Sclerema Neonatorum in a Full-Term Infant Showing Favorable Prognosis

Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. The prognosis of SN is generally very poor, with a high fatality rate. We report the case of a full-term infant with delayed onset of SN showing good prognosis. A 4-month-old Korean male infant presented with diffuse waxy, sclerotic skin lesions on the whole body, sparing the face, nipples, and genital area, which began developing at 2 months of age. Histopathologic findings of the sclerotic skin lesions showed wide, fibrous intersecting bands in the subcutaneous fat tissue. Only sparse infiltration of lymphocytes and histiocytes was observed in the fat lobules and septa. Based on clinical presentation and histopathologic findings, he was diagnosed with SN. The patient survived with conservative care and had mild improvement of the skin lesions on his follow-up visit at 12 months of age.

Clinical study of renal function of newborns with sclerema neonatorum / 中国综合临床

Objective To investigate the changes of urinary brush border membrane enzymes and serum cystatin C (Cys C) in newborns in order to develop early diagnostic indicators for sclerema neonatorum (SN) renal dysfunction.Methods Sixty-night cases with sclerema neonatorum and 30 cases of normal newborns were enrolled in this study.Of all sclerema neonatorum cases,39 cases were mild and 30 cases were moderate to severe sclerema neonatorum.Immunoca talytic assay method was adapted to detect the level of urinary brush border membrane.The levels of serum Cys C,blood urea nitrogen (BUN),creatinine (Cr) and urinary β32 microglobulin (β2-MG) in two groups were also measured.Results The levels of urinary brush border membrane enzyme,β2-MG and serum Cys C in sclerema neonatorum group were (40.09 ± 7.29) U/L,(4.65 ± 1.33) mg/L and (1.84 ± 0.32) mg/L,higher than those in control group ((23.19 ± 5.62) U/L,(2.49 ± 0.77) mg/L and (1.07 ± 0.25) mg/L; t =10.34,7.47 and 10.55,P ＜ 0.01).The levels of urinary brush border membrane enzyme and serum Cys C in moderate to severe sclerema neonatorum group were (42.06 ± 7.59) U/L and (1.93 ± 0.34) mg/L,higher than those in the mild group ((38.57 ± 6.70) U/L,(1.77 s0.29) mg/L;t =2.24,2.11,P ＜0.05).The abnormality rates of urinary brush border membrane enzyme and serum Cys C in sclerema neonatorum group were 79.7％ (55/69) and 72.5％ (50/69),higher than β2-MG abnormal rate (52.2％ (36/69),x2 =12.95,12.11,P ＜ 0.01).In sclerema neonatorum group,urinary brush border membrane enzyme was positively correlated with β2-MG (r =0.560,P ＜ 0.01),and serum Cys C was positively correlated with BUN,Cr (r =0.314,0.287,P ＜ 0.05).Conclusion Renal injury is common in SN.Urinary brush border membrane enzyme and serum Cys C are served as the diagnostic parameters for early detection of renal injury in SN.