Sclerema Neonatorum in a Term Infant: A Case Report and Literature Review

Author:

Nakalema Gloria1ORCID,Egesa Walufu Ivan1ORCID,Kumbakulu Patrick Kumbowi1ORCID,Nduwimana Martin1ORCID,Anaya Amnia Diaz2,Kizito Mirembe Stephen3,Kavuma Daniel4

Affiliation:

1. Department of Paediatrics and Child Health, Faculty of Clinical Medicine and Dentistry, Kampala International University, Kampala, Uganda

2. Department of Pathology, Faculty of Clinical Medicine and Dentistry, Kampala International University, Kampala, Uganda

3. Department of Dermatology, Faculty of Clinical Medicine and Dentistry, Kampala International University, Kampala, Uganda

4. Department of Surgery, Faculty of Clinical Medicine and Dentistry, Kampala International University, Kampala, Uganda

Abstract

Sclerema neonatorum (SN) is a rare form of panniculitides with an obscure incidence, aetiology, and pathogenesis. This condition is peculiar to preterm neonates, especially those with comorbidities such as sepsis, congenital anomalies, congenital heart disease, and gastrointestinal and respiratory diseases. Usually, it presents within the first seven days of life, but may develop a few weeks later. Typically, SN manifests with diffuse hardening of the skin and subcutaneous tissues that subsequently bind to the underlying muscle and bone, often beginning in the buttocks, thighs, or trunk, and progressing to other body parts, but sparing the soles, palms, and genitalia. Diagnosis is based on clinical characteristics. It has been associated with a high mortality, despite aggressive use of various treatment modalities such as antibiotics, steroids, fresh blood transfusion, exchange blood transfusion, and intravenous immunoglobins. This is a report of a macrosomic term neonate who presented with typical clinical and histopathological features of SN.

Publisher

Hindawi Limited

Subject

General Medicine

Reference28 articles.

1. Panniculitis: A dermatopathologist's perspective and approach to diagnosis

2. Sclerema neonatorum

3. Effect of fresh blood transfusion in the treatment of sclerema neonatorum;E. Sarwono;Paediatrica Indonesiana,1973

4. Sclerema neonatorum. A report of nine cases;G. Villacorte;The Ohio State Medical Journal,1967

5. Sclerema neonatorum: report of an unusual case;J. Joncas;Canadian Medical Association Journal,1959

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