Neonatal erythroderma

被引:20
作者
Fraitag, Sylvie [1 ]
Bodemer, Christine [2 ]
机构
[1] Hop Necker Enfants Malad, Dept Pathol, Serv Anat Pathol, F-75743 Paris 15, France
[2] Univ Paris 05, AP HP, Dept Dermatol,Hop Necker Enfants Malad, Natl Reference Ctr Genodermatosis MAGEC, Paris, France
关键词
Erythroderma; ichthyoses; Netherton syndrome; Omenn syndrome; skin biopsy; CONGENITAL ICHTHYOSIFORM ERYTHRODERMA; VERSUS-HOST DISEASE; SEVERE COMBINED IMMUNODEFICIENCY; COMBINED IMMUNE-DEFICIENCY; NETHERTON-SYNDROME; OMENN-SYNDROME; DIFFERENTIAL-DIAGNOSIS; MUTATIONS; GENE; EXPRESSION;
D O I
10.1097/MOP.0b013e32833bc396
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Purpose of review Neonatal erythroderma is a potentially life-threatening condition in neonates less than 1 month old. During the first month of life, erythroderma is generally a presentation of genodermatosis, primary immune deficiency, or, more exceptionally, severe psoriasis, metabolic disease or infection. Atopic erythroderma is observed later in life, usually after the age of 1 month. Rapid determination of the underlying cause is crucial for better management. However, the diagnosis is often a challenge for the clinician and is frequently delayed due to the nonspecific nature of the clinical signs. We summarize the different causes of neonatal erythrodermas and list their clinical, biological, histological, and sometimes genetic characteristics. Recent findings Severe erythroderma, typified by early onset, skin induration, severe alopecia and failure to thrive, is immediately suggestive of immunodeficiency or Netherton syndrome. In such cases, an early skin biopsy may be particularly of use in allowing accurate differentiation between these two disorders. Summary This review outlines the clinical and histological features of these disorders and suggests an approach to their differential diagnosis and management.
引用
收藏
页码:438 / 444
页数:7
相关论文
共 34 条
[1]   IN-UTERO ACUTE GRAFT-VERSUS-HOST DISEASE IN A NEONATE WITH SEVERE COMBINED IMMUNODEFICIENCY [J].
ALAIN, G ;
CARRIER, C ;
BEAUMIER, L ;
BERNARD, J ;
LEMAY, M ;
LAVOIE, A .
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 1993, 29 (05) :862-865
[2]   LEKTI proteolytic processing in human primary keratinocytes, tissue distribution and defective expression in Netherton syndrome [J].
Bitoun, E ;
Micheloni, A ;
Lamant, L ;
Bonnart, C ;
Tartaglia-Polcini, A ;
Cobbold, C ;
Al Saati, T ;
Mariotti, F ;
Mazereeuw-Hautier, J ;
Boralevi, F ;
Hohl, D ;
Harper, J ;
Bodemer, C ;
D'Alessio, M ;
Hovnanian, A .
HUMAN MOLECULAR GENETICS, 2003, 12 (19) :2417-2430
[3]  
BODEMER C, 1991, MED INFANTILE, V1, P25
[4]  
BURTON JL, 1992, ROOK WILKINSON EBLIN, V1, P537
[5]  
CADOZ M, 1982, PATHOL BIOL, V30, P522
[6]   CONGENITAL CUTANEOUS CANDIDIASIS [J].
CHAPEL, TA ;
GAGLIARDI, C ;
NICHOLS, W .
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 1982, 6 (05) :926-928
[7]   Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome [J].
Chavanas, S ;
Bodemer, C ;
Rochat, A ;
Hamel-Teillac, D ;
Ali, M ;
Irvine, AD ;
Bonafé, JL ;
Wilkinson, J ;
Taïeb, A ;
Barrandon, Y ;
Harper, JI ;
de Prost, Y ;
Hovnanian, A .
NATURE GENETICS, 2000, 25 (02) :141-142
[8]  
CONNELLY EA, 2006, TXB PEDIAT DERMATOLO, V1, P105
[9]   Identical mutations in RAG1 or RAG2 genes leading to defective V(D)J recombinase activity can cause either T-B-severe combined immune deficiency or Omenn syndrome [J].
Corneo, B ;
Moshous, D ;
Güngör, T ;
Wulffraat, N ;
Philippet, P ;
Le Deist, F ;
Fischer, A ;
de Villartay, JP .
BLOOD, 2001, 97 (09) :2772-2776
[10]   Mutations in CHD7 in patients with CHARGE syndrome cause T-B plus natural killer cell plus severe combined immune deficiency and may cause Omenn-like syndrome [J].
Gennery, A. R. ;
Slatter, M. A. ;
Rice, J. ;
Hoefsloot, L. H. ;
Barge, D. ;
McLean-Tooke, A. ;
Montgomery, T. ;
Goodship, J. A. ;
Burt, A. D. ;
Flood, T. J. ;
Abinun, M. ;
Cant, A. J. ;
Johnson, D. .
CLINICAL AND EXPERIMENTAL IMMUNOLOGY, 2008, 153 (01) :75-80