Figure 1.1
(a) Excoriations, postinflammatory hypopigmentations and atrophic scarring on trunk and arms; (b) Close-up view of excoriated lesions and scarring with isolated milia on arm; (c) Erosions, crusting and atrophic scarring on the scalp; (d) Erosion on buccal mucosa
What Is Your Diagnosis?
Bullous pemphigoid (BP)
Mucous membrane pemphigoid
Brunsting-Perry pemphigoid
Discussion (1)
Diagnosis of BP is not always easy and straightforward. Manifestations of BP might resemble those of a variety of dermatoses, including drug reactions, contact dermatitis, prurigo, fixed urticaria, vasculitis, arthropod reaction and scabies (Table 1.1). Although the recent availability of ELISAs have facilitated the search of circulating autoantibodies, diagnosis still relies on a combination of clinical, histopathological and immunopathological features, particularly direct IF microscopy findings [5].
Table 1.1
Clinical presentations of bullous pemphigoid
Chronic prurigo, prurigo nodularis-like features |
Papular pemphigoid |
Eczematous lesions |
Erythema multiforme-like and Lyell-like pemphigoid |
Lymphomatoid papulosis-like |
Ecthyma-like |
Palmo-plantar lesions (dysidrosiform pemphigoid) |
Intertrigo (vegetating pemphigoid) |
Vesicular pemphigoid |
Erythrodermic pemphigoid |
Brunsting-Perry form (variant of cicatricial pemphigoid with skin lesions) |
Localized forms |
Pretibial |
Peristomal |
Umbilical |
“Stump” pemphigoid |
On paralyzed body sites |
On irradiated/traumatised body sites |
Our patient presented with chronic excoriated lesions and post-inflammatory changes predominantly localized on the upper trunk and his head and as well as isolated lesions of the buccal mucosa. Immunopathological findings were consistent with the pemphigoid group of autoimmune bullous disorders. We favor the diagnosis of an unusual form of chronic prurigo-like BP [5]. Nevertheless, our case presented also with features of the so called Brunsting-Perry variant of mucous membrane pemphigoid [2] with a peculiar extensive cutaneous involvement. In the absence of well recognized criteria, a conclusive classification of our case is not possible.