A verrucous 'tumour' on the scalp.

Case History A 67-year-old Sikh male was diagnosed with pemphigus vulgaris in 1996 when he presented with oral erosions. His disease remained controlled on long-term, low-dose prednisolone therapy. In 2004, he developed a verrucous keratotic plaque over the occipital region of his scalp and it had been gradually enlarging (Fig. 1). Biopsies were performed in 2004 and 2008 and histological features in the epidermis of the specimens consist of orthokeratosis, parakeratosis, digitated epidermal hyperplasia, extensive suprabasal clefting, and acantholysis (Fig. 2). A dense infi ltrate of plasma cells and lymphocytes were seen in the dermis. Direct immunofl uorescence showed intercellular IgG and C3 deposition throughout epidermis. Indirect immunofl uorescence was positive with antibody titers of more than 1/160. The levels of anti-desmoglein 3 antibody and anti-desmoglein 1 antibody were also elevated at 206 U/mL (negative if value is less than 7) and 81.4 U/mL (negative if value is less than 14) respectively. What is the diagnosis? A. Squamous cell carcinoma B. Skin metastases from a primary cancer C. Pemphigus vegetans D. Vegetating cicatricial pemphigoid E. Vegetating pyoderma Discussion Pemphigus vegetans is the least common variant of pemphigus vulgaris. The diagnosis of pemphigus vulgaris is supported by the patient's background history, presence of erosions within the lesion, histological features of suprabasal diffuse acantholysis, features on direct immunofl uorescence study, and positive indirect immunofl uorescence and desmoglein 1 and 3 levels. The levels of desmoglein 1 and 3, in addition, correlate with disease activity. There are 2 forms of pemphigus vegetans. Our patient has the Neumann subtype, which is typifi ed by bullae coalescing into vegetating masses, evolving into dry, hyperkeratotic and fi ssured lesions. This type is often refractory to treatment. The other form is the Hallopeau subtype, consisting of a polycyclic eruption of pustules that transform into papillomas and eventually fl attening into dark brown plaques. Spontaneous remission is possible for this latter subtype. The mainstay of treatment is systemic corticosteroids. Combinations with immunosuppressants (such as mycophenolate mofetil) or etretinate have also been tried with success. 1 Other treatment modalities include utilising extracorporeal photopheresis (ECP), carbon dioxide laser and anti-CD20 (Rituximab). 2,3 It is important to note that the underlying disease activity in pemphigus should be adequately suppressed to prevent recurrence of the lesions. In our patient, he received a variety of topical medications for his scalp lesion, consisting of betamethasone 0.1% scalp lotion, 5-fl uorouracil, and 10% salicyclic acid. …