CASE REPORT
MATTIA,
A CASE OF
“AUTOIMMUNE KALEIDOSCOPE"
Teresa Giani MD
University of
Florence
Ann Meyer Children’s Hospital
Florence
Italy
MEDICAL HISTORY
√ Family history: fatherÆ urticaria
√ Past history: uninformative
• 4 months of recurrent episodes of diffuse, migratory,
pruriginous maculopapular eruption at trunk and proximal
extremities
+
• abdominal pain
√ Recent history (12-year-old)
• History: uninformative
• PRICK Skin test: negative
• RAST test: negative
• PRIST: IgE 1070 U/ml (n.v. <100)
• Routinary blood test: eosinophils 14.7%
CHRONIC
IDIOPATHIC
URTICARIA
(autoimmune?)
RECENT MEDICAL HISTORY (One month later)
subtotal villous atrophy, deep crypts with a dense
plasma cell infiltrate in the lamina propria
ENDOSCOPIC INTESTINAL BIOPSY:
SEROLOGICAL TESTS
•anti-gliadin,AGA, IgA 32 (vn<7); IgG 20 (vn<15)
•anti endomisium, EmA, IgA positive
•Antitransglutaminase, tTG, IgA 20 (vn<7)
COELIAC
DISEASE
Abdomen: soft, painful, distended and tympanic
A gastroenterology opinion was requested
• abdominal symptoms resolved
• autoantibodies normalized
• urticaria lesions occasionally still
recurred with a milder expression
Gluten-free diet (optimal compliance)
3 months
One year later (Mattia is 13-year-old)
COMPLAINING OF
• Pain and swelling: wrist, metacarpophalangeal, interphalangealand ankle joints
• Morning stiffness
PHYSICAL EXAM
• Diffuse limited range of motion (ankles, wrists and fingers); no swelling
• Urticaria lesions (trunk)
RHEUMATOLOGY
UNIT
RHEUMATOLOGY UNIT
LAB STUDIES:
Serum angiotensinconverting
Routinary tests
Thyroid function tests + auto-antibodies
Circulating immune complexes
anti-Borrelia burgdorferi antibodies
C3 and C4
•ANA
•anti-double-stranded DNA
•anti SS-A, Anti SS-B
•anti-Sc 70l
•anti RNA polymerases
•Reumatoid factor
•anti RNP
•anti-centromere
•anti PM-Scl
•anti Sm
enzyme
RESULTED OVER AND
OVER NEGATIVE
I
HLA: class I A 2-26 (10), B 13-18, C 6-7, Bw 4,6; class II DQ 2-3, DR 7-11
Raynaud’s
phenomenon
became evident at fingers and
toes after expositio