May 2009 – Case of the Month #3
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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 Month by sending microscopic images in JPG or GIF format, with a clinical history and any other images (gross, dermoscopy, immunostains, etc.) that may be helpful, to NatPernick@Hotmail.com. We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) after we send out the case. Please only send cases with a definitive diagnosis.
Dermatology Case of the Month #3
Clinical History
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.
Micro images: low power #1; #2; #3; high power; C3; IgG
What is your diagnosis?
(scroll down to continue)
Diagnosis:
Pemphigus Foliaceus
Discussion
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 (Am J Pathol 2008;173:1628). 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. Immunoglobulin may be useful for pemphigus patients who are resistant to steroids (J Am Acad Dermatol 2009;60:595).
The differential diagnosis includes pemphigus vulgaris (deep cleavage planes) and other acantholytic diseases, such as Darier’s disease, Hailey-Hailey disease (inherited), viral vesicles, drug related pemphigus-like lesions, and transient acantholytic dermatosis. Patients with pemphigus foliaceus may also have other autoimmune disorders or thymoma.
References: eMedicine, Wikipedia
Nat Pernick, M.D., President
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