We were experienced a case of sclerema neonatorum which was developed in a 13 days old newborn, and we report it with a brief review.
Humans ; Infant, Newborn ; Sclerema Neonatorum*

Sclerema neonatorum is an uncommon and fatal disease which encountered in premature & debilitated newborn infants. Recently we experienced a case of sclerema neonatorum in premature infant with congenital heart disease which was confirmed by autopsy. We present a case with a brief review of literatures.
Autopsy ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Infant, Premature ; Sclerema Neonatorum*

Sclerema neonatorum is a lesion of degeneration, necrosis or crystallization of adipose tissue of new born, and is classified into two types; the generalized type in which death occurs usually within one week without treatment, and the nodular type which is localized and heals spontaneously. In this report, nodular type was presented. Nut to palm sized indurated plaques on deltoid region, upper back and hip were noted 3 weeks after birth. Histopathologic findings of necrosis of subcutaneous tissue with early granulomatous reaction were showed in biopsy specimen. Literature was briefly reviewed for the discussion of clinical and microscopic findings of this disease.
Adipose Tissue ; Biopsy ; Crystallization ; Hip ; Necrosis ; Nuts ; Parturition ; Sclerema Neonatorum ; Subcutaneous Tissue

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.
Follow-Up Studies ; Genitalia ; Histiocytes ; Humans ; Infant* ; Lymphocytes ; Male ; Nipples ; Prognosis* ; Rare Diseases ; Sclerema Neonatorum* ; Skin ; Subcutaneous Fat

Subcutaneous fat necrosis of newborn is an uncommon disorder characterized by firm subcutaneous plaques and nodules usually appearing shortly after birth. A female newborn with brain death developed diffuse hardening of trunk, extremity, cheek and buttock in the first 5 weeks of the life. A clinical diagnosis of sclerema neonatorum was made. But histologic examination revealed patchy areas of fat necrosis with crystallization, infiltration of histologic cells and several multinucleated giant cells containing needle-shaped clefts. Calcium deposits were also demonstrated within the necrotic area. So, we diagnosed this case as subcutaneous fat necrosis of newborn with generalized hardening of the skin.
Brain Death ; Buttocks ; Calcium ; Cheek ; Crystallization ; Diagnosis ; Extremities ; Fat Necrosis ; Female ; Giant Cells ; Humans ; Infant, Newborn* ; Necrosis* ; Parturition ; Sclerema Neonatorum ; Skin ; Subcutaneous Fat*