JAAD Case Reports · 2018 · 10 citations · 6 references
MedicineDermatologyPathologyAutoimmunityImmunopathologyInflammationAutoimmune DiseasesAutoimmune Skin DiseasesImmunodeficiency DisordersInflammatory Skin DiseasesInflammatory Skin DisordersSkin InfectionsAntibody Deficiency SyndromesAutoimmune Skin ManifestationsAutoimmune Skin PathologyEosinophilic DermatosesEosinophilic Skin DisordersInflammatory Bowel Disease ImmunopathogenesisOpportunistic Skin InfectionsX-linked Agammaglobulinemia
Bruton agammaglobulinemia (XLA) is an X-linked genetic disorder characterized by severe antibody deficiency. The cornerstone of treatment is immunoglobulin replacement therapy, but patients remain at risk for recurrent sinopulmonary, gastrointestinal, and skin infections. XLA has also been associated several inflammatory and autoimmune diseases1 such as arthritis, inflammatory bowel disease, and, more recently, eosinophilic fasciitis.2 Helicobacter cinaedi is reportedly involved in several cutaneous manifestations ranging from cellulitis3,4 to superficial ulcers on erythematous, eroded plaques.
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