Chapter 58 - Linear Immunoglobulin A Dermatosis and Chronic Bullous Disease of Childhood

Question 1

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
40% have C3
24% have IgG
Almost all IgA1

Answer 1

Linear IgA

Question 2

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
None reported with C3
Almost all have IgA1

Answer 2

Drug-induced IgA

Question 3

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
9% have IgG
May have C3
Almost all have IgA1

Answer 3

CBDC

Question 4

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

LOCATION OF IMMUNOREACTANTS
1. Lamina lucida (BP-like)
2. At and below lamina lucida (EBA-like)
3. Above and below lamina lucida 

Answer 4

Linear IgA

Question 5

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

LOCATION OF IMMUNOREACTANTS
Lamina lucida or sublamina densa

Answer 5

CBDC

Question 6

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

CIRCULATING IgA AUTOANTIBODIES
May have low titer against epidermal basement membrane antigens

Answer 6

Linear IgA

Question 7

TABLE 58-1
Immunoreactants and Circulating Antibodies

TYPE

CIRCULATING IgA AUTOANTIBODIES
Most have low titer against epidermal side of sodium split-skin

Answer 7

CBDC

Question 8

TABLE 58-1
Immunoreactants and Circulating Antibodies

LOCATION OF IMMUNOREACTANTS

BP-like Linear IgA

Answer 8

Lamina lucida

Question 9

TABLE 58-1
Immunoreactants and Circulating Antibodies

LOCATION OF IMMUNOREACTANTS

EBA-like Linear IgA

Answer 9

At and below lamina lucida

Question 10

TABLE 58-1
Immunoreactants and Circulating Antibodies

LOCATION OF IMMUNOREACTANTS

CBDC

Answer 10

Lamina lucida or sublamina densa

Question 11

TABLE 58-2
Pharmacological Treatments

First-line
(2)

Answer 11

Dapsone
Sulfapyridine

Question 12

TABLE 58-2
Pharmacological Treatments

First-line adjuvant
(1)

Answer 12

Low-dose prednisone

Question 13

TABLE 58-2
Pharmacological Treatments

Second-line
(2)

Answer 13

Mycophenolate mofetil
Intravenous immunoglobulin

Question 14

TABLE 58-2
Pharmacological Treatments

Second-line adjuvant
(2)

Answer 14

Antibiotics (including trimethoprim-sulfamethoxazole, dicloxacillin, erythromycin, flucloxacillin)
Topical tacrolimus

Question 15

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

EPIDEMIOLOGY
Often adults at the 4th decade of life; slight female predominance

Answer 15

Linear IgA dermatosis

Question 16

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

EPIDEMIOLOGY
Adults

Answer 16

Drug-induced linear IgA

Question 17

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

EPIDEMIOLOGY
Occurs before 5 years of age; slight female predominance

Answer 17

Chronic bullous disease of childhood

Question 18

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

CLINICAL PRESENTATION
Similar to Dermatitis herpetiformis; annular or grouped papules, vesicles, and bullae on extensors, including elbows, knees, and buttocks; pruritus is less severe than in DH

Answer 18

Linear IgA dermatosis

Question 19

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

CLINICAL PRESENTATION
Vary from erythema multiforme–like, to toxic epidermal necrolysis–like with widespread bullae; Koebner phenomenon may be present

Answer 19

Drug-induced linear IgA

Question 20

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

CLINICAL PRESENTATION
Tense bullae in a “cluster-of-jewels” appearance; collarette of blisters may be present

Answer 20

Chronic bullous disease of childhood

Question 21

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

MUCOSAL INVOLVEMENT
Oral involvement in up to 70% of patients

Answer 21

Linear IgA dermatosis

Question 22

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

MUCOSAL INVOLVEMENT
Mucosal involvement less prominent

Answer 22

Drug-induced linear IgA

Question 23

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

MUCOSAL INVOLVEMENT
Mucosal involvement noted, but less common

Answer 23

Chronic bullous disease of childhood

Question 24

TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood

DIAGNOSIS

DISEASE ASSOCIATIONS AND TRIGGERS
Possible association with ulcerative colitis; ultraviolet light is the chief physical trigger

Answer 24

Linear IgA dermatosis

Question 25

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DIAGNOSIS DISEASE ASSOCIATIONS AND TRIGGERS Vancomycin most commonly implicated; interferon-a, influenza vaccine, lithium, phenytoin, sulfamethoxazole-trimethoprim, furosemide, atorvastatin, captopril, diclofenac, ketoprofen, and infliximab

Answer 25

Drug-induced linear IgA

Question 26

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DIAGNOSIS DISEASE ASSOCIATIONS AND TRIGGERS Possible association with infectious mononucleosis and Paecilomyces lung infection in the setting of chronic granulomatous disease

Question 27

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DISEASE ASSOCIATIONS AND TRIGGERS Disease associated with Linear IgA dermatosis

Answer 27

Ulcerative colitis

Question 28

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DISEASE ASSOCIATIONS AND TRIGGERS Chief physical trigger of Linear IgA dermatosis

Answer 28

Ultraviolet light

Question 29

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DISEASE ASSOCIATIONS AND TRIGGERS Most commonly implicated in Drug-induced linear IgA

Answer 29

Vancomycin

Question 30

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DISEASE ASSOCIATIONS AND TRIGGERS Disease associated with Chronic bullous disease of childhood

Answer 30

Infectious mononucleosis and Paecilomyces lung infection in the setting of chronic granulomatous disease

Question 31

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DIAGNOSIS COURSE Unpredictable course; varies from spontaneous remission to longstanding disease

Answer 31

Linear IgA dermatosis

Question 32

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DIAGNOSIS COURSE May not result in recurrence

Answer 32

Drug-induced linear IgA Rechallenge of drug may not result in recurrence

Question 33

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood DIAGNOSIS COURSE Self-limited; remission within 2 years of onset

Answer 33

Chronic bullous disease of childhood

Question 34

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood TREATMENT Linear IgA dermatosis

Answer 34

Dapsone, sulfapyridine; response within 24-48 h; low-dose prednisone may suppress blister formation

Question 35

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood TREATMENT Drug-induced linear IgA

Answer 35

Discontinuation of causative drug; sometimes initiation of dapsone is helpful

Question 36

TABLE 58-4 Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood TREATMENT Chronic bullous disease of childhood

Answer 36

Dapsone, sulfapyridine; small doses of prednisone may be of use; mycophenolate mofetil as a steroid-sparing agent; topical tacrolimus