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.

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.