Chapter 57 - Intercellular Immunoglobulin (Ig) A Dermatosis (IgA Pemphigus)

Question 1

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

Subcorneal pustular dermatosis (SPD)-type intercellular IgA dermatosis (IAD)

Answer 1

Desmocollin 1 (Dsc1)

Question 2

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

Intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD

Answer 2

Unknown

Question 3

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

IgA pemphigus vegetans (PVeg)

Answer 3

Desmogleins or desmocollins

Question 4

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

IgA pemphigus foliaceus (PF)

Answer 4

Desmoglein 1 (Dsg1)

Question 5

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

IgA pemphigus vulgaris (PV)

Answer 5

Desmoglein 3 (Dsg3)

Question 6

TABLE 57-1
Classification of Subtypes of Intercellular IgA Dermatosis and Their Autoantigens

AUTOANTIGENS

Undetermined-type IAD

Answer 6

Undetermined, desmogleins or desmocollins

Question 7

TABLE 57-2
Summary of Intercellular IgA Dermatosis Subtypes

DIAGNOSIS

Superficial flaccid pustules in the periphery of annular of herpetiform erythemas, most prominent in axillae and groin; (−) Nikolsky; no mucosal involvement

Answer 7

SPD-type IAD

Question 8

TABLE 57-2
Summary of Intercellular IgA Dermatosis Subtypes

DIAGNOSIS

Demarcated atypical pustular lesions scattered on entire body; “sunflower like” configuration; may have mucosal involvement

Answer 8

IEN-type IAD

Question 9

TABLE 57-2
Summary of Intercellular IgA Dermatosis Subtypes

DIAGNOSIS

Vegetating skin lesions with erosions; scalp and intertriginous areas most affected

Answer 9

PVeg-type IAD

Question 10

TABLE 57-2
Summary of Intercellular IgA Dermatosis Subtypes

DIAGNOSIS

Clinical and histopathologic similarity to PF and PV, but with IgA reactivity mainly with Dsg1 and Dsg3

Answer 10

PF and PV-type IAD

Question 11

TABLE 57-2
Summary of Intercellular IgA Dermatosis Subtypes

DIAGNOSIS

IAD patients that do not meet any of the other subtypes.

Answer 11

Undetermined-type IAD