Principles and practice Care of the Infant with Sclererna Neonatorurn CECILIA KINSEL FERGUSON,
High-risk infants are particularly prone to sepsis which can be accompanied by sclerema neonatorum. This condition is manifested by skin hardening. Causation of hardening of a sick infant's skin must be identified for appropriate treatment to be implemented. Nursing care of the infant with sclerema is very complex. Throughout the course of the disease, the infant frequently requires antimicrobial therapy, ventilatory support, exchange transfusions, precise intake and output measurements, and temperature control. Psychosocial support for the family and infant is also an important part of the nurse's role. In spite of standard therapy, the mortality rate for infants with sclerema remains high. New advances, such as exchange transfusions, give some hope for the survival of these very ill infants.
High-risk neonates face many obstacles after birth. A somewhat rare, but often lethal, complication for the sick neonate is sclerema neonatorum. Neonatal specialists disagree on whether to classify sclerema as a primary disease or as only a symptom of grave underlying pathology. Regardless of its classification, sclerema is a condition with an extremely high mortality rate.' Sclerema is defined simply as a hardening of the skin and subcutaneous tissue. T h e skin of the affected areas becomes totally inflexible and pale, mottled, orjaundiced in color. T h e tissue hardening usually begins in the hands, thighs, and buttocks and then eventually involves the whole body except for the palms, soles, and genitalia. In the very small preterm infant, total body involvement is common. As the disease progresses, the neonate's body assumes a board-like rigidity and appears similar to a corpse.2
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has not been documented. But one could expect that as more low weight neonates (below 1500 grams) are surviving the first week of life, the potential for sepsis will increase. With increased incidence of sepsis comes an increased potential for the development of sclerema. Infants with sepsis seem to be at Infants at Risk particularly high risk for the deInfants who develop sclerema are velopment of sclerema. Immunocharacteristically very ill and alglobulins provide the first line of ready in a debilitated state. Suscepdefense for the infant against sepsis. IgG, the most abundant type of imtible infants include preterm or munoglobin, is normally transfull-term infants who have had an ferred from the mother to the fetus intracranial hemorrhage, pneuduring the third trimester of pregmonitis, a preexisting respiratory or nancy. Thus, a preterm infant gastrointestinal infection, cold stress, shock, and/or ~epticemia.'-~ misses the opportunity to receive this immunity. Maternal IgA does Sepsis occurs in approximately not cross the placental barrier. IgM one in 1000 live births in infants is produced by a 20-week old fetus weighing 2500 grams or more. In in only minute amounts. These infants weighing less than 1000 immunologic deficiencies increase grams, the incidence increases to the neonate's potential for infec74 in 1000 live birth^.^ T h e precise t i ~ n . ~ incidence of sclerema neonatorum Sclerema usually occurs in the first week of life, but can be seen anytime in the first few months after birth. T h e disease usually lasts three or four days and ends with the infant's death. If the infant survives, the cutaneous changes resolve within about two weeks.'
Table 1. Infantile Integumentary Diseases with Skin Hardening
Sclerema Neonatorum Etiology
Neonates 1-1 2 weeks of age. Initially cheeks, buttocks, thighs. Then involves whole body except palms, soles, genitalia.
Increase in saturated fat; decrease in unsaturated fat; low unsaturated fat causes fat solidification. No fat necrosis occurs. Nonspecific changes; edema and enlargement of fat panniculi. Lasts 3-4 days; cutaneous changes resolve in 2 weeks. Supportive with emphasis on underlying disease. Extremely poor.
Scleredema Unknown; usually follows acute infection; may be related to streptococcal hypersensitivity. 113 cases before age 10. 1/2 cases before age 20. Begins on neck, spreads to face, shoulders, chest.
Scleroderma (Morphea) Unknown.
Related to cold or obstetric trauma.
Well newborns, 1st to 2nd week life. Highly localized in areas where fat pads present: cheeks, neck, back, thighs, shoulders, buttocks. Altered fat secondary to ischemia; fat necrosis occurs.
Found on face, trunk, limbs.
Actual pathology obscure; cutaneous changes follow febrile illness.
Lymphocytic infiltrate around blood vessels and collagen bundles; thickened collagen replaces subcutaneous fat.
Normal appearing epidermis; swollen dermal collagen; normal blood vessels. Subsides in 3-24 months.
Localized atrophy and hyalinization.
No effective treatment.
Spontaneous resolution in 3-5 years, leaving a brown pigmentation. Time is only treatment.
Good; may be residual deformities.
