Sweet Syndrome in Early Infancy

Mohamed Ben Rejeb; Sonia Boudaya; Emna Bahloul; Fatma Frikha; Mariem Amouri; Hamida Turki

doi:10.4103/ijpd.IJPD_32_20


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CASE REPORT

Year : 2021 | Volume : 22 | Issue : 1 | Page : 77-79

Sweet Syndrome in Early Infancy

Mohamed Ben Rejeb, Sonia Boudaya, Emna Bahloul, Fatma Frikha, Mariem Amouri, Hamida Turki
Department of Dermatology, Hospital Hédi Chaker, Sfax, Tunisia

Date of Submission	26-Feb-2020
Date of Decision	08-Apr-2020
Date of Acceptance	07-May-2020
Date of Web Publication	31-Dec-2020

Correspondence Address:
Mohamed Ben Rejeb
Department of Dermatology, Hospital Hédi Chaker, Sfax
Tunisia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijpd.IJPD_32_20

Abstract

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory disease in pediatrics. It presents with fever, blood neutrophilia, and painful erythematous plaques with a dermal neutrophilic infiltrate. In general, it is associated with infections, hematologic and solid tumor, immunodeficiency, and certain drugs. It can easily be mistaken for infection and can lead to prescribe antibiotics or antiviral agents. Here, we describe a case of a 6-week-old infant who developed SS following a pulmonary infection. The patient presented with ocular manifestations and fully recovered after 1 week of corticosteroids.

Keywords: Infant, neutrophilic dermatoses, sweet syndrome

How to cite this article:
Rejeb MB, Boudaya S, Bahloul E, Frikha F, Amouri M, Turki H. Sweet Syndrome in Early Infancy. Indian J Paediatr Dermatol 2021;22:77-9

How to cite this URL:
Rejeb MB, Boudaya S, Bahloul E, Frikha F, Amouri M, Turki H. Sweet Syndrome in Early Infancy. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 22];22:77-9. Available from: https://www.ijpd.in/text.asp?2021/22/1/77/305805

Introduction

Sweet syndrome (SS) or acute febrile neutrophilic dermatosis is typically accompanied by fever and blood and tissue neutrophilia, leading to the development of painful erythematous violaceous skin lesions, and histopathologically by the presence of dermal neutrophilic infiltrate. It can easily be mistaken for infection and can lead to prescribe antibiotics or antiviral agents. Pediatric SS is rare, accounting for 5%–8% of cases,^[1] and infancy presentations are even rarer. We report a particular form of SS occurring in a 6-week-old female infant.

Case Report

A 6-week-old female infant with a medical history of facial angioma presented to the hospital with a 1-week history of a sudden onset of febrile inflammatory skin lesions. The cutaneous lesions had started on the face and neck and then spread to the trunk and limbs. The skin lesions developed at the same time as fever. There was a history of decreased appetite, productive cough, and rhinorrhea few days before the onset of lesions. There was neither history of drug intake nor previous signs, suggesting systemic involvement. During general examination, the infant was febrile (39°C), tachypneic (44 breaths/min), and tachycardic (150 beats/min). Lung auscultation revealed bronchial rales, and ophthalmological examination showed bilateral conjunctivitis. Otherwise, there was no palpable hepatosplenomegaly or lymphadenopathy. During cutaneous examination of the face, lower limbs, and abdomen, there were multiple, erythematous violaceous plaques and nodules with infiltrate and raised borders varying from 0.5 to 2 cm [Figure 1]. Most cutaneous lesions were surrounded by the epidermal collar peeled secondary to edema [Figure 2]. However, there was no mucous membrane involvement. Biological investigation revealed a microcytic hypochromic anemia with hemoglobin of 9.2 mg/dL, total count of 17,300 white blood cells/dL (no abnormal cells), elevated neutrophil count at 11,090/mL, and normal platelet count 203,000 per mm³. C-reactive protein was 138 mg / dL. Lumbar puncture and cytobacteriological examination of the urine were normal. The chest radiography showed interstitial opacities and bronchial walls thickening. A serology of Epstein–Barr virus, cytomegalovirus, and PVB19 was negative. The lactate dehydrogenase level was normal, and serum immunoglobulins were within normal limits. A skin biopsy demonstrated a dense neutrophilic infiltrate in the upper dermis with occasional eosinophils and exocytosis of neutrophils into the epidermis [Figure 3] and [Figure 4]. Minute foci of fibrinoid necrosis in the upper dermis have been noted. Based on both clinical and histopathologic findings, the infant was diagnosed with SS. Treatment for respiratory infection was started with cefotaxime (100 mg/kg/day) and vancomycin (40 mg/kg/day) associated with a cure of methylprednisolone administered (2 mg/kg/day) for 1 week. There was a good response to steroids with clear improvement of lesions with mainly a regression of edema, infiltration, and erythema [Figure 5] along with the disappearance of fever and respiratory and ocular symptoms. At 1-year follow-up, the infant had no recurrences and no underlying signs of a systemic disease.

