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INTRODUCTION

KEY POINTS

  • The causes of acquired bullous diseases are multiple and can be broadly differentiated based on the age of onset and the chronicity of the disease.

  • Autoimmune blistering diseases can be challenging to diagnose and treat.

  • Potential issues that will affect management of autoimmune bullous diseases in patients with skin of color include a difference in prevalence of specific autoimmune bullous diseases, disease presentation, postinflammatory dyspigmentation and scarring, and for some, access to care.

  • Large multicenter controlled trials are needed to show differences in response to treatment, if any, between patients with skin of color and Caucasian patients with autoimmune bullous diseases.

Cutaneous blisters appear because of disruption of the desmosomal or hemidesmosomal cell junctions or because of cytolysis of keratinocytes. The differential diagnoses are diverse, and the epidemiology and presentation of cutaneous blisters can be different in patients with darker skin. Advances in treatment have improved the outlook for patients with chronic blistering diseases such as autoimmune blistering diseases and hereditary epidermolysis bullosa, but these treatments can be expensive, require regular monitoring and follow-up, and may not be routinely available. The main concern for patients with skin of color is cosmetically significant postinflammatory hyper- and hypopigmentation as well as potential cutaneous scarring after resolution of the blistering disease.

In this chapter, we will present an approach to diagnosing blistering diseases. We will then focus on the epidemiology, clinical presentation, diagnosis, and treatment of autoimmune blistering diseases and highlight the recent literature involving African, Hispanic, and Asian patient populations.

APPROACH TO BLISTERING DISEASES

An approach to diagnosing blistering diseases is to consider the age of the patient and the tempo of the clinical presentation [Figures 32-1 and 32-2].

FIGURE 32-1.

Approach to diagnosing blistering diseases in children.

FIGURE 32-2.

Approach to diagnosing blistering diseases in adults. PUVA, psoralen plus ultraviolet A.

Blistering genodermatoses are rare and present predominantly during childhood. Epidermolysis bullosa (EB) is due to an inherited defect of structural components of basal keratinocytes (such as hemidesmosomal proteins, keratins 5 and 14, desmoplakin, and plakophillin) and demonstrates varying severity of skin and extracutaneous involvement.1,2,3,4 Some types of EB present in older children and can be mistaken for acquired blistering diseases. These include localized EB simplex with palmoplantar bulla, late-onset junctional EB, or dystrophic EB pruriginosa. Other causes of inherited childhood blistering diseases include cutaneous porphyrias5 and epidermolytic ichthyosis.6 Although inherited blistering diseases predominantly present in childhood, the most frequent cause of blistering in children is still from acquired causes [Figure 32-1].

Acquired blistering diseases are the predominant form of blistering disease seen in adults [Figure 32-2]. ...

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