You are in: eMedicine Specialties > Dermatology > PEDIATRIC DISEASES Sclerema NeonatorumArticle Last Updated: Oct 2, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Amy J Theos, MD, Director of Pediatric Dermatology, Assistant Professor, Department of Dermatology, University of Alabama at Birmingham Amy J Theos is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Pediatric Dermatology Coauthor(s): Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham Editors: Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: SN, skinbound disease, skin-bound disease, subcutaneous fat disorder, neonatal subcutaneous fat disorder, neonatal subcutaneous fat disease INTRODUCTIONSection 2 of 10
  BackgroundThe classic description of sclerema neonatorum (SN) is credited to Underwood, who described it in 1784 and appropriately termed it "skinbound disease." In 1817, Alibert introduced the term sclerema, derived from the Greek word skleros, meaning hard. SN is a disorder of the subcutaneous fat in debilitated neonates and is considered best as a sign of a potentially fatal underlying disease process and not a specific disease entity (Warwick, 1963). PathophysiologyIn an infant, fat has a higher saturated-to-unsaturated fatty acid ratio compared to adult fat and thus, a higher melting point. Prematurity, hypothermia, shock, and metabolic abnormalities have been postulated to further increase this ratio, possibly as a result of enzymatic alteration allowing precipitation of fatty acid crystals within the lipocytes. This condition has been suggested to result in the dramatic clinical findings in affected skin. X-ray diffraction techniques have confirmed that infants with SN have an increase in saturated fats and that the crystals within the fat cells are composed of triglycerides (Kellum, 1968). FrequencyUnited StatesThe exact incidence of SN is unknown. All studies describe SN as extremely rare. The number of reported cases in recent years has declined, probably as a result of better neonatal care. Mortality/MorbidityBecause SN invariably is associated with serious underlying disease, the mortality rate is high. In different series, the reported mortality rates range from 67-88%, with death occurring hours to days after onset. If the underlying disease is treated successfully, the skin softens and returns to normal. RaceNo racial predilection has been reported. SexSN shows a slight male predominance, with an estimated male-to-female ratio of 1.5:1. AgeSN is a disease confined to the newborn period. SN can present at birth, but onset within the first week of life is more common. The oldest reported infant presented with Pseudomonas septicemia at age 106 days. CLINICALSection 3 of 10
HistoryOne half of affected infants are premature, and the others are full term but have a serious underlying illness. They are often of low birth weight (<2500 g) and have cyanosis and low Apgar scores (Milunsky, 1966). In one series, 75% of the mothers were healthy, while 25% had preeclampsia, placenta previa, or infection. Labor is usually normal, and delivery is spontaneous and nontraumatic. PhysicalPhysical findings appear suddenly, first on the thighs and buttocks and then, spreading rapidly, often affecting all parts of the body except the palms, soles, and genitalia. The involved skin is pale, waxy, and firm to palpation. The skin cannot be pitted or pinched up because it is bound to the underlying tissues. The affected infant often displays flexion contractures at the elbows, knees, and hips; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement. CausesAssociated underlying conditions include pneumonia, septicemia, hypothermia, metabolic acidosis, respiratory distress syndrome, congenital heart defects, gastroenteritis, and intestinal obstruction. DIFFERENTIALSSection 4 of 10
Hutchinson-Gilford Progeria Scleredema Subcutaneous Fat Necrosis of the Newborn
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