One of the problems in identiT h e pathophysiological occurfying sclerema is its similarity to rences in sclerema are somewhat other diseases of the integumentary obscure: histological studies show system. When tissue hardening ocnonspecific changes including curs, other diseases that also must edema and enlargement of the sepbe considered include scleredema, tae of fat p a n n i c ~ l iAlso, . ~ a change scleroderma, and subcutaneous fat in the ratio of saturated fat (increases) to unsaturated fat (de- . necrosis' (Table 1). Differentiation of sclerema creases) occurs. T h e stimuli for this neonatorum from these similar inchange is thought to be related to tegumentary diseases is very ima neonatal deficiency of the enportant. T h e infant who is diagzymes necessary for the conversion nosed with sclerema has a quesof palmitic and stearic acids to oleic tionable chance for recovery; the acid. This results in the overainfant with scleredema, sclerobundance of saturated fats. Because derma, or subcutaneous fat necrosis of the low unsaturated fat content, will live. This information is of utfat solidification occurs-accountmost importance to the infant's ing for the hard wooden feel of the family who deserve a realistic exinfant's
Subcutaneous Fat Necrosis
Granulomatous infiltrate containing foreign body giant cells with fat crystals. Resolves spontaneously over weeks to months. No effective treatment; avoid hot packs to involved areas. Good.
pectation of the outcome of their infant's illness. N o specific laboratory tests are available to aid in the diagnosis of sclerema. T h e diagnosis is based primarily upon symptomatology and failure of the infant to respond favorably to standard medical treatment for the underlying illness.
Symptoms Knowing that high-risk infants have a predisposition to sepsis and, therefore, to sclerema, the nurse caring for these infants must be very observant for the development of early symptoms. Often, the infant is already being treated for sepsis but is not showing indications of improvement. T h e infant continues to have problems such as a fluc-
tuating temperature, apnea, unstable blood pressure, and/or poor feeding. Also, a slight seepage of fluid from the hands, thighs, or buttocks may begin. This then progresses to hardening of the tissue in these areas. Medical Treatment Once these symptoms are noted, a stronger approach should be taken. The identified underlying disorder must be battled vigorously by standard medical methods. In addition, recent research data document the need for even more aggressive treatment of these infant~.~” Exchange transfusions have been shown to decrease significantly the mortality rate of septic infants with sclerema. T h e positive response following exchange transfusions is believed to occur as a result of increased IgM and IgA levels, removal of endotoxins, improvement in perfusion and tissue oxygenation, and enhancement of humoral and cellular infammatory response.6 Another advance in the treatment of the infant with sepsis accompanied by sclerema involves transfusions of polymorphonuclear‘ leukocytes.’ This treatment is started as soon as symptoms of sepsis are evident and is continued on a daily basis until evidence of sepsis disappears. A significantly lower mortality rate was seen for infants with sclerema who received polymorphonuclear leukocyte transfusions than for those who received only conventional antibiotics and supportive therapy .’ Nursing Care Even though the medical treatment for infants with sclerema is very limited, the nursing responsibilities are vast. Planning therapeutic nursing care is based upon accurate assessment of the infant’s status. This can best be done by
November/December-1983 JOGN Nursing
Observe the infant’s behavior for subtle changes. Check for bowel sounds at frequent intervals. Infant Nursing Care Plan Assist with septic work-up if required. Problem One Administer prescribed doses of Objective Data Body temperature below 96°F antibiotics. rectally Assist with exchange transfusions as necessary. Pallor Transfuse with polymorphonuBradycardia clear leukocytes as ordered. Bradypnea Problem Three Cool skin Objective Data Nursing Diagnosis Decrease in urine output below Alteration in ability to maintain 1 to 2 cc/kg/hr. adequate temperature related Tense fontanels to decreased peripheral cirPitting edema in areas not hardculation manifested by preened by sclerema cipitous drop in temperature. Nursing Diagnosis Expected Outcome Alteration in renal perfusion reRectal temperature will return to lated to decreased systemic 98 to 99°F. circulation manifested by deNursing Intervention creased urine output. Increase temperature of radiant Expected Outcome warmer or isolette. Infant’s urinary output will avCheck infant’s rectal temperaerage 1 to 2 cc/kg/hr. ture every 30 minutes (axillary Nursing Intervention temperature unreliable due to Accurately assess infant’s hourly decreased peripheral circulaintake and output utilizing tion). diapers weighed on a gram Use heated floatation mattress. scale before and after use. Apply heat cradle or appropriate (Because of stiffening of the substitute. limbs in sclerema, it is difficult Decrease drafts around radiant to utilize a pediatric urine colwarmer. lector). Problem Two Measure urine specific gravity Objective Data every four hours. Hypo- or hyperthermia Closely monitor IV intake. Change in behavior Closely monitor infant’s BUN, Increasing periods of apnea creatinine, and potassium. Decreasing bowel sounds Problem Four Nursing Diagnosis Alteration in ability to fight in- . . Objective Data Tachypnea with shallow respifection related to decreased ratory movement. immunologic function in the Bradypnea following a period of newborn. tachypnea (signals onset of reExpected Outcome spiratory failure). Infant’s temperature will stabiDeteriorating changes in infant’s lize. arterial blood gas values. Infant’s blood cultures will rePooling of fluids in the lungs. main negative. Nursing Diagnosis Nursing Intervention Alteration in respiratory funcClosely assess infant’s temperation related to tissue hardenture.
