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Image Correspondence
ARTICLE IN PRESS
doi:
10.25259/IJPGD_236_2025

Isolated Vulval Pemphigus Vulgaris

Department of Dermatology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

*Corresponding author: Krati Mehrotra Department of Dermatology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India. kratimehrotra10@gmail.com

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Kumawat P, Mehrotra K, Yadav AK, Khunger N. Isolated Vulval Pemphigus Vulgaris. Indian J Postgrad Dermatol. doi: 10.25259/IJPGD_236_2025

A 38-year-old female presented with a complaint of a single, painful lesion on her vulva for 4 months. On examination, a single erythematous ulcer of size 4 × 0.5 cm, well-defined margins and non-tender was present on the inner aspect of the left labia minora [Figure 1a]. The venereal disease research laboratory serology was non-reactive. Gram’s stain and herpes simplex virus polymerase chain reaction (PCR) were negative. Histopathological examination showed a focal suprabasal split and a row of tombstone-like appearance of basal keratinocytes [Figure 1b and c]. Direct immunofluorescence showed IgG and C3 intercellular deposits [Figure 1d]. The patient had desmoglein 3 antibody positivity, thus confirming the diagnosis of pemphigus vulgaris.

(a) Single erythematous ulcer of size 4 × 0.5 cm with well-defined margins, non-tender, present on the inner aspect of the left labia minora (black arrow). (b) Photomicrograph showing supra-basal split (H& E, ×100) (blue arrow). (c) Photomicrograph showing a row of tomb-stone like appearance of basal keratinocytes (H&E, ×400) (black circle). (d) Direct immunofluorescence showing intercellular deposition of IgG in the epidermis (×400).
Figure 1:
(a) Single erythematous ulcer of size 4 × 0.5 cm with well-defined margins, non-tender, present on the inner aspect of the left labia minora (black arrow). (b) Photomicrograph showing supra-basal split (H& E, ×100) (blue arrow). (c) Photomicrograph showing a row of tomb-stone like appearance of basal keratinocytes (H&E, ×400) (black circle). (d) Direct immunofluorescence showing intercellular deposition of IgG in the epidermis (×400).

In view of unresponsiveness to oral steroids and azathioprine, she was treated with 2 doses of rituximab injection with complete remission [Figure 2a and b]. We highlight the importance of isolated lesions of pemphigus on the vulva and it is important to remember the non-venereal causes of genital ulcers.

(a) Post rituximab (induction phase) – ulcer reduction, (b) Post rituximab – complete clearance of ulcer at 6 months.
Figure 2:
(a) Post rituximab (induction phase) – ulcer reduction, (b) Post rituximab – complete clearance of ulcer at 6 months.

Ethical approval:

Institutional Review Board approval is not required.

Declaration of patient consent:

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient have given their consent for their images and other clinical information to be reported in the journal. The patient understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Financial support and sponsorship: Nil.


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