Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka

Abstract

Sclerema is associated with a high fatality rate and scleremic infants with diarrhoea who present with septic shock are at a greater risk of death.

Recurrent Sclerema in a Young Infant Presenting with Severe Sepsis and Severe Pneumonia: An Uncommon but Extremely Life-threatening Condition

“…Unlike our case infant, the majority of cases die within hours or days of the onset of the condition, regardless of intensive treatment (1,2). Septic shock is the most significant predictor of death probably due to the compromised and ineffective peripheral circulation (4,11). Prematurity, hypothermia, hypoxia, abdominal distension, and pneumonia have been found to be other factors associated with a high fatality rate in scleremic infants (4).…”

“…However, the magnitude of the problem is still unknown. It is more frequently observed in neonates but has also been reported in older infants (1,2,4). Sclerema is characterized by the deposition of crystals, primarily of triglycerides, in the subcutaneous fat that can be determined by x-ray diffraction; however, some studies reported subcutaneous fibrosis, not crystallization of fat contents, as the conspicuous feature (3,6,7).…”

“…It is a life-threatening condition which occurs in young infants with overwhelming sepsis, hypothermia, hypoxaemia, hyponatraemia, acidosis, and hypocalcaemia (1). The reported case fatality from sclerema in different series ranged from 30% to 100% (2-4), and deaths usually occur within hours to days of onset of the condition (5). Although sclerema no longer exists in developed countries, it still develops in young infants in resource-poor countries, like Bangladesh.…”

“…As the progression of sclerema is very rapid, aggressive management is a sine qua non to reduce the morbidity and deaths from this condition (1,8). Important aspects of management of sclerema include intensive therapy for sepsis, circulatory support, blood transfusion, and correction of fluid and electrolyte imbalance (1,4,8). Although no randomized trials have been done to evaluate the efficacy of any management of sclerema till date, transfusion of fresh blood with or without exchange has been shown to have dramatic response in young infants with sclerema (9-11).…”

Neonatal dermatological emergencies

“…Hypothermia, severe malnutrition, septic shock, serum ammonia and C-reactive protein levels are considered as predictors of mortality. [70] Clinical features: The disease manifests during first 1-2 weeks of life in severely debilitated term and preterm infants. It is characterized by sudden onset of diffuse hardening of skin initially involving the lower legs and later spreading to thighs, buttocks, trunk, and cheeks.…”