Patient Case: Epidermolysis Bullosa Acquisita (EBA)
A 55-year-old man presents to the dermatology clinic with a six-month history of fragile skin, blisters, and slow-healing erosion on his hands, elbows, knees, and feet. He reports that the blisters often appear after minor trauma, such as rubbing his skin or wearing tight shoes. Over time, the affected areas have developed scarring and milia (small white cysts). He denies any mouth ulcers or recent illness.
His medical history is significant for long-standing inflammatory bowel disease (Crohn’s disease), for which he has been receiving immunosuppressive therapy.
On examination, the patient has tense bullae and erosions on trauma-prone areas such as the hands, elbows, and knees, with scarring and milia formation. The Nikolsky sign is negative (skin does not shear off with slight pressure).
A skin biopsy with direct immunofluorescence shows linear IgG deposits along the dermoepidermal junction, confirming a diagnosis of epidermolysis bullosa acquisita (EBA). The patient is started on systemic corticosteroids and immunosuppressive therapy to control the disease.
- What is the most likely diagnosis in this patient?
a) Pemphigus vulgaris
b) Epidermolysis bullosa acquisita (EBA)
c) Bullous pemphigoid
d) Dermatitis herpetiformis - Which clinical feature is characteristic of epidermolysis bullosa acquisita (EBA)?
a) Flaccid blisters with oral mucosal involvement
b) Tense blisters on trauma-prone areas with scarring and milia formation
c) Pruritic vesicles grouped on extensor surfaces
d) Widespread erosions with target-like lesions - Which diagnostic test is most useful in confirming EBA?
a) Tzanck smear
b) Direct immunofluorescence of skin biopsy
c) Potassium hydroxide (KOH) preparation
d) Bacterial culture of blister fluid - What is the first-line treatment for epidermolysis bullosa acquisita?
a) Topical corticosteroids only
b) Systemic corticosteroids and immunosuppressive therapy
c) Oral antibiotics and wound care
d) Antiviral therapy with acyclovir
