Neonatal and Infantile Erythrodermas

Pruszkowski, A; Bodemer, Christine; Fraïtag, Sylvie; Teillac-Hamel, D.; Amoric, J C; Prost, Y. De

doi:10.1001/archderm.136.7.875

Cited by 113 publications

(20 citation statements)

References 23 publications

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“…Petechiae and purpura due to thrombocytopenia are typical signs in Wiskott-Aldrich syndrome; diarrhoea and vesiculobulbous lesions distributed in a periorificial and acral pattern on the face, the scalp, the hands, the feet and the anogenital areas are typical signs in acrodermatitis enteropathica; diarrhoea and periorificial eczematous eruption are typical signs in biotinidase deficiency. In contrast to these conditions, NS presents with hair shaft anomalies such as TI and others [18]. Although hyper-IgE syndrome had to be considered as one of the initial differential diagnosis of NS for our patients, it was excluded due to the absence of recurrent infections, especially cold abscesses, typical face appearance, defective neutrophil chemotaxis and dental or skeletal anomalies [11,14].…”

Section: Discussionmentioning

confidence: 99%

Netherton syndrome: report of identical twins presenting with severe atopic dermatitis

et al. 2006

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We report the cases of 4-year-old identical twin sisters who presented with severe atopic dermatitis with intractable skin manifestations and multiple food allergies. Netherton syndrome (NS) (OMIM 256500) was suspected due to very high serum IgE levels, growth retardation, severe food allergies and typical hair finding (trichorrhexis invaginata). A definite diagnosis was made by genetic analysis. Our cases are unique in being the first identical twins with NS diagnosed by a novel mutation in the SPINK5 gene. NS should be considered in differential diagnosis in children who have generalized erythema with intractable eczematous lesions and elevated levels of IgE.

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Section: Discussionmentioning

confidence: 99%

Netherton syndrome: report of identical twins presenting with severe atopic dermatitis

et al. 2006

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“…[6] could establish the etiological diagnosis only almost after 11 months of onset. This study observed that the cause of erythroderma in the neonate included immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton syndrome (18%), and eczematous or papulosquamous dermatitis (20%), and five patients (10%) had erythroderma of unknown origin.…”

Section: Causes Of Neonatal Erythrodermamentioning

confidence: 99%

Neonatal erythroderma: Diagnostic and therapeutic challenges

2012

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Erythroderma a life-threatening entity during the first one month, and many a time, a manifestation of genodermatosis, immune deficiency, psoriasis, metabolic diseases, and infections. Atopic dermatitis presenting as erythroderma is usually observed later, after this one-month period, and hence not a common differential for neonatal exfoliative dermatitis. Although a rare entity, there is a paucity of studies on this and in contrast to adults, some may manifest as cardinal signs of primary disease conditions.

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“…18% of 51 cases with neonatal and infantile erythrodermas were finally diagnosed as Netherton’s syndrome [27]. The main hair abnormality in Netherton’s syndrome was initially named bamboo hair and later called trichorrhexis invaginata.…”

Section: Hair Shaft Alterations In Ectodermal Dysplasiasmentioning

confidence: 99%

Hair Shaft Abnormalities – Clues to Diagnosis and Treatment

Itin¹,

Fistarol

2005

Dermatology

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Hair dysplasias are congenital or acquired alterations which often involve the hair shaft. Hair shaft abnormalities are characterized by changes in color, density, length and structure. Hair shaft alterations often result from structural changes within the hair fibers and cuticles which may lead to brittle and uncombable hair. The hair of patients with hair shaft diseases feels dry and looks lusterless. Hair shaft diseases may occur as localized or generalized disorders. Genetic predisposition or exogenous factors produce and maintain hair shaft abnormalities. Hair shaft diseases are separated into those with and those without increased hair fragility. In general, optic microscopy and polarized light microscopy of hair shafts provide important clues to the diagnosis of isolated hair shaft abnormalities or complex syndromes. To establish an exact diagnosis of dysplastic hair shafts, a structured history and physical examination of the whole patient are needed which emphasizes other skin appendages such as the nails, sweat and sebaceous glands. Profound knowledge on hair biology and embryology is necessary to understand the different symptom complexes. Therapy of hair shaft disorders should focus on the cause. In addition, minimizing traumatic influences to hair shafts, such as drying hair with an electric dryer or permanent waves and dyes, is important. A short hairstyle is more suitable for patients with hair shaft disorders.

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Neonatal and Infantile Erythrodermas

Cited by 113 publications

References 23 publications

Netherton syndrome: report of identical twins presenting with severe atopic dermatitis

Netherton syndrome: report of identical twins presenting with severe atopic dermatitis

Neonatal erythroderma: Diagnostic and therapeutic challenges

Hair Shaft Abnormalities – Clues to Diagnosis and Treatment

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