Letters to the Editor

6. 7.

successfully treated with botulinum toxin A. Dermatol Surg 2009;35:1300-2. Prabhu G, Laddha P, Manglani M, Phiske M. Hidradenitissuppurativa in a HIV-infected child. J Postgrad Med 2012;58:207-9. Randhawa HK, Hamilton J, Pope E. Finasteride for the treatment of hidradenitissuppurativa in children and adolescents. JAMA Dermatol 2013;149:732-5.

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Pemphigus vulgaris localised exclusively to the penis Sir, Pemphigus vulgaris is a chronic, autoimmune bullous disease affecting the mucosae and skin. Though disease usually starts in the oral mucosa, which is the most frequently affected site, involvement of other mucosae may be seen.[1] There are only a few reports documenting the involvement of penile skin[2] of which only a single report documents penile erosions as the first manifestation of pemphigus vulgaris in two patients. In both of these cases, pemphigus vulgaris started in the penile area, followed by oral mucosal lesions in both and skin erosions in one patient.[3] We report here a patient with chronic, painful penile erosions with maceration who was diagnosed as pemphigus vulgaris. We were unable to find previous reports of isolated penile pemphigus vulgaris. A 49-year-old male was admitted to our hospital with painful penile erosions that were slowly progressive for 3½ months. The patient had previously responded poorly to courses of topical and systemic antiviral, antibiotic, antifungal agents, and topical corticosteroids. On dermatological examination, multiple confluent erythematous erosions were observed on the glans, corona, and shaft of the penis. The penile skin had a whitish macerated and fragile appearance [Figure 1a]. There were no mucosal or cutaneous lesions elsewhere on the body. Treponema pallidum hemagglutination assay (TPHA) and enzyme-linked immunosorbent assay (ELISA) for herpes simplex virus (HSV) type 2 IgM and IgG were negative. A punch biopsy 298

taken from the penile erosion revealed suprabasal blistering with acantholysis [Figure 2a and b]. Direct immunofluorescence (DIF) studies of the perilesional penile mucosa demonstrated IgG and complement (C3) deposits in the intercellular area [Figure 3a and b]. ELISA for Dsg3 and Dsg1 were positive in 1:1000 and 1:100 dilutions respectively (normal < 1:10). Based on the results of clinical evaluation and histopathological and immunofluorescence studies a diagnosis of pemphigus vulgaris localized to the penis was made. The patient was treated with oral prednisone (1 mg/kg) and azathioprine (2 mg/kg/day). The penile lesions cleared completely after 3 months of therapy [Figure 1b] after which the prednisone dose was tapered gradually. Additional skin and other mucosal lesions were not observed during follow-up. The patient remains in remission after 16 months of follow up. Mucosal involvement occurs commonly in pemphigus vulgaris. Although the oral mucosa is involved most

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Figure 1: (a) Multiple erythematous erosions with whitish maceration on the penis. (b) Complete healing occurred after treatment

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Figure 2: (a) Suprabasal acantholytic blister formation. (H and E ×100) (b) The basal cells show a tombstone appearance together with acantholytic cells (H and E ×400)

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Figure 3: Intercellular epidermal staining for complement C3 (a) and IgG (b) (×100)

Indian Journal of Dermatology, Venereology, and Leprology | May-June 2015 | Vol 81 | Issue 3

Letters to the Editor

frequently, other mucosal areas may also be affected, albeit less commonly. In a retrospective analysis of 1209 cases, Chams-Davatchi et al.[4] reported involvement of the oral cavity, conjunctiva, nasal, vaginal, laryngeal/esophageal, and perineal mucosae in 81%, 16%, 11%, 9%, 8%, and 2% of cases, respectively. Akhyani et al. have reported a much higher incidence of genital mucosal involvement in 51% of 77 cases with muco-cutaneous involvement.[5] According to this study, the genital mucosa was the second most commonly affected mucosal site in pemphigus vulgaris. In another study, the presence of genital lesions was found to be associated with treatment resistance and was recognized as a poor prognostic factor and a marker of disease severity.[1]

Address for correspondence: Dr. Ozlem Su, Sigirtmac Sokak, Park Solare Sitesi, B Blok, Daire 12 Osmaniye/Bakirköy, Istanbul, Turkey. E-mail: [email protected]

REFERENCES 1. 2. 3. 4. 5.

Malik M, Ahmed AR. Involvement of the female genital tract in pemphigus vulgaris. Obstet Gynecol 2005;106:1005-12. Sami N, Ahmed AR. Penile pemphigus. Arch Dermatol 2001;137:756-8. Stieger M, Pelivani N, Ramelet AA, Beltraminelli H, Borradori L. Penile erosions as first manifestation of pemphigus vulgaris: A misleading presentation. Acta Derm Venereol 2013;93:248-9. Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z, et al. Pemphigus: Analysis of 1209 cases. Int J Dermatol 2005;44:470-6. Akhyani M, Chams-Davatchi C, Naraghi Z, Daneshpazhooh M, Toosi S, Asgari M, et al. Cervicovaginal involvement in pemphigus vulgaris: A clinical study of 77 cases. Br J Dermatol 2008;158:478-82.

Genital involvement in males with pemphigus vulgaris has been reported only rarely.[2,3] Sami and Ahmet reported involvement of the penile skin in 12 male patients with pemphigus. However, in none of these cases was the disease limited to penile skin only.[2] Recently, Stieger et al.[3] described two cases of pemphigus vulgaris presenting with erosions over the glans penis and coronal sulcus. In both these patients, though the disease started from penile area it later affected the oral mucosa also. One of the patients also developed cutaneous lesions on follow up.[3] The initial presentation with penile erosions alone led to a delay in diagnosis in both these cases. Both patients were treated with 1 mg/kg/body weight prednisone and azathioprine.

Pemphigus vulgaris associated with surgery: A rare association

Possible differential diagnoses for long-standing penile erosions include infectious etiologies (herpes simplex virus, syphilis, candidiasis, and mycobacterial infection), fixed drug eruption, erosive lichen planus, genital aphthosis of Behçet’s disease, and premalignant and malignant conditions like plasma cell balanitis, Paget’s disease, erythroplasia of Queyrat and squamous cell carcinoma. Because isolated penile involvement as a presenting feature of pemphigus vulgaris is not commonly described, the diagnosis may be missed. We also believe that the presence of genital lesions in pemphigus vulgaris is indicative of likelihood of resistance to treatment and these patients may require high-dose systemic steroids and stronger immunosuppressive regimens.

Sir, A 63-year-old Chinese woman was admitted with blisters and erosions with scabbing, involving the trunk, limbs and the scalp for 1 year. A few blisters initially appeared around a surgical incision, 2 weeks following an appendectomy. It was associated with pain and pruritus. The blisters were flaccid, and ruptured easily to form erosions and scabs. The lesions then began to appear all over the body over the next six months involving the trunk, limbs and scalp. She was diagnosed to have a skin infection and treated with debridement of the surgical wound along with systemic and topical antibiotics at a local hospital. The lesions did not resolve, after which she consulted at our hospital.

Ozlem Su, Didem Dizman, Dilek Biyik Ozkaya, Pelin Yildiz1, Cuyan Demirkesen2, Nahide Onsun

Cutaneous examination revealed flaccid vesicles and bullae containing clear liquid, along with crusts. They were distributed over the trunk, extremities, and around the appendectomy scar in the right iliac fossa. The face, mucous membranes, and perineum were not

Departments of Dermatology and Venereology, 1Pathology, Bezmialem Vakif University, Medical Faculty, 2Department of Pathology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey

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