Delayed-Onset Sclerema Neonatorum in a Critically Ill Premature Infant.

Sclerema neonatorum is extremely rare in the 21st century. We report a premature infant managed in a neonatal intensive care unit with delayed development of sclerema neonatorum.

Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.

Sclerema neonatorum (SN) is a rare neonatal panniculitis that typically develops in severely ill, preterm newborns within the first week of life and often is fatal. It usually occurs in preterm newborns with delivery complications such as respiratory distress or maternal complications such as eclampsia. Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining. Similar to ET, intravenous immunoglobulin (IVIG) enhances both humoral and cellular immunity and thus may decrease mortality associated with SN. We report a case of SN in a term newborn who subsequently developed septicemia. Biopsy showed subcutaneous, needle-shaped clefts without associated necrosis, inflammation, or calcifications. Treatment with IVIG led to notable but short-term clinical improvement. Sclerema neonatorum remains a poorly understood and difficult to treat neonatal disorder. Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management.

OBJECTIVE: To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema. STUDY DESIGN: PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized. RESULT: SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN. CONCLUSION: The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

[Adiponecrosis subcutanea neonatorum in congenital pneumonia]. / Adiponecrosis subcutanea neonatorum bei konnataler Pneumonie.

In the course of a connatal pneumonia, a 7-day-old female newborn developed symmetrical subcutaneous nodules on her back, shoulders, and upper arms. These skin lesions were accompanied by hypercalcemia. Histological examination confirmed the putative clinical diagnosis of subcutaneous fat necrosis of the newborn. We discuss the differential diagnoses, therapeutic strategies, and prognosis of this uncommon disorder of the fat tissue.

[Dermatological particularities and pathologies of premature infants]. / Particularités et pathologies dermatologiques du nourrisson prématuré.

In contrast with the full-term infant, the skin of the preterm neonate is structurally and functionally immature, especially birth occurred before 30 weeks gestation. The inefficiency of the epidermal barrier may result in dehydration, thermal instability and toxic reactions from percutaneous absorption of topically applied agents. An increased risk for bacteremia and sepsis exist because of the easily injured skin, combined with compromised immunity. The present article summarizes the consequences of this skin immaturity and the different means to avoid them. We shall also describe 2 pathologies more frequent in premature infants: sclerema neonatorum and acquired zinc deficiency.

A case of Hutchinson-Gilford progeria syndrome mimicking scleredema in early infancy.

We report a case of Hutchinson-Gilford progeria syndrome (HGPS). The patient showed the characteristics of scleredema at the age of 2.5 months but developed all the manifestations of HGPS gradually until 10 months old. The possibility of development of HGPS should by considered in any case of scleredema at birth or in early infancy.

Sclerema neonatorum--histopathologic study.

Histopathological study of 10 cases sclerema neonatorum showed subcutaneous fibrosis with lobulation in all the cases. 8 (80 percent) cases showed non-specific chronic inflammation of dermis and thinning of epidermis with atrophy of rete pegs and hypercollgenisation of dermis was evident in 70 percent cases. Fat necrosis with crystallisation of fat content was not a conspicuous feature.

Sclerema neonatorum and subcutaneous fat necrosis of the newborn in the same infant.

Sclerema neonatorum (SN) is a rare disease of the newborn characterised by diffuse hardening of the subcutaneous adipose tissue. The condition is distinct from the localised violacious nodules observed in subcutaneous fat necrosis of the newborn (SCFN). Both conditions were diagnosed within the first 2 weeks of life in the same infant. SN and SCFN are unusual conditions in the newborn, the former usually associated with a severe clinical illness, following delivery or major surgery. The skin lesions of these two conditions are distinct and do not usually occur simultaneously. SN is associated with a poor prognosis.

Sclerema neonatorum and subcutaneous fat necrosis of the newborn.

Sclerema neonatorum and subcutaneous fat necrosis are rare disorders affecting the panniculus of the newborn. This review attempts to put into perspective their similarities and differences in light of historical, biochemical, pathologic, and etiologic considerations. Recent therapeutic modalities and the prognosis are discussed.

Scleredema in an infant.

A 3-month-old infant with scleredema had associated cytomegalovirus pneumonia with a rapidly fatal course. Because differentiation of scleredema from scleredema neonatorum, subcutaneous fat necrosis, and scleroderma may be difficult, histologic diagnosis is important. Cetylpyridinium chloride was used successfully as a fixative to demonstrate acid mucopolysaccharides histologically.

Sclerema neonatorum. Light and electron microscopic studies.

Specimens from four cases of sclerema neonatorum were studied by light and electron microscopy. The connective tissue bands of the cutis and subcutis were thickened and alternated with loosely distributed bands in broad, distended areas of basal substance. Extensive areas areas filled with cross-banded structures were observed in the dermis. In the interlobular septa of the subcutaneous and perirenal fat splitting of fibers into microfibrils was visible. Changes in the capillaries and small blood vessels were also noted. Neither fat cells filled with rosettes of needle-like crystals, nor any inflammatory infiltrates of foreign body type giant cells were found in subcutaneous tissue.

Subcutaneous fat necrosis of the newborn.

Subcutaneous fat necrosis of the newborn (SFNN) developed in a 1-week-old black boy. His mother had received numerous medications for eclampsia. Birth was by Caesarean section and complicated by meconium aspiration. There were numerous nodules over the back, buttocks and extremities that yielded a caseous-like material. Microscopically, these nodules showed crystallization and necrosis of the fat. Hypoglycemia, pneumonia, oliguria, thrombocytopenia, seizures and urinary infection were associated with the cutaneous problem and led to a fatal outcome 2 weeks after birth.