosis, we tested for myeloproliferative neoplasms (e.g., BCR/ABL and JAK2/V617F mutations), which were negative. No bone marrow aspiration was performed. Histopathology of skin biopsy was diagnosed compatible with Sweet syndrome.7 Varicella and Langerhans cell histiocytosis were ruled out. We started treatment with antibiotics (cefotaxime + ampicillin ± VCM) and acyclovir, but four days after the diagnosis was obtained, prednisolone (initial dose 2 mg/kg followed by a taper; for 2 weeks) was introduced. As summarized in Table 1, serum CRP levels and leukocytosis improved in 2 weeks, in association with fading of the rash. On the other hand, platelet counts (>1.0 million/µL) and hepatic dysfunction appeared probably as adverse effects of treatment; however, laboratory data were normalized in 4 weeks. During the 4 weeks of hospitalization, her perineal problems were not evident and the patient was discharged with negative CRP and no rash. However, the patient was hospitalized again 2 weeks later with suspected vulvovaginal cellulitis. Her labia majora was red and swollen, and the perianal skin had sores. Cefazolin was prescribed. During this second hospitalization, the medical staff noticed vaginal fecal leakage. Rectovestibular fistula with normal anus was confirmed using a barium enema examination. Eventually, we diagnosed this case as Sweet’s syndrome based on the clinical,8 as well as histopathological,7 findings which occurred as a para-inflammatory phenomenon associated with perineal infection caused by the fistula. Written informed consent was obtained from the patient’ parents for publication of this case report.


Neonatal Sweet's Syndrome Associated with Rectovestibular Fistula with Normal Anus.

Shinozuka J, Tomiyama H, Tanaka S, Tahara J, Awaguni H, Makino S, Maruyama R, Imashuku S - Pediatr Rep (2015)

Biopsied specimen of skin shows a confluent neutrophilic infiltrate in the dermis and epidermis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508622&req=5

fig002: Biopsied specimen of skin shows a confluent neutrophilic infiltrate in the dermis and epidermis.
Mentions: A 1.5-month-old female Japanese child was hospitalized due to fever and rash in 2014. Initial presentation included papules resembling varicella on the scalp. Birth history was uneventful. Upon admission, the patient (4.9 kg in weight, 48 cm in height) was febrile (37.7°C), and had a heart rate of 144 beats/min. She had signs of an upper respiratory infection, including cough and rhinorrhea. Papules were scattered on the face, neck, and trunk (Figure 1). On admission, she had leukocytosis associated with high C-reactive protein (CRP). Liver and renal functions were within normal limits (Table 1). We first suspected varicella lesions or pyoderma gangrenosum as infectious diseases and Langerhans cell histiocytosis as neoplastic disease; however, on the second day of hospitalization, at the dermatology clinic, Sweet’s syndrome was highly suspected due to symptoms such as fever, neutrophilic leukocytosis, painfully looking erythematous plaques as well as nodules, and a skin biopsy was performed (Figure 2). Furthermore, to rule out neoplastic leukocytosis, we tested for myeloproliferative neoplasms (e.g., BCR/ABL and JAK2/V617F mutations), which were negative. No bone marrow aspiration was performed. Histopathology of skin biopsy was diagnosed compatible with Sweet syndrome.7 Varicella and Langerhans cell histiocytosis were ruled out. We started treatment with antibiotics (cefotaxime + ampicillin ± VCM) and acyclovir, but four days after the diagnosis was obtained, prednisolone (initial dose 2 mg/kg followed by a taper; for 2 weeks) was introduced. As summarized in Table 1, serum CRP levels and leukocytosis improved in 2 weeks, in association with fading of the rash. On the other hand, platelet counts (>1.0 million/µL) and hepatic dysfunction appeared probably as adverse effects of treatment; however, laboratory data were normalized in 4 weeks. During the 4 weeks of hospitalization, her perineal problems were not evident and the patient was discharged with negative CRP and no rash. However, the patient was hospitalized again 2 weeks later with suspected vulvovaginal cellulitis. Her labia majora was red and swollen, and the perianal skin had sores. Cefazolin was prescribed. During this second hospitalization, the medical staff noticed vaginal fecal leakage. Rectovestibular fistula with normal anus was confirmed using a barium enema examination. Eventually, we diagnosed this case as Sweet’s syndrome based on the clinical,8 as well as histopathological,7 findings which occurred as a para-inflammatory phenomenon associated with perineal infection caused by the fistula. Written informed consent was obtained from the patient’ parents for publication of this case report.

Bottom Line: Rectovestibular fistula was confirmed after the signs of inflammation subsided and the rash disappeared.In the literature, we found another case of neonatal Sweet's syndrome associated with rectovestibular fistula in a Japanese female neonate.The perineal region should be screened for anomalies following diagnosis of Sweet's syndrome in neonates.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatrics, Uji-Tokushukai Medical Center , Uji, Kyoto, Japan.

ABSTRACT
Sweet's syndrome, characterized by fever and a painful erythematous rash with a dermal neutrophilic infiltrate, develops primarily due to paraneoplastic phenomena in adults. Sweet's syndrome is very rare in neonates. We report a Japanese female neonate (age <2 months), who developed Sweet's syndrome with episodes of perineal infection in association with congenital rectovestibular fistula with normal anus. Sweet's syndrome was diagnosed basing on clinical features and histopathology of biopsied skin tissues. Rectovestibular fistula was confirmed after the signs of inflammation subsided and the rash disappeared. In the literature, we found another case of neonatal Sweet's syndrome associated with rectovestibular fistula in a Japanese female neonate. The perineal region should be screened for anomalies following diagnosis of Sweet's syndrome in neonates.

No MeSH data available.


Related in: MedlinePlus