Indian Journal of Dermatology
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Year : 2014  |  Volume : 59  |  Issue : 1  |  Page : 105
Pemphigus vulgaris in an elderly patient

Department of Dermatology, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India

Date of Web Publication23-Dec-2013

Correspondence Address:
Milind A Patvekar
Department of Dermatology, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.123537

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How to cite this article:
Patvekar MA, Sadana D. Pemphigus vulgaris in an elderly patient. Indian J Dermatol 2014;59:105

How to cite this URL:
Patvekar MA, Sadana D. Pemphigus vulgaris in an elderly patient. Indian J Dermatol [serial online] 2014 [cited 2020 Nov 24];59:105. Available from: https://www.e-ijd.org/text.asp?2014/59/1/105/123537


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

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Figure 2: Photomicrograph (H and E, ×400) showing supra-basal cleft with tomb-stone appearance of basal cells

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Figure 3: Fish-net appearance seen in direct immunofluorescence

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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. [3],[4] 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.

   References Top

1.Kanwar AJ, De D. Pemphigus in India. Indian J Dermatol Venereol Leprol 2011;77:439-49.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Kar PK, Murthy PS, Rajagopal R. Management of pemphigus vulgaris during acute phase. Indian J Dermatol Venereol Leprol 2003;69:109-13.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, et al., editors. Fitzpatrick's Dermatology in General Medicine. Vol. 1., 7 th ed. New York: Mc Graw Hill Publishers; 2