The 2.5 year old boy presented with an itchy skin rash above the elbows, knees and buttocks. He had been treated for atopic dermatitis, but did not improve. Since the distribution of the symptoms suggested dermatitis herpetiformis (DH), we performed immunofluorescence from the perilesional skin and could confirm the diagnosis by the characteristic IgA staining in the dermal papilla (Fig. 15.1) and by the endomysium antibody (EMA) positivity, diagnostic for celiac disease (CD) (that time no transglutaminase (TG) 2 or 3 ELISAs were available) . He also underwent a small bowel biopsy by Crosby capsule and showed mild partial villous atrophy and a pathologic IgA staining in the jejunum - today recognized as TG2 bound IgA staining- and was treated by gluten free diet (GFD) and by low dose dapsone. While there were no laboratory side effects from the drug detected, his steps became uncertain, and could not run as before. When we stopped the medication his movement recovered. He went on solely with the GFD and his skin became symptom-free. Several years later the patientâ€™s father developed severe weight loss and was treated for anorexia nervosa. He asked us whether we could check him for CD, since he did not feel the psychological urge to hunger, but suffered from recurrent diarrhea. He was diagnosed with an acute CD. That time, his other son was also investigated for gluten sensitivity and he was also EMA positive, and had mild partial villous atrophy on jejunal biopsy. The mother was not affected by the disease, and none of the family members had associated diseases at that time.
ASJC Scopus subject areas
- Immunology and Microbiology(all)