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.
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. [email protected]
). Proc (Bayl Univ Med Cent) 2017;30(3):360–361
Figure 3. Linear IgA deposition along dermal-epidermal junction.
inﬁltrate. A few prior cases of oral LABD with a lichenoid histology mimicking lichen planus have been reported (1). Two types of LABD have been identiﬁed, drug-induced and spontaneous (2). In drug-induced LABD, lesions tend to be more severe than in the spontaneous form. Various case reports have described drug-induced LABD presenting as toxic epidermal necrolysis (3). Commonly implicated drugs include vancomycin (4), beta-lactam antibiotics, captopril, and nonsteroidal antiinﬂammatory drugs. The diﬀerential diagnosis for reticular changes of the buccal mucosa includes primarily lichen planus, lichenoid drug reaction, and contact allergy. Biopsy in our patient revealed a lichenoid inﬂammatory inﬁltrate, compatible with lichen planus. She was not taking any medications. Furthermore, she denied any recent dental work, minimizing the possibility of a contact allergy to components of her ﬁllings in nearby teeth.
Establishing the correct diagnosis can guide appropriate treatment. In recent years, indirect and direct immunoﬂuorescence have emerged as tools that can be used in conjunction with histopathology in making these diﬃcult diagnoses (5). It is important to consider oral LABD as a diﬀerential in cases of clinically suspected oral lichen planus, especially those that fail to respond to initial therapies. Thus, direct immunoﬂuorescence studies should be pursued to distinguish between the two entities and guide appropriate treatment. Dapsone has been reported as a successful treatment option for management of oral LABD in cases refractory to topical and systemic steroids. Early diagnosis and management of LABD can induce rapid improvement and may decrease the incidence of relapse after treatment withdrawal (6). 1. 2. 3.
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Linear IgA bullous dermatosis mimicking oral lichen planus