Chapter 3: Vesiculobullous Diseases

QUESTIONS

QUESTION 3-1. Many dermatologic conditions have more than one name. Please match the following diseases with their alternative designation.

• 1) Pemphigus foliaceus

• 2) Transient acantholytic dermatosis

• 3) Benign familial pemphigus

• 4) Keratosis follicularis

A) Darier disease

B) Hailey-Hailey disease

C) Grover disease

D) Fogo selvagem

QUESTION 3-2. Which of the following statements regarding intraepidermal vesicular and pustular diseases is INCORRECT?

A) Acantholysis is defined as a disruption in cohesion between adjacent keratinocytes resulting in separated and rounded keratinocytes rimmed by a brightly eosinophilic band of keratin. Intercellular bridges are lost.

B) With spongiosis, intercellular bridges are more prominently seen due to the accumulation extracellular intraepidermal edema.

C) Cytolysis characteristically results in large numbers of acantholytic keratinocytes.

D) Pustules contain abundant inflammatory cells, usually neutrophils.

E) Traumatic or immune-mediated cytolysis may result in blister formation due to destruction of keratinocytes and their desmosomal adhesion molecules.

QUESTION 3-3. Which of the following statements regarding the lesion illustrated below is LEAST accurate?

A) ELISA studies with monkey esophagus conducted on the patient’s peripheral blood will be positive for antibodies directed against desmosomal cadherins. These antibody concentrations show a general correlation with disease activity.

B) Direct immunofluorescence studies will demonstrate intercellular deposition of IgG and C3d within the epidermis.

C) Oral lesions are the first manifestation in 10% to 15% of patients and almost invariably develop during the course of the disease.

D) Clinically these lesions present as persistent, small, pin-sized, skin-colored, and slightly pruritic papules arising on the upper back and chest. Mucosal involvement is absent.

E) About 30% of cases will exhibit intercellular deposition of both IgM and IgA by direct immunofluorescence studies.

QUESTION 3-4. A 50-year-old patient presents with multiple flaccid bullae of the oral mucosa and lips as well as on the back, scalp, axilla, groin, and trunk. The patient reports that these lesions developed on normal skin without any prior erythema. The blisters extend laterally with compression. Many of the bulla on the lips and mouth are ruptured revealing underlying painful erosions. Slight rubbing of the uninvolved skin results in exfoliation of the outermost layer and the formation of a blister within minutes. A biopsy of the peripheral edge ...