Clinical Challenge: Blistering Rash on Abdomen, Thighs

A punch biopsy taken from the border of a new lesion was consistent with bullous pemphigoid, an autoimmune blistering disease that typically affects the elderly. Classic bullous pemphigoid presents as multiple 1-3cm tense bullae arising from normal-appearing or inflamed skin.1,2A...

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A punch biopsy taken from the border of a new lesion was consistent with bullous pemphigoid, an autoimmune blistering disease that typically affects the elderly. Classic bullous pemphigoid presents as multiple 1-3cm tense bullae arising from normal-appearing or inflamed skin.^1,2

A preceding prodromal or preblistering phase may occur weeks to months before the onset of the bullae.^1,3,4 These patients present with either eczematous or urticarial skin lesions.⁵ Many are initially diagnosed with hives due to the moderate to severe pruritus.¹

The lesions of bullous pemphigoid commonly present on the trunk, flexor surfaces of the arms and legs, the groin, and axilla.⁵ Oral blisters are noted in a significant percentage.

Cutaneous bullae are tense in contrast to the flaccid blisters of pemphigus vulgaris. Nikolsky’s sign in bullous pemphigus is negative, meaning that applying tense pressure on the blister will not extend the lesion to the normal skin. Bullae rupture within a week, leaving erosions and slight crusting.¹

Biopsy from the periphery of a lesion reveals a subepidermal blister and a mixed inflammatory infiltrate. Confirmation of the diagnosis is made by immunofluorescent staining, which manifests deposition of immunoglobulin G (IgG) and complement in a linear band at the dermal-epidermal junction. Circulating autoantibodies are highly specific and correlate with disease activity.⁵

The goal of therapy is to reduce inflammation and squelch autoantibody formation. Oral tetracycline and corticosteroids have been used for decades to manage bullous pemphigoid but prolonged use of the latter may have untoward consequences.

Application of the superpotent topical steroid clobetasol propionate cream has been demonstrated to control disease without serious adverse events.⁶ Refractory cases may respond to the rituximab, which suppresses antibody-producing B cells.⁷

Megha D. Patel is a student at the Commonwealth Medical College in Scranton, Pennsylvania.

Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College and an adjunct assistant professor of dermatology at the University of Pennsylvania Medical College. He practices dermatology in Hazleton, PA.

References

1. Habif TP. Clinical Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2010. 
2. Di Zenzo G, Marazza G, Borradori L. Bullous pemphigoid: physiopathology, clinical features and management. Adv Dermatol. 2007;23:257-288.
3. Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges.2011;9:844-856.
4. Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid.Dermatol Clin. 2011;29:427-438.
5. Kasperkiewicz M, Zillikens D, Schmidt E. Pemphigoid diseases: pathogenesis, diagnosis, and treatment.Autoimmunity. 2012; 45:55-70.
6. Joly P, Roujeau JC, Benichou J, et al; Bullous Diseases French Study Group. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. 2002;346:321-327.
7. Dupuy A, Viguier M, Bédane C, et al. Treatment of refractory pemphigus vulgaris with rituximab (anti-CD20 monoclonal antibody). Arch Dermatol. 2004;140:91-96.