Pemphigus vulgaris in an elderly patient.

Sir,

We report here a 94-year-old woman who was apparently all right till 2 years back. However, when she developed soreness of oral cavity that was treated with multi-vitamins without much relief, she developed flaccid blisters for 2 months on the chest, abdomen, and forearms. There were erosions of buccal mucosa with edema of lips and gums and the lower lip showed crusts. Well-demarcated hyperpigmented circular patches were seen over the chest and abdomen as well as a sequel of rupture of bullae with some crusted lesions was seen [Figure 1]. Shearing pressure on peri-lesional skin and periphery of the lesion demonstrated peeling of the skin (positive marginal Nikolskiy's sign). Skin biopsy taken from a freshly developed vesicle on the right forearm showed supra-basal cleft formation and row of “tomb-stone” appearance of few basal cells [Figure 2], which was suggestive of a diagnosis of pemphigus vulgaris (PV). Direct immunofluorescence of peri-lesional biopsy showed “fish-net” appearance with IgG deposits in the intercellular spaces [Figure 3], which confirmed the diagnosis of PV.

Figure 1

Multiple discoid crusted plaques on an erythematous base over abdomen

Figure 2

Photomicrograph (H and E, ×400) showing supra-basal cleft with tomb-stone appearance of basal cells

Figure 3

Fish-net appearance seen in direct immunofluorescence

The patient was started on 40 mg of prednisolone and 50 mg of cyclophosphamide, and she responded within 15 days with clearance of oral lesions and reduction in skin lesions in the next 2 months. Since it is a great risk to keep a geriatric patient on high doses of steroid for longer time, cyclophosphamide was introduced little early as a steroid-sparing drug, and it is also helpful in early tapering of steroids.

Pemphigus is a chronic epidermal immunobullous disease with potentially fatal outcome. Corticosteroid based treatment, along with adjuvants, has significantly brought down the high mortality rates that had been observed in pre-corticosteroid era.[1] Survival of the patients has improved dramatically since systemic corticosteroids were introduced; however, high doses of corticosteroids are required to suppress blistering. Morbidity and mortality from pemphigus today is directly proportional to the dose of corticosteroid administered. Due to the problem of corticosteroid-related side effects, adjuvant immunosuppressive agents have been added to the treatment of pemphigus to attain a steroid-sparing effect. In the last two decades, a variety of agents have been used including gold, dapsone, azathioprine, methotrexate, and cyclophosphamide.[2]

The mean age of onset of lesions is 50-60 years of age; however, the range is broad and the disease may start in the elderly and in children.[34] Although limited data are available regarding the incidence of pemphigus, in general, it ranges from 0.76 to 5 new cases/million/year.[4]

In a recent study by Ingen-Housz-Oro et al., immunobullous disorders have been reported in elderly patients in the age group ranging from 70-96 years with a high mortality rate within 12 months of onset.[5] However, no case with this late onset has been reported in the Indian literature. We report this case for its very late onset at the age of 94 years.