Erythroderma

 

Omenn Syndrome

 

Omenn Syndrome - Patients develop generalized erythroderma or an eczematous rash with scaling. This rash may result in alopecia or loss of eyebrows/lashes. Oligoclonally expanded T cells infiltrate the skin (biopsies may reveal a lymphocytic infiltrate of CD3+CD4+ T cells).

 

Netherton Syndrome

 

Netherton Syndrome - This disease is caused by mutations in SPINK5 and is characterized by severe dermatitis, erythroderma, bamboo hair, and immunodeficiency (hypogammaglobulinemia, specific antibody deficiency, low switched memory B cells, and decreased NK cell function). Staphylococcal skin infections and upper respiratory infections are common.

 

IPEX Syndrome

 

IPEX Syndrome - The majority of patients have skin disease which manifests as severe atopic dermatitis. However, erythroderma, exfoliative dermatitis, psoriasis-like lesions, and pemphigous nodularis have also been observed. Patients also have infant-onset severe enteropathy with villous atrophy and autoimmune disease such as Type I diabetes, thyroid disease, or autoimmune cytopenias.