Figure 1: Erythematous annular plaques with elevated borders over the trunk

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Figure 2: Erythematous plaques over the face surrounded by epidermal collar with bilateral conjunctivitis

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Figure 3: A dense neutrophilic infiltrate in the upper dermis with occasional eosinophils and exocytosis of neutrophils into the epidermis (H and E, ×200)

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Figure 4: A massive neutrophilic infiltrate in the upper dermis and exocytosis of neutrophils into the epidermis (H and E, ×50)

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Figure 5: Full recovery of cutaneous symptoms after just 1 week of systemic corticosteroid

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Discussion

SS is an acute febrile neutrophilic dermatosis which was first described by Robert Douglas Sweet in 1964. The cases reported in children are rare (8% of all cases) and equally distributed between the genders.^[1],[2] Neonatal and infantile SS are even more uncommon. According to literature, the youngest patient developed lesions at the age of 10 days.^[3] In infants, the dermatosis is usually preceded by symptoms of infection in the upper respiratory tract or gastrointestinal tract. The incubation period can vary from 1 to 3 weeks before the onset of cutaneous lesions.^[4] Some authors suggested that infectious agents might be triggers of the inflammatory response in cutaneous lesions.^[5] The diagnostic criteria were originally proposed by Su and Liu in 1986 and modified by Von Den Driesch in 1994 [Table 1]. Findings in patients must fulfill both the major criteria and at least two of the minor criteria for the diagnosis of SS.^[6] Revised diagnostic criteria have been published recently by Nofal et al., with only two required clinical and histopathological features for the diagnosis of SS even in the absence of the minor/variable features.^[6] In children, the syndrome is usually of the classic “parainfectious” subtype and commonly follows a respiratory or a gastrointestinal infection.^[7] In contrast to the adult population, in which 11%–33% of SS cases are paraneoplastic, the pediatric population appears to have a lower risk for hemoproliferative disorders such as myeloid or lymphoblastic leukemia.^[4],[7] Recently published cases noted a significant association between infantile recurrent SS and immunodeficiency, including HIV infection.^[2],[4] In addition to skin and mucosal lesions, SS can also be presented with extracutaneous manifestations, particularly when associated with malignancy. Eyes, neuromuscular system, joints, kidneys, lungs, heart, and liver are the most frequent extracutaneous sites reported in SS. However, such extracutaneous manifestations tend be rarer in children.^[4]

Table 1: Diagnostic criteria for sweet syndrome proposed by von Den Driesch

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First-line treatment for SS is based on systemic corticosteroid therapy typically dosed at 1–2 mg/kg/day for 4–6 weeks.^{[1],[2],[3],[8]} Full recovery of lesions without any scars in usually obtained in 2–4 weeks.^[9] Rarely, lesions heal leaving postinflammatory elastolysis or acquired cutis laxa.^[5] As to skin recurrence, it depends on the etiology and is mostly reported in infants who have a serious underlying condition.^[4],[8] In this case report, we highlight a new observation of a pediatric SS occurring in early infancy (at the age of 6 weeks). The diagnosis was based on clinical and histopathologic findings according to the most recent update of the diagnostic criteria of SS. Physical examination, repeated complete blood counts, and review of the peripheral smear allowed excluding a malignant blood disorders. The etiological investigation concluded to the most classic idiopathic presentation: parainfectious form following a bronchopulmonary infection. As for extracutaneous symptoms, our patient manifested with bilateral conjunctivitis which is exceptional at this age. We also noted rapid response to treatment with corticosteroids administered for a week period and no further recurrence of symptoms. Finally, even after the symptoms have regressed, infant should be followed up because SS may precede a malignancy, may accompany, or may reveal recurrent disease.^[1],[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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