Chap 57 - IgA Pemphigus

Question 1

A patient presents with superficial flaccid pustules in the periphery of annular or herpetiform erythemas, most commonly on the axilla and groin; DIF showed IgA deposition to the keratinocyte cell surface in the upper epidermis.

Diagnosis?

Answer 1

SPD-type IAD
(Subcorneal Pustular Dermatosis-Type IgA Dermatosis

Question 2

A patient presents with discrete, flaccid pustules that coalesce and form annular, circinate, or serpiginous patterns, mostly on the axilla, groin, abdomen. DIF is negative.

Diagnosis?

Answer 2

Subcorneal Pustular Dermatosis

Question 3

T/F - SPD-type IAD and classical SPD without IgA antibodies can be differentiated clinically

Answer 3

False.
Because SPD-type IAD and classical SPD without
IgA antibodies show exactly the same clinical and
histopathologic features, these 2 diseases should be
differentiated by detection of IgA anti–cell-surface anti
bodies by direct immunofluorescence.

Question 4

A patient presented with well-demarcated atypical pustular lesions scattered on
entire body with a “sunflower like” configuration.

What is the diagnosis?

Answer 4

IEN-type IAD (intraepidermal neutrophilic IgA dermatosis)

Patient may show mucosal involvement

Question 5

Answer 5

Remember

Question 6

Histopath of a pustule showed neutrophilic pustule formation in the middle epidermis

Diagnosis?

Answer 6

(IEN-type IAD) Intraepidermal neutrophilic IgA dermatosis–type intercellular IgA dermatosis

Question 7

Most frequently found underlying disease in IAD is?

Answer 7

Ulcerative Colitis

May also have multiple myeloma or B-cell lymphoma

Question 8

DIF findings show IgA deposition to the cell surfaces in the entire epidermis

Answer 8

IEN-type IAD
(entire as in IEN)

Question 9

DIF shows IgA deposition to keratinocyte cell surfaces in the uppermost epidermis

Answer 9

SPD-type IAD

Question 10

DIF shows IgA deposition to the cell surfaces in the entire epidermis, being stronger in the UPPER epidermis

Answer 10

PF-type IAD

(entire, pero mas UPPER epidermis)

Question 11

DIF shows IgA deposition to the cell surfaces in the lower epidermis

Answer 11

PV-type IAD

Question 12

Complementary DNA Transfection Method will show what findings?

Answer 12

Almost all patients with SPD-type IAD show IgA reactivity with Dsc1

Question 13

This is the preferred diagnostic method for various
AIBDs and is more sensitive than either indirect immunofluorescence, immunoblotting, or cDNA transfection

Answer 13

ELISA

Question 14

This is the preferred test for detecting IgA reactivity with Dsc1, Dsc2, and Dsc3 in various types of IAD

Answer 14

IgA ELISA using mammalian recombinant proteins

Question 15

Most common therapy for IAD? Dose?

Answer 15

Oral dapsone 50 to 200 mg/day

Question 16

This is the second choice therapy for IAD

Answer 16

“relatively low doses of corticosteroids (0.2 to 0.5 mg/kg/day, equivalent to prednisolone) are given in IAD”

Question 17

To suppress activity of neutrophils, this drug is used, albeit inconsistent effectiveness

Answer 17

Colchicine

(gusto mo yun, albeit?)

Question 18

Dsc1 is the major autoantigen for what IgA Dermatosis?

Answer 18

SPD-type intercellular IgA dermatosis