Photo Quiz

Tense Bullae and Pruritus

 

Am Fam Physician. 2020 Mar 1;101(5):305-306.

A 72-year-old man presented with a worsening pruritic rash and painful blisters on his trunk, bilateral upper and lower extremities, face, and groin. The rash appeared two months prior. He had a history of hypertension and diabetes mellitus.

Physical examination revealed extensive, nearly confluent, pink-red plaques associated with tense and flaccid bullae, as well as erosions resulting from ruptured bullae (Figure 1). He had larger hyperpigmented patches with erosions, ulcerations, and inflammatory, red borders on his trunk. Tense bullae were largely localized to the inflammatory borders. There were no mucosal lesions.

The patient's complete blood count results were remarkable for an eosinophil count of 1,800 per uL (1.80 × 109 per L; 14%; twice the upper limit of normal). Serum immunoglobulin E (IgE) levels were elevated at 700 kU per L (reference range: 0 to 115 kU per L). Histologic examination of a punch biopsy from the edge of a tense bulla revealed a subepidermal blister with a mixed inflammatory infiltrate including eosinophils. Direct immunofluorescence of a punch biopsy from adjacent skin showed linear IgG deposition at the dermal-epidermal junction.

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FIGURE 1


FIGURE 1

Question

Based on the patient's history, physical examination, and laboratory findings, which one of the following is the most likely diagnosis?

A. Bullosis diabeticorum.

B. Bullous pemphigoid.

C. Pemphigus vulgaris.

D. Stevens-Johnson syndrome.

Discussion

The answer is B: bullous pemphigoid. Bullous pemphigoid is an autoimmune disorder characterized by autoantibodies targeting hemidesmosomal proteins of the skin and potentially mucous membranes. The primary autoantigens implicated are collagen XVII and dystonin-e. Classic bullous pemphigoid presents with tense, serous, or hemorrhagic bullae of 1 to 3 cm in diameter on the trunk and extremities that are often associated with urticarial plaques.1 In patients of color, these urticarial plaques may become hyperpigmented over time as in this patient. Typically, the skin shows a mixture of tense bullae, as well as erosions and ulcerations from ruptured bullae. Patients describe intense pruritus and often have eosinophilia.

Bullous pemphigoid is most common in women and older adults. The typical age of onset is 60 to 80 years. The Asboe-Hansen sign, also known as the indirect Nikolsky sign, is a common finding in patients with bullous pemphigoid. This sign is the

Author disclosure: No relevant financial affiliations.

Address correspondence to Matthew D. Vesely, MD, PhD, at matthew.vesely@yale.edu. Reprints are not available from the authors.

References

1. Bağci IS, Horváth ON, Ruzicka T, et al. Bullous pemphigoid. Autoimmun Rev. 2017;16(5):445–455.

2. Sárdy M, Kostaki D, Varga R, et al. Comparative study of direct and indirect immunofluorescence and of bullous pemphigoid 180 and 230 enzyme-linked immunosorbent assays for diagnosis of bullous pemphigoid. J Am Acad Dermatol. 2013;69(5):748–753.

3. Bolognia JL, Cerroni L, Schaffer JV, eds. Dermatology. 4th ed. Elsevier; 2018.

This series is coordinated by John E. Delzell Jr., MD, MSPH, associate medical editor.

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