Pemphigus vulgaris with early eye involvement

To the Editor,

More than half the patients with pemphigus vulgaris (PV) present with oral lesions which may precede the cutaneous lesion by several months.[1] Other mucous membranes like conjunctiva, vulva, cervix uteri, oesophagus and rectal mucosa may also be involved. We report a case of PV who primarily had eye involvement and subsequently developed oral, genital and skin lesions.

A 24-year-old male presented with redness and pain of eyes for 10 months, oral ulcers for 4 months, multiple ulcers on penile and scrotal skin and single vesicle on right axilla for 10 days. The eye problem was initially diagnosed by an ophthalmologist as spring catarrh and patient was given subconjunctival injection of 20 mg of triamcinolone acetonide. With this, patient was free of eye symptoms for 1½ months, after which the redness of eye recurred. On examination both bulbar conjunctivae showed erythema, congestion and mild erosion. There were multiple superficial ulcers and whitish patches on the mucosa of cheeks, palate, tongue and lips. One to two small flaccid vesicles were present on right axilla and buttock. Penile shaft and scrotal skin showed multiple small superficial ulcers with purulent discharge. Nikolsky sign was positive. Tzanck smear showed acantholytic cells. Histology from oral mucosa and skin showed suprabasilar split with acantholytic cells within the cleft and single row of basal cells below the cleft, as the main features, which was consistent with pemphigus vulgaris. Direct immunofluorescence could not be done due to non-availability. Routine haemogram, blood sugar, renal and the liver function tests were normal. X-ray chest was normal.

Patient was started on 60mg equivalent of prednisolone daily and all his lesions resolved within 2 weeks. The dose of steroid was gradually tapered to 30 mg/day and patient is on follow up.

On reviewing the literature, there are many reports of PV initially presenting on the oral mucosa followed by skin lesions and associated involvement of other mucous membranes.[1] Although PV can initially manifest as eye lesions,[2] the unusual feature in this patient was the long duration of eye involvement alone for 6 months before oral and skin lesions appeared. No such report could be found in earlier literature review.