Indian Journal of Dermatology
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Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 107
Pyoderma gangrenosum in a patient with pemphigus vulgaris: An unusual association

1 Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Amrinder J Kanwar
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.147892

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How to cite this article:
Keshavamurthy V, Kanwar AJ, Saikia UN. Pyoderma gangrenosum in a patient with pemphigus vulgaris: An unusual association. Indian J Dermatol 2015;60:107

How to cite this URL:
Keshavamurthy V, Kanwar AJ, Saikia UN. Pyoderma gangrenosum in a patient with pemphigus vulgaris: An unusual association. Indian J Dermatol [serial online] 2015 [cited 2020 Sep 23];60:107. Available from: http://www.e-ijd.org/text.asp?2015/60/1/107/147892


Pyoderma gangrenosum (PG) is a rare inflammatory condition classified under neutrophilic dermatosis. Its pathogenesis is largely unknown; however, its association with systemic diseases in nearly 50% of cases points towards an underlying immune dysregulation. Although PG has been mainly seen in association with inflammatory bowel disease, arthritis and hematological malignancies, it has been described in association with various autoimmune disorders. However, till date PG has not been reported in association with pemphigus vulgaris (PV).

A 27-year-old female patient of oral PV for 8 months presented with complains of rapidly progressive, painful ulcer over the left thigh and a few flaccid vesicles as well as bullae over the trunk and proximal lower extremities since 1 week. The ulcer started as a painful erythematous nodule, which soon ruptured to form a necrotic ulcer with copious purulent discharge. Her current treatment included oral prednisolone 10 mg/day and azathioprine 100 mg/day. On examination, a deep necrotic ulcer of size 4×6 cm was noted over the anterolateral aspect of left thigh [Figure 1]a. The ulcer was exquisitely tender and had a necrotic purulent base with surrounding erythema and semi-adherent greenish brown crust. Surrounding the ulcer and over the trunk were 8-10 flaccid blisters containing clear fluid [Figure 1]a. Nikolsky's sign was positive. Oral mucosal examination revealed erosions involving the gingival margins and buccal mucosa [Figure 1]b.
Figure 1: (a) Clinical photography showing a large necrotic ulcer, with purulent base and surrounding erythema over the anterolateral aspect of left thigh. A flaccid bulla can also be appreciated below the ulcer (arrow). (b) Clinical photography showing gingival erosions

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A skin biopsy from the ulcer margin showed necrotic epidermis with intense neutrophilic infiltrate, capillary proliferation and fibrin deposition [Figure 2]a. A biopsy from the edge of the bulla showed suprabasal acantholysis with tomb stone appearance of basal cells [Figure 2]b. Direct immunofluorescence (DIF) from perilesional skin showed intercellular deposits of IgG and C3. Bacterial, fungal and mycobacterial culture of the pus did not show any organism growth. Hematological and biochemical parameters were within normal limits. Serological analysis for anti-nuclear antibody and rheumatoid factor were negative. Based on these features a diagnosis of PG in a patient of mucocutaneous PV was made. The patient was started on oral prednisolone 80 mg per day and azathioprine 100 mg/day was continued. The cutaneous lesions of PV disappeared within a week and the ulcer showed signs of healing. Oral steroids were tapered in 4 months and the cutaneous lesion healed with scarring. After a follow-up duration of 8 months the patient continues to be in remission with no other systemic abnormalities.
Figure 2: (a) Photomicrography of lesional skin biopsy from ulcer margin showing necrotic epidermis with intense neutrophilic infiltrate and fibrin deposition, H and E × 10X. (b) Photomicrography of a lesional skin biopsy from flaccid vesicle showing suprabasal acantholysis with tomb stone arrangement of basal keratinocytes, H and E × 40X

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PG has been described in association with various systemic and cutaneous disorders. Since there are no serological or pathological investigations that can diagnose PG with certainty, the diagnosis of PG is based on clinical and histological features, exclusion of other disorders with similar presentation and therapeutic response to systemic steroids. Su et al. [1] have proposed diagnostic criteria for diagnosis of PG and our patient fulfilled two major and three minor criteria. On extensively reviewing the literature, we found no previous report of coexistence of PG and PV. Oral lesions of PV can be misdiagnosed as pyostomatitis vegetans the mucosal equivalent of PG. [2] The presence of suprabasal acantholytic cleft on histopathology and the presence of intercellular immune deposits on DIF help in differentiating these two entities. [2] It is well established that IL-8, a neutrophilic chemoattractant, is an important cytokine in the pathogenesis of PG. [3] The role of IL-8 in PV is controversial. [4] However, Keskin et al. in an 18 month prospective study of 10 patients found that there was a significant reduction in the level of IL-8 following treatment in comparison to baseline values. [5] It is possible that in our patient acute flare of PV (transformation of mucosal PV to mucocutaneous PV) was associated with an elevated level of IL-8, contributing to the pathogenesis of PG. In conclusion, we report a previously undescribed association of PG with PV.

   References Top

Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: Clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol 2004;43:790-800.  Back to cited text no. 1
Nico MM, Hussein TP, Aoki V, Lourenço SV. Pyostomatitis vegetans and its relation to inflammatory bowel disease, pyoderma gangrenosum, pyodermatitis vegetans, and pemphigus. J Oral Pathol Med 2012;41:584-8.  Back to cited text no. 2
Oka M, Berking C, Nesbit M, Satyamoorthy K, Schaider H, Murphy G, et al. Interleukin-8 overexpression is present in pyoderma gangrenosum ulcers and leads to ulcer formation in human skin xenografts. Lab Invest 2000;80:595-604.  Back to cited text no. 3
Mortazavi H, Babaeijandaghi F, Akbarzadeh M, Rezaei N, Amirzargar AA, Daneshpazhooh M, et al. The influence of systemic therapy on the serum levels of IL-6 and IL-8 in pemphigus vulgaris. J Eur Acad Dermatol Venereol 2013;27:387-90.  Back to cited text no. 4
Keskin DB, Stern JN, Fridkis-Hareli M, Razzaque Ahmed A. Cytokine profiles in pemphigus vulgaris patients treated with intravenous immunoglobulins as compared to conventional immunosuppressive therapy. Cytokine 2008;41:315-21.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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