Bullous pemphigoid in disguise!!
Bullous pemphigoid (BP) is a common cause of pruritus in the elderly population. I came across three cases in the last 3 months with my colleague Dr Eman El Nabarawy, in which the disease did not present with the classic tense bullae that we are all used to.
All three cases were elderly who presented with intense itching of long durations associated with erythematous plaques, some are edematous, erythematous papules and crusted lesions. Bullous pemphigoid was not necessarily present in the given clinical differential diagnosis. Biopsies from the papules or the plaques showed subepidermal edema, vacuolar degeneration along the DEJ and variable degrees of spongiosis with esinophils within the epidermis. An esinophil rich infiltrate was seen in the dermis. Only focally, subepidermal clefting was seen. In one case, the biopsy showed an excoriated area of the epidermis, which could have been easily overlooked as non-specific manifestation of itching, however, few esinophils within the epidermis and a tiny subepidermal cleft beside the excoriated area were noticed. Biopsies from crusted lesions showed intact dermal papillae pattern beneath the crust as well as esinophil rich infiltrate. DIF studies confirmed BP.
Dermatologists should be aware of BP as a cause of persistent itching in elderly even in the absence of blisters. Pre-bullous stages of the diseases may be prolonged and in some occasions, the disease only presents with non bullous lesions. The most important thing is that these pre-bullous (or non bullous) lesions are not necessarily urticarial, they may be eczematous or crusted. Although, esinophils within the epidermis can be caused by many causes, I am starting to take them seriously as a sign more linked with BP at least in an elderly patient presenting with persistent itching.
All three cases were elderly who presented with intense itching of long durations associated with erythematous plaques, some are edematous, erythematous papules and crusted lesions. Bullous pemphigoid was not necessarily present in the given clinical differential diagnosis. Biopsies from the papules or the plaques showed subepidermal edema, vacuolar degeneration along the DEJ and variable degrees of spongiosis with esinophils within the epidermis. An esinophil rich infiltrate was seen in the dermis. Only focally, subepidermal clefting was seen. In one case, the biopsy showed an excoriated area of the epidermis, which could have been easily overlooked as non-specific manifestation of itching, however, few esinophils within the epidermis and a tiny subepidermal cleft beside the excoriated area were noticed. Biopsies from crusted lesions showed intact dermal papillae pattern beneath the crust as well as esinophil rich infiltrate. DIF studies confirmed BP.
Dermatologists should be aware of BP as a cause of persistent itching in elderly even in the absence of blisters. Pre-bullous stages of the diseases may be prolonged and in some occasions, the disease only presents with non bullous lesions. The most important thing is that these pre-bullous (or non bullous) lesions are not necessarily urticarial, they may be eczematous or crusted. Although, esinophils within the epidermis can be caused by many causes, I am starting to take them seriously as a sign more linked with BP at least in an elderly patient presenting with persistent itching.
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