Differential Diagnosis of Neonatal Erythroderma

Ott, Hagen; Hoeger, Peter H.

doi:10.1002/9781444345384.ch11

Cited by 3 publications

(7 citation statements)

References 66 publications

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“…Neonatal erythroderma is uncommon. Such patients are at risk of fluid loss, leading to hypernatraemic dehydration, hypothermia and increased risk of infections 1 . Causes can be divided into inflammatory (atopic dermatitis, psoriasis, seborrhoeic dermatitis), infectious (staphylococcal scalded skin syndrome, candidiasis), drug‐induced, genodermatoses and immunodeficiency diseases, such as OS, GVHD, DiGeorge syndrome and Wiskott–Aldrich syndrome 1 …”

Section: Discussionmentioning

confidence: 99%

Neonatal erythroderma

et al. 2020

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A 3-month-old boy presented with erythroderma. He had been born at term following an uncomplicated pregnancy; his parents were consanguineous. At the age of 2 weeks, he developed a widespread erythematous scaly rash, and from the age of 4 weeks, he developed recurrent respiratory tract infections and intermittent diarrhoea. By the age of 3 months, his weight had dropped dramatically to the 0.4% centile, he was failing to thrive, had a hepatosplenomegaly and lymphadenopathy of unknown origin and required admission to the paediatric high dependency unit.Physical examination revealed a nonpruritic erythematous scaly eruption, which extended to cover the entire body surface area (Fig. 1) and widespread scale on the scalp with alopecia (Fig. 2).Laboratory investigations revealed hypogammaglobulinaemia [IgG 0.56 g/L (normal range 2.4-8.8 g/L), IgA < 0.03 g/L (0.10-0.50 g/L), IgM 0.06 g/L (0.20-1.00 g/L)], while IgE was elevated [782 kU/L (0-10.9 kU/L)]. Full blood count revealed eosinophilia [2.99 9 10 9 /L (0.1-1.0 9 10 9 /L)], lymphocyte counts were normal and cell subsets identified no B cells and normal T cells. Liver function tests were deranged [gammaglutamyl transferase 752 IU/L (1-55 IU/L), alkaline phosphate 429 IU/L (126-524 IU/L), aspartate aminotransferase 110 IU/L (10-77 IU/L]) with an elevated international normalized ratio of 1.83 (normal level 1). Histopathological findingsA 3-mm punch biopsy was taken from the abdomen and at low power (Fig. 3a), compact hyperkeratosis with minimal parakeratosis was seen. At high power (Fig. 3b), a florid interface dermatitis, comprising lymphocytes, satellite cell necrosis and apoptotic keratinocytes, was identified, resembling a graft-versushost disease (GVHD) picture. Few eosinophils were seen. There were no features of ichthyosis or eczema.

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Section: Discussionmentioning

confidence: 99%

Neonatal erythroderma

et al. 2020

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“…The pathogenesis of LD remains unclear, but it has been attributed to several etiologies (Table 1). LD has been correlated with a malfunction in opsonization of yeast and S. aureus and is possibly associated with Omenn syndrome, other forms of SCID, complement C3 and C5 deficiency, hypogammaglobulinemia and hypergammaglobulinemia E. 3,8,12,14,18,26,27 Other studies have also suggested an association with familial atopy, 14,18 celiac disease, 28 Langerhans cell histiocytosis, 29 metabolic acidosis, 11 and several nutritional factors such as biotin deficiency. 2,4,6,23 LD may arise from a deficiency in opsonization of yeast and S. aureus through a deficit of complement factors.…”

Section: Pathogenesismentioning

confidence: 99%

“…It is a syndrome characterized by a clinical tetrad of severe, widespread, seborrheic‐like erythroderma beginning on the scalp or diaper area, followed by a persistent gastrointestinal disturbance, marked wasting and weight loss, and recurrent staphylococcal and candidal infections 6‐13 . Although originally reported in 1908 as a rare, autosomal dominant (AD) trait associated with a defect in yeast opsonization from dysfunctional complement 5 (C5), the “LD” phenotype may occur in infants with a variety of immunodeficiency disorders, such as complement 3 (C3) or complement 4 (C4) dysfunction, severe combined immunodeficiency (SCID), hypogammaglobulinemia, and hyperimmunoglobulinemia E 2,3,6,14,15 …”

Section: Introductionmentioning

confidence: 99%

“…Leiner's disease (LD), also known as erythroderma desquamativum (ED), is a life-threatening condition that arises within the first few months of life and lasts several weeks to months. [1][2][3][4][5][6][7][8] It is a syndrome characterized by a clinical tetrad of severe, widespread, seborrheic-like erythroderma beginning on the scalp or diaper area, followed by a persistent gastrointestinal disturbance, marked wasting and weight loss, and recurrent staphylococcal and candidal infections. [6][7][8][9][10][11][12][13] Although originally reported in 1908 as a rare, autosomal dominant (AD) trait associated with a defect in yeast opsonization from dysfunctional complement 5 (C5), the "LD" phenotype may occur in infants with a variety of immunodeficiency disorders, such as complement 3 (C3) or complement 4 (C4) dysfunction, severe combined immunodeficiency (SCID), hypogammaglobulinemia, and hyperimmunoglobulinemia E. 2,3,6,14,15 Austrian pediatrician Carl Leiner 7 in his original series during 1908 concluded that LD is a potentially fatal systemic condition that has a genetic link, a correlation with breastfed infants, and characteristic severe gastrointestinal disturbances.…”

Section: Introductionmentioning

confidence: 99%

“…Leiner's disease (LD), also known as erythroderma desquamativum (ED), is a life‐threatening condition that arises within the first few months of life and lasts several weeks to months 1‐8 . It is a syndrome characterized by a clinical tetrad of severe, widespread, seborrheic‐like erythroderma beginning on the scalp or diaper area, followed by a persistent gastrointestinal disturbance, marked wasting and weight loss, and recurrent staphylococcal and candidal infections 6‐13 .…”

Section: Introductionmentioning

confidence: 99%

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Leiner's disease (erythroderma desquamativum): A review and approach to therapy

Sanghvi

Schwartz

2020

Dermatologic Therapy

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Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic‐like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer‐reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh‐frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life‐threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.

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Proposal for a 6‐step approach for differential diagnosis of neonatal erythroderma

Cuperus

Bygum

Boeckmann

et al. 2022

Acad Dermatol Venereol

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The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the occasionally severe and life-threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case-by-case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 -May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non-congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft-versus-host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential † Member of the ERN-SKIN-subthematic group Ichthyosis

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Differential Diagnosis of Neonatal Erythroderma

Cited by 3 publications

References 66 publications

Neonatal erythroderma

Neonatal erythroderma

Leiner's disease (erythroderma desquamativum): A review and approach to therapy

Proposal for a 6‐step approach for differential diagnosis of neonatal erythroderma

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