Sclerema Neonatorum and Subcutaneous Fat Necrosis of the Newborn

Fretzin, David F.; Arias, Angelito M.

doi:10.1111/j.1525-1470.1987.tb00764.x

Cited by 55 publications

(31 citation statements)

References 42 publications

(12 reference statements)

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“…These include Pena-Shokeir syndrome [Pena and Shokeir, 1974a], Neu-Laxova syndrome [Neu et al, 1971;Laxova et al, 1972], cerebro-oculofacio-skeletal (COFS) syndrome [Pena and Shokeir, 1974b], and lethal multiple pterygium syndrome [Hall, 1984]. Others include skin dysplasia, e.g., aplasia cutis congenita [Frieden, 1986], scleredema [Fretzin and Arias, 1987], Paraná hard-skin syndrome [Cat et al, 1974], or stiff skin syndrome [Esterly and McKusick, 1971]. Absence of neurological abnormalities, the differences in clinical presentation, and the characteristic histopathological and ultrastructural findings distinguish restrictive dermopathy from those syndromes.…”

Section: Discussionmentioning

confidence: 98%

Restrictive dermopathy: Report and review

et al. 1997

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Restrictive dermopathy (RD) is a lethal autosomal recessive genodermatosis (MIM No. 275210) in which tautness of the skin causes fetal akinesia or hypokinesia deformation sequence (FADS). Polyhydramnios with reduced fetal movements is followed by premature delivery at around 31 weeks gestation. Manifestations include a tightly adherent, thin, translucent skin with prominent vessels, typical facial changes, generalized joint contractures, enlarged fontanelles, dysplasia of clavicles, respiratory insufficiency, and an enlarged placenta with short umbilical cord. Histologic abnormalities of the skin include thin dermis with paucity and hypoplasia of the appendages and abnormally arranged collagen bundles. Elastic fibers are nearly missing. The subcutaneous fat is slightly increased. These skin findings usually appear after 22 or 24 weeks of gestation, which is why prenatal diagnosis with skin biopsy may fail. This disease is easily differentiated from other congenital FADS, such as Pena-Shokeir syndrome, COFS syndrome, Parana hard-skin syndrome, etc. We report on an affected boy of consanguineous parents and 30 previous cases are reviewed.

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Section: Discussionmentioning

confidence: 98%

Restrictive dermopathy: Report and review

et al. 1997

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“…The main clinical differential diagnosis of SFCN is SN; these two pathologies may be distinguished because children with SN are usually premature neonates, they are severely ill, and the skin biopsy shows no fat necrosis [5]. Erythema nodosum also has to be considered in the differential diagnosis; this is a septal panniculitis, with little fat necrosis of the lobules and no crystals.…”

Section: Discussionmentioning

confidence: 99%

Effectiveness of pamidronate in severe neonatal hypercalcemia caused by subcutaneous fat necrosis: a case report

et al. 2008

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Subcutaneous fat necrosis of the newborn (SCFN) is a panniculitis that develops in fatty areas during the first weeks of life after foetal distress or perinatal complications. Prognosis is generally good with complete regression, but it can be complicated by metabolic abnormalities like hypoglycemia, hypertriglyceridemia, thrombocytopenia, and also potentially life-threatening hypercalcemia. We report a case of severe hypercalcemia complicating SCFN in a newborn who was treated with hyperhydration, furosemide, prednisone, and pamidronate.

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“…They can prompt a granulomatous inflammatory response. These crystals are seen in subcutaneous fat necrosis of the newborn and PSP 15–17 . The exact mechanism of PSP remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Poststeroid panniculitis

et al. 2007

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Poststeroid panniculitis (PSP) is a rare complication of systemic corticosteroid therapy. Clinically, erythematous nodules and indurated plaques develop on the cheeks of children within days or weeks following rapid systemic steroid tapering or cessation. The clinical differential diagnosis of childhood cheek erythema is broad. However, PSP can be identified by clinical history and, if necessary, with a biopsy. Histologically, PSP presents as lobular panniculitis with a mixed inflammatory infiltrates without vasculitis. Needle-shaped clefts within adipocytes are characteristic. The histological differential diagnosis of adipocytes containing needle-shaped crystals is limited. We describe a case of poststeroid panniculitis and discuss the clinicopathological features and pertinent differential diagnoses.

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Sclerema Neonatorum and Subcutaneous Fat Necrosis of the Newborn

Cited by 55 publications

References 42 publications

Restrictive dermopathy: Report and review

Restrictive dermopathy: Report and review

Effectiveness of pamidronate in severe neonatal hypercalcemia caused by subcutaneous fat necrosis: a case report

Poststeroid panniculitis

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