Chapter 58 - Linear Immunoglobulin A Dermatosis and Chronic Bullous Disease of Childhood Flashcards
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
40% have C3
24% have IgG
Almost all IgA1
Linear IgA
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
None reported with C3
Almost all have IgA1
Drug-induced IgA
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
COMPOSITION OF CUTANEOUS IMMUNOREACTANTS
9% have IgG
May have C3
Almost all have IgA1
CBDC
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
Linear IgA
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
LOCATION OF IMMUNOREACTANTS
Lamina lucida or sublamina densa
CBDC
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
CIRCULATING IgA AUTOANTIBODIES
May have low titer against epidermal basement membrane antigens
Linear IgA
TABLE 58-1
Immunoreactants and Circulating Antibodies
TYPE
CIRCULATING IgA AUTOANTIBODIES
Most have low titer against epidermal side of sodium split-skin
CBDC
TABLE 58-1
Immunoreactants and Circulating Antibodies
LOCATION OF IMMUNOREACTANTS
BP-like Linear IgA
Lamina lucida
TABLE 58-1
Immunoreactants and Circulating Antibodies
LOCATION OF IMMUNOREACTANTS
EBA-like Linear IgA
At and below lamina lucida
TABLE 58-1
Immunoreactants and Circulating Antibodies
LOCATION OF IMMUNOREACTANTS
CBDC
Lamina lucida or sublamina densa
TABLE 58-2
Pharmacological Treatments
First-line
(2)
Dapsone
Sulfapyridine
TABLE 58-2
Pharmacological Treatments
First-line adjuvant
(1)
Low-dose prednisone
TABLE 58-2
Pharmacological Treatments
Second-line
(2)
Mycophenolate mofetil
Intravenous immunoglobulin
TABLE 58-2
Pharmacological Treatments
Second-line adjuvant
(2)
Antibiotics (including trimethoprim-sulfamethoxazole, dicloxacillin, erythromycin, flucloxacillin)
Topical tacrolimus
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
Linear IgA dermatosis
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
DIAGNOSIS
EPIDEMIOLOGY
Adults
Drug-induced linear IgA
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
Chronic bullous disease of childhood
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
Linear IgA dermatosis
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
Drug-induced linear IgA
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
Chronic bullous disease of childhood
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
Linear IgA dermatosis
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
DIAGNOSIS
MUCOSAL INVOLVEMENT
Mucosal involvement less prominent
Drug-induced linear IgA
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
Chronic bullous disease of childhood
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
Linear IgA dermatosis
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
Drug-induced linear IgA
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
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
Ulcerative colitis
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
Ultraviolet light
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
Vancomycin
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
Infectious mononucleosis and Paecilomyces lung infection in the setting of chronic granulomatous disease
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
Linear IgA dermatosis
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
DIAGNOSIS
COURSE
May not result in recurrence
Drug-induced linear IgA
Rechallenge of drug may not result in recurrence
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
Chronic bullous disease of childhood
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
TREATMENT
Linear IgA dermatosis
Dapsone, sulfapyridine; response within 24-48 h; low-dose prednisone may suppress blister formation
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
TREATMENT
Drug-induced linear IgA
Discontinuation of causative drug; sometimes initiation of dapsone is helpful
TABLE 58-4
Clinical Comparison between Linear IgA, Drug-Induced IgA, and Chronic Bullous Disease of Childhood
TREATMENT
Chronic bullous disease of childhood
Dapsone, sulfapyridine; small doses of prednisone may be of use; mycophenolate mofetil as a steroid-sparing agent; topical tacrolimus
