Case

Age

(y)

Sex

Age at onset (y)

Pruritus

Mucous membrane involvement

Localization

Aspects of lesions

Histology

DIF

IIF

1

6

M

5.5

-

conjonctiva

genitalia

Face, forearms, knee

Bullae and crusted erosions

Subepidermal bullae (SEB), dermal inflammation with eosinophils

Predominant linear IgA deposits with IgG on DEJ

-

2

4

F

NP

+

-

Trunk, limbs

Bullea , infected erosions

SEB, eosinophils

Predominant linear IgA deposits with C3 on DEJ

NPF

3

11

F

11

+

-

Face, limbs

Vesiculo-bullaes, erosions, erythema

SEB, eosinophils

Linear IgA deposits on DEJ

NPF

4

3

F

3

+

-

Trunk, limbs

Infected bullea, erythema

SEB, eosinophils

Predominant linear IgA deposits with C3 on DEJ

NPF

5

11

F

NP

NP

NP

NP

NP

SEB, eosinophils in the papilla

Predominant linear IgA deposits with IgG on DEJ

NP

6

1.8

F

NP

+

-

Scalp, face, buttocks, limbs

Pseudo-urticarial erythema, bullea, erosions

SEB, eosinophils, neutrophils , dermal inflammation

Linear IgA deposits

on DEJ

NPF

7

6

M

6

+

-

Face, neck

Bullea, erosions, erythema

SEB, eosinophils, dermal inflammation

Predominant linear IgA deposits with IgG, C3

Circulating IgG

8

1.5

M

1

+

-

Face, trunk, limbs

Erythema, Bullae, erosions

SEB, eosinophils, dermal inflammation

Predominant linear IgA deposits with IgM

NPF

9

2

M

2

+

-

Leg, arms, forearms

Vesiculo-bullae, crusted erosions,

SEB, eosinophils, dermal inflammation

Linear IgA deposits

NPF

10

0.6

M

0.8

+

-

Face, neck, hands, thighs

Bullae

SEB, eosinophils in pthe papilla, dermal inflammation

Linear IgA deposits

NPF

11

4.5

M

4.5

+

Cheilitis, conjunctivitis

Scalp, neck, back, limbs

Infected bullae

SEB, eosinophils and neutrophils in inflammatory dermis

Predominant linear IgA deposits with C3

NPF

12

3

M

3

+

-

Genitalia, lower limbs

Infected bullae

SEB, eosinophils, dermal inflammation

Linear IgA deposits

NPF

13

4

M

3

+

-

Face, trunk, hands, lower legs

Veciculo-bullae, erosions

NP

Predominant linear IgA deposits with IgM

NPF

14

15

M

14

+

-

Scalp, face, trunk, limbs

Erythema, vesiculo-bullae

SEB, eosinophils, dermal inflammation

Linear IgA deposit

-

15

5

M

5

-

Genitalia

Face, buttocks, limbs

Erythema, vesiculo-bullae, erosions

SEB, eosinophils, dermal inflammation

Predominant linear IgA deposits with C1q

NPF

16

8

M

8

+

-

Limbs

Erythema, vesiculo-bullae, pustules, impetigo like lesions

SEB, eosinophils and neutrophils in the dermis

Predominant linear IgA deposits with IgM and C1q on DEJ

NPF

17

3

M

2.5

+

-

Chest, perineum, lower legs

Annular bullae with impetigo like lesions

NP

Predominant linear IgA deposits on DEJ

NPF

18

2.5

M

1.5

+

-

Face, trunk, genitalia, limbs

Erythema, vesiculo-bullae

SEB, eosinophils and neutrophils in the dermis

Predominant linear IgA deposits with C3 on DEJ

Circulating IgG anti BMZ

19

1

M

1

+

-

Scalp, face, genitalia, limbs

Erythema, bullies, erosions

NP

Predominant linear IgA deposits with C3 and C1q on DEJ

negative

20

2

M

2

+

Genital erosions

Scalp, trunk, limbs

Erythema, bullae,

NP

Predominant linear IgA deposits with C3 and C1q

NPF

21

3

F

3

+

-

Face, limbs

Erythema, Vesiculo-bullae, erosions

NP

Predominant linear IgA deposits with IgG and C3 on DEJ

NPF

22

12

F

10

+

-

Trunk, buttocks, lower limbs

Vesiculo-bullae, erosions, impetigo-like lesions

SEB, dermal inflammation

Predominant linear IgA deposits with IgG on DEJ

NPF

23

7

F

7

+

-

Face, neck, buttocks, peri-anal region, limbs

Bullae and pustules

SEB, dermal inflammation

Linear IgA deposits on DEJ

NPF

24

2

M

1

-

-

Lower legs

Vesiculo-bullae, erosions

NP

Predominant linear IgA deposits with C1q on DEJ

NPF

25

8

F

5

-

-

Lower legs

Vesiculo-bullae, erosions, impetigo-like lesions

NP

Linear IgA deposits on DEJ

NPF

26

4

M

4

+

-

Face, buttocks, genitalia, limbs

Erythema, infected vesiculo-bullae, erosions

SEB, dermal inflammation

Linear IgA deposits on DEJ

NPF

27

16

M

14

NP

-

Upper and lower limbs

Bullae

NP

Linear IgA deposits on DEJ

NPF

28

12

M

11

+

-

Genitalia, lower limbs

Vesiculo-bullae

SEB, dermal inflammation

Predominant linear IgA deposits with IgM on DEJ

NPF

29

1.5

M

1

+

-

Scalp, face, trunk, limbs

Vesiculo-bullae, erosions

SEB, dermal inflammation

Predominant linear IgA deposits with C3 on DEJ

NPF

30

11

M

9.5

+

-

Face, perineum, limbs

Erythema, Vesiculo-bullae

SEB, dermal inflammation with neutrophils

Predominant linear IgA deposits with IgM on DEJ

Negative

31

7

M

7

-

-

Trunks, limbs

Erythema, Vesiculo-bullae

SEB, dermal inflammation

Predominant linear IgA deposits on DEJ

NPF