Baylor University Medical Center Proceedings July 2017 - 360

Linear IgA bullous dermatosis mimicking oral lichen planus
Sheevam Shah, MD, Brooke Mohr, MD, and Palak Parekh, MD

Linear IgA bullous dermatosis (LABD) is an autoimmune disease characterized by blisters on the skin and mucous membranes. Polycyclic,
grouped bullae on cutaneous skin are the most characteristic clinical
feature of LABD. Lesions are often indistinguishable from bullous pemphigoid or may resemble dermatitis herpetiformis. Oral lesions may be
seen in 5% to 70% of patients with LABD, and in some cases, the oral
mucosa may be the only area of involvement. Herein, we report a patient
with LABD involving only the oral mucosa clinically. The entity histologically mimicked oral lichen planus, which challenges accurate diagnosis
and emphasizes the importance of direct immunofluorescence.

L

inear IgA bullous dermatosis (LABD) is an autoimmune
disease that presents with bullae and blisters on the skin
and is characterized on direct immunofluorescence by
linear deposition of IgA along the basement membrane
zone. Involvement of the oral, nasal, pharyngeal, or esophageal
mucosa may also be present. Rarely, these mucosal lesions may
be the only clinical manifestation.

Figure 1. Eroded area on buccal mucosa with overlying whitish change.

CASE STUDY
A woman in her late 50s presented to the dermatology
clinic after being referred by her dentist for evaluation of buccal mucosal changes of her right posterior mouth. The patient
reported mild pain in the area over the previous 2 weeks. She
denied any previous treatments for the oral changes. She also
denied recent initiation of any new medications or recent invasive dental work. Her medical history was significant only
for a 6 pack-year history of smoking. Physical exam revealed
a white, reticular plaque on the right buccal mucosa with erosions (Figure 1). Incisional biopsy demonstrated a lichenoid
inflammatory infiltrate (Figure 2). Direct immunofluorescence
showed linear deposition of IgA (Figure 3).
Figure 2. Band-like lichenoid inflammatory infiltrate.

DISCUSSION
Polycyclic, grouped bullae on cutaneous skin-which have
been described as a "cluster of jewels" or "string of pearls"-are the
most characteristic clinical feature of LABD. Clinical diagnosis
of LABD is difficult due to its similarity to bullous pemphigoid
and dermatitis herpetiformis. Characteristic histological features
include a subepidermal blister with a prominent neutrophilic
360

From Texas A&M Health Science Center College of Medicine (Shah, Parekh)
and Department of Dermatology, Scott & White Memorial Hospital (Shah, Mohr,
Parekh), Temple, Texas.
Corresponding author: Sheevam Shah, MD, Scott & White Dermatology Clinic -
Temple Northside, 409 West Adams Avenue, Temple, TX 76501 (e-mail: sheevam.
shah@bswhealth.org).
Proc (Bayl Univ Med Cent) 2017;30(3):360-361



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