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.
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.
A 12-year-old girl with the Dowling-Meara type of epidermolysis bullosa simplex. It is the most severe form with extensive, severe blistering over many areas of the body including, the (A) trunk, (B) extremities, and (C) the hands. (Used with permission from Richard P, Usatine, MD.)
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).
Recessive dystrophic epidermolysis bullosa in an adolescent with a mitten deformity and flexion contractures at the wrists. (Used with permission from Kane KS, Lio PA, Stratigos AJ, Johnson RA. Color Atlas & Synopsis of Pediatric Dermatology, 2nd edition, Figure 5-3b, New York, NY: McGraw-Hill, 2009.)
Etiology and Pathophysiology
Blistering occurs at different levels for these 3 types of EB:
Epidermolysis bullosa simplex (Figure 157-1) blisters within the epidermis (most superficial).2,3
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).
Junctional epidermolysis bullosa blisters at the dermal-epidermal junction (Figure 157-5).
Log In to View More
If you don't have a subscription, please view our individual subscription options below to find out how you can gain access to this content.
Want remote access to your institution's subscription?
Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.
If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.
AccessPediatrics Full Site: One-Year Subscription
Connect to the full suite of AccessPediatrics content and resources including 20+ textbooks such as Rudolph’s Pediatrics and The Pediatric Practice series, high-quality procedural videos, images, and animations, interactive board review, an integrated pediatric drug database, and more.
Pay Per View: Timed Access to all of AccessPediatrics
24 Hour Subscription $34.95
48 Hour Subscription $54.95
Pop-up div Successfully Displayed
This div only appears when the trigger link is hovered over.
Otherwise it is hidden from view.