36
Management
Table 10C. Management of Dermatologic Disease
Manifestation Associated IEI
Rash not responsive to therapy Various IEI
Granulomatous skin disease CID, CVID, CGD
Moderate-to-severe atopic
dermatitis
HIES, WAS
Refractory warts CID
Table 10D. Management of Rheumatologic and
Musculoskeletal Disease
Manifestation Associated IEI
Reduced bone mineral density
and minimal trauma fractures
STAT3-HIES
Retained primary teeth STAT3-HIES
SLE and SLE-like symptoms Antibody deficiencies, CGD, CGD carriers
Small-medium vessel vasculitis Complement deficiency, WAS
Inflammatory myopathy
Persistent non-infectious oligo-
or poly-articular arthritis
Antibody deficiencies, WAS