Guidance for assessment of erythroderma in neonates and infants for the pediatric immunologist

Abstract

Neonatal and infantile erythroderma (NIE) represents the common clinical phenotype of heterogeneous diseases ranging from benign and transient skin conditions to fatal multiorgan disorders. NIE regularly demands a comprehensive diagnostic workup in a multiprofessional setting, especially if newborns and young infants with the disease develop a failure to thrive and concomitant infectious, neurologic, or metabolic complications. By obtaining a detailed medical history and performing a thorough clinical examination, targeted diagnostic steps can be scheduled for most affected children. If NIE occurs in the early neonatal period, lesional skin biopsy and histology are often indicated. Likewise, if monogenic skin or immunologic diseases are suspected, genetic testing with customized panels of potentially underlying genes is mandatory. Of note, if acute symptoms such as severe infections, metabolic acidosis, or seizures occur, rapid microbiologic and metabolic investigations are warranted to rule out immunodeficiency and inborn errors of metabolism.

Local rhinitis needs allergen‐challenges for diagnosis, late infancy supplementation of probiotics prevents eczema, and milk oral immunotherapy is effective in the long term

“…allergists are used to linking this clinical sign to atopic dermatitis, but nevertheless should be well aware of other, less frequent diagnosis. 1 Also often presenting with a skin rash, drug allergy is the theme of the second review: Marina Atanaskovic-Markovic et al have in the frame of an EAACI task force reviewed the diagnosis and management of drug-induced anaphylaxis. 2 The topic of the third review relates to another major focus of the journal, primary immunodeficiencies, with Parisa Amirifar et al 3 reviewing ataxia-telangiectasia.…”

“…The editorial team of Pediatric Allergy and Immunology and myself welcome you to this new edition of the journal. Educational publications open the issue: In the first, Hagen Ott reviews the various clinical conditions related to erythroderma in neonates and infants; allergists are used to linking this clinical sign to atopic dermatitis, but nevertheless should be well aware of other, less frequent diagnosis . Also often presenting with a skin rash, drug allergy is the theme of the second review: Marina Atanaskovic‐Markovic et al have in the frame of an EAACI task force reviewed the diagnosis and management of drug‐induced anaphylaxis .…”

Neonatal Erythroderma as an Early Sign of Primary Immunodeficiency

“…These neonates need a warm and humid environment and require an adequate liquid intake and topical therapy with emollients to guarantee proper skin hydration. 3 As reported, [4][5][6] differential diagnoses of neonatal erythroderma include monogenic skin diseases (eg, epidermolytic ichthyosis, Netherton syndrome), infections, benign skin diseases (ie, infantile psoriasis, atopic dermatitis, or seborrheic dermatitis), immune disorders, metabolic diseases, and drug-related erythroderma. Failure to thrive, severe infections, neurologic symptoms, or signs of metabolic imbalance are red flags for urgent diagnostic and therapeutic interventions.…”

“…Failure to thrive, severe infections, neurologic symptoms, or signs of metabolic imbalance are red flags for urgent diagnostic and therapeutic interventions. 4 Erythroderma with alopecia, failure to thrive, diarrhea, hepatosplenomegaly, and lymphadenopathy are suggestive of PID, a complex of signs and symptoms called Omenn syndrome. In this case, the severe and extensive skin phenotype was due to autoreactive T cells responsible for a graft-versushost disease-like phenotype.…”

“…Because benign erythematous disorders are rare in neonates, further analyses, such as complete blood count, serum electrolytes, blood gas analyses, and serum IgE levels, are often necessary. 4 Immunologic phenotype and genetic tests are crucial in case a PID is suspected. In desquamative erythroderma, a skin biopsy is also important.…”