16 August 2006 – Case of the Week #56
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We thank Dr. Ankur Sangoi, Stanford University, Stanford, California (USA) for contributing this case. We invite you to contribute a Case of the Week by sending an email to NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG or GIF format, a short clinical history, your diagnosis and any other images (gross, immunostains, EM, etc.) that may be helpful or interesting. We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) for your time after we send out the case. Please only send cases with a definitive diagnosis.
Case of the Week #56
The patient is an 85 year old Mexican woman with a one month history of very pruritic and scaly eroded plaques on her trunk with a few on her face. There were no mucosal lesions or intact vesicles.
Microscopic images: low power #1, #2, #3, high power, C3, IgG
What is your diagnosis?
(scroll down to continue)
Pemphigus is a collection of rare, nonhereditary, chronic, autoimmune diseases with flaccid blisters and denuded skin. They typically affect men and women equally, usually ages 30 to 59 years.
Pemphigus foliaceus is a chronic blistering disease that typically spares the mucous membranes, but involves healthy appearing skin with blisters that form after rubbing (Nikolsky’s sign). It typically has a mild course because the blisters are superficial. Often the lesions do not appear bullous because crusts and erosions replace the bullae. Histologically, there is acantholysis of the granular cell layer of the epidermis, with rounded keratinocytes and a few inflammatory cells. Eosinophils may be present.
The blisters in pemphigus foliaceus are due to IgG antibodies against desmoglein 1, a desmosomal glycoprotein expressed within the granular cell layer. IgG immunostains for this antibody show a net-like pattern. C3 deposition has also been described (Br J Dermatol 1997;136:249)
An endemic form, called fogo selvagem (“wild fire” in Portugese) occurs in Brazil (Br J Dermatol 2006;155:446, Int J Dermatol 2005;44:293).
Treatment is typically corticosteroids or immunosuppressive agents.
The differential diagnosis includes other forms of pemphigus (erythematosus-also superficial cleavage but typically involves the face; vulgaris and vegetans-deep cleavage planes) and other acantholytic diseases, such as Darier’s disease, Hailey-Hailey disease, viral vesicles, drug related pemphigus-like lesions, and transient acantholytic dermatosis. Patients with pemphigus foliaceus may also have other autoimmune disorders or thymoma.
Nat Pernick, M.D.
30100 Telegraph Road, Suite 404
Bingham Farms, Michigan (USA) 48025