The correct answer is B. Omenn syndrome; flow cytometry and lymphocyte subset analysis.

Key features of Omenn syndrome include neonatal onset erythroderma with generalized scaling and sparse hair (including eyebrows). Patients have elevated IgE and eosinophilia with failure to thrive and recurrent bacterial infections. Mutations in either RAG1/RAG2 will result in defective T-cell production.

Answer A) Netherton syndrome has hair shaft abnormalities and normal B and T-cell function

Answer C) Although there is a collodion membrane and plate-like scaling, there is no immune dysfunction in this condition

Answer D) This question stem describes a more chronic course and staphylococcal scalded skin syndrome has an acute onset, positive Nikolsky sign and lacks eosinophilia and elevated IgE.

Answer E) Hyper IgE is associated with multiple dysmorphic features including coarse facies, retained primary teeth and has much milder skin involvement

References:

Alikhan, A., & Hocker, T. L. (2023). Review of Dermatology E-Book. Elsevier Health Sciences; Paller, A. S., & Mancini, A. J. (2020). Paller and Mancini-Hurwitz Clinical Pediatric Dermatology E-Book: A textbook of skin disorders of childhood and adolescence. Elsevier Health Sciences.

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