Distinct Characteristics in Japanese Dermatitis Herpetiformis: A Review of All 91 Japanese Patients over the Last 35 Years
Table 1
Clinical characteristics of 91 patients.
or age/ of data available
Gender
Male
61/91
Female
30/91
Age at the initial visit, mean ± SD (range), years
(1–87)
Male
(1–87)
Female
3
(18–72)
Age at onset, mean ± SD (range), years
(1–87)
Male
(1–87)
Female
(14–72)
Site of lesion
Elbow
(39.3%)
Knee
(34.5%)
Buttock
(35.7%)
Elbow and/or knee and/or buttock
(44.0%)
Face
(13.1%)
Ear
(10.7%)
Neck
(9.5%)
Scalp
(7.1%)
Groin
(4.8%)
At least one predilection site
(58.3%)
Extremities*
(48.8%)
Trunk**
(65.5%)
Whole body***
(7.1%)
IgA deposition in the papillary dermis
Granular
(54.9%)
Fibrillar
(36.3%)
Granular and fibrillar
(7.7%)
Cluster
(1.1%)
Other deposition in the papillary dermis
C3
(22.0%)
IgG
(9.9%)
IgM
(4.4%)
Fibrinogen
(2.2%)
GSE
(3.3%)
Jejunum mucosa biopsy
Villous atrophy****
(50.0%)
No change*****
(50.0%)
Associated diseases
Diabetes mellitus
(8.8%)
Lymphoma
(3.3%)
Thyroid disease
(1.1%)
Sjögren syndrome
(1.1%)
HLA antigen§
DR4
(41.9%)#
DR9
(48.4%)##
Other antibodies
Antireticulin antibodies
(0.0%)
Antigliadin antibodies
(0.0%)
Antiendomysial antibodies
(0.0%)
Anti-tTG IgA antibodies
(0.0%)
Anti-eTG IgA antibodies
(100.0%)
Therapy
Dapsone
(75.6%)
Dapsone + gluten-free diet
(8.5%)
Gluten-free diet + topical steroid
(1.2%)
Topical steroid
(11.0%)
Others†
(3.7%)
*Not including cases limited only to elbow or knee; **not including cases limited only to buttock, neck, or groin; ***not including cases limited to combination of predilection sites; ****including 1 patient with GSE; *****no patients had GSE; GSE: gluten-sensitive enteropathy; tTG: tissue transglutaminase, eTG: epidermal transglutaminase; †including minocycline and topical steroid, salazosulfapyridine, and zinc oxide ointment; §frequency in HLA antigens of control was depicted from [90]. #, ##, corrected .