identification of a nursing diagnosis for each of the infant’s problems.
ing in the chest wall causing inability of chest wall movement. Expected Outcome Infant’s respiratory rate will remain between 30 to 60 per minute. Nursing Intervention Accurately assess respiratory rate on an hourly basis. Closely monitor arterial blood gases and report findings. Perform frequent chest auscultation to assess for pooling of fluids in the infant’s lungs. Suction the infant frequently since infants do not cough effectively. Prepare infant for possible endotracheal intubation with placement on respirator. Problem Five Objective Data Increase in abdominal girth Absence of bowel sounds Nursing Diagnosis Dysfunction in activity of gastrointestinal system related to generalized ischemia of bowel. Expected Outcome Infant will have active bowel sounds. Nursing Interoention Regularly measure abdominal girth. Keep infant on NPO status. Place nasogastric tube to gravity drainage to promote gastric decompression. Provide infant with IV hyperalimentation o r IV fluids t o provide for infant’s caloric and/or Auid needs. Problem Six Objective Data Reddened areas on skin Broken areas on skin Stiffening of extremities Nursing Diagnosis Alteration in skin and muscle in-
tegrity related to decreased peripheral blood flow a n d hardening of subcutaneous tissue. Expected Outcome Infant’s integumentary system will remain intact. Nursing Intervention Frequently change infant’s position by turning infant or using a roto-kinetic bed. Utilize water mattress to decrease pressure on infant’s skin. Use a sponge doughnut under infant’s head to maintain neck alignment. Utilize regular passive range of motion exercises to improve circulation. Problem Seven Objective Data Parental refusal to see infant. Parental hope alternating with periods of despair. Nursing Diagnosis Dysfunction of parental ability to cope with infant’s deteriorating condition related to lack of preparation for seriousness of infant’s status. Expected Outcome Infant’s parents will slowly come to accept the condition of their infant. Nursing Intervention Maintain honesty with parents at all times. Encourage parental contact with infant while providing support for them during visits. Respect family’s need for grieving and for privacy. Make appropriate referrals to Social Service Department or Spiritual Care Department. Provide for follow-up home visits with family after infant’s death or dismissal from hospital.
Summary Once the nurse has cared for infants with sclerema, it is easy to become pessimistic regarding the outcome of every baby with this disease. T h e mortality rate for infants with neonatal sepsis accompanied by sclerema remains very high: the rate has not changed over the last five years in spite of aggressive antibiotic therapy for septic infant^.^" But, with the help of new supportive therapy and excellent nursing care, some infants are surviving. All nursing care is planned and implemented with hope for each infant’s survival.
References 1. Behrman RE. Neonatology. 2nd ed. St. Louis: CV Mosby Co., 1977. 2. Rudolph A M . Pediatrics. N e w York: Appleton-Century-Crofts, 1977. 3. Paxson CL, ed. van Leeuwen’s newborn medicine. 2nd ed. Chicago. Year Book Medical Publishing, Inc., 1979. 4. Schaffer A, Avery ME. Diseases of the newborn. Philadelphia: WB Saunders Co., 1977. 5. Rosen F. Neonatal immunity. In: Smith C, Nelson N , eds. The physiology of the newborn infant. Springfield: Charles C Thomas, 1976. 6. Vain Nestor S, et al. Role of exchange transfusion in the treatment of severe septicemia. Pediatrics 1980;66:693-7. 7. Laurenti F, et al. Polymorphonuclear leukocyte transfusion for the treatment of sepsis in the newborn infant. J Pediatr 1981;98:1 18-22. Address for correspondence: Cecilia Kinsel Ferguson, RNC, MSN 5702 Bogart Drive, San Antonio, T X 78240. Cecilia Kinsel Ferguson is an associate professorof nursing at San Antonio College in Texas. Ms. Ferguson is a member of ANA and Sigma Theta Tau.
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