Rare skin diseases in adults

ABSTRACT: Certain papulosquamous and bullous diseases are more common in older adults than in younger persons. For example, the mean age of onset of pemphigus vulgaris is about 50 to 60 years. Because this disease can be life-threatening, prompt diagnosis is crucial. In elderly persons, dermatomyositis often signals underlying malignancy. Cicatricial pemphigoid and linear IgA bullous disease are rare subepidermal blistering diseases that occur more frequently in older adults. Coma bullae are typically seen in elderly patients who have fallen and lain unconscious for hours or days. Stasis bullae can develop in patients who have limited or no mobility. These lesions result from chronic edema caused by hypoalbuminemia, venous thrombosis, congestive heart failure, or hepatic and renal failure.

Although inflammatory dermatoses are generally less prevalent among elderly persons than among younger adults, some of these dermatoses—particularly vesiculobullous eruptions—are more common in patients over age 60. In this second part of my 2-part series on papulosquamous and bullous cutaneous diseases in older adults, I review the diagnosis and treatment of pemphigus vulgaris, dermatomyositis, cicatricial pemphigoid, linear IgA bullous disease, coma bullae, and stasis bullae. In part 1 (CONSULTANT, March 2011, page 168), I discussed psoriasis, bullous pemphigoid, and Grover disease.

PEMPHIGUS VULGARIS

Clinical features. Pemphigus vulgaris belongs to the group of systemic autoimmune blistering diseases of the skin and mucous membranes. The mean age of onset is about 50 to 60 years. Because pemphigus can be life-threatening, prompt diagnosis is crucial.

Figure 1 – The flaccid blisters of pemphigus vulgaris rupture easily and leave painful erosions, as seen in this elderly woman.

In patients with pemphigus vulgaris, flaccid blisters develop on the skin; these rupture easily, leaving painful denuded surfaces (Figure 1) that can sometimes be crusted (Figure 2) and are often geometric in shape. Bullae and erosions typically manifest on the upper trunk; head; neck; and intertriginous areas, such as the axillae (Figure 3) and groin. The disease usually begins in the oral cavity. It may be localized, or it may become generalized and involve 20% to 50% of the skin in severe disease. Involvement of ocular, nasal, pharyngeal, laryngeal, esophageal, vaginal, penile, and anal mucosa typically occurs in severe disease. Healing usually occurs without scarring and is often accompanied by postinflammatory hyperpigmentation.

Figure 2 – Pemphigus vulgaris manifests in this man as crusted plaques.

Figure 3 – Extensive axillary involvement is evident in this man with pemphigus vulgaris.

Treatment. Corticosteroids are the mainstay of treatment. Immunosuppressants, such as mycophenolate mofetil and azathioprine, can be used as corticosteroid-sparing agents. Rituximab and intravenous immunoglobulin have also been effective in patients with pemphigus vulgaris.1

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