Chapter 157: Other Bullous Diseases

Introduction

There are a number of bullous diseases other than pemphigus and bullous pemphigoid that are important to recognize. Epidermolysis bullosa belongs to a family of inherited diseases where blister formation can be caused by even minor skin trauma. PLEVA (pityriasis lichenoides et varioliformis acuta) is a minor cutaneous lymphoid dyscrasia that can appear suddenly and persist for weeks to months. Dermatitis herpetiformis is a recurrent eruption that is usually associated with gluten and diet-related enteropathies. These diseases will be discussed in succession.

Epidermolysis Bullosa

Patient Story

A 12-year-old girl with the Dowling-Meara type of epidermolysis bullosa (EB) simplex presents to her pediatrician for a URI. While examining her respiratory tract, the pediatrician notes the extensive, severe blistering over many areas of the body including, the (A) trunk, (B) extremities, and (C) the hands (Figure 157-1). She has been followed by a dermatologist since early childhood when the EB simplex was first diagnosed. It turns out that she only has a viral URI so no antibiotics are needed and standard treatment with fluids and analgesics is recommended. Her mom states that the girl has an appointment with her dermatologist next month.

Epidemiology

• Epidermolysis bullosa (EB) is a family of inherited diseases characterized by skin fragility and blister formation caused by minor skin trauma.¹

• There are autosomal recessive and autosomal dominant types; the severity of this disease may vary widely.

• Onset is in childhood and in later years severe dystrophic deformities of hands and feet are characteristic (Figure 157-2).

Etiology and Pathophysiology

Blistering occurs at different levels for these 3 types of EB:

1. Epidermolysis bullosa simplex (Figure 157-1) blisters within the epidermis (most superficial).^2,3

2. Dystrophic epidermolysis bullosa (dominant and recessive) has vesiculobullous skin separation occurring at the sub-basal lamina level of the dermis (deepest layer of all 3 types; Figures 157-2 to 157-4).

3. Junctional epidermolysis bullosa blisters at the dermal-epidermal junction (Figure 157-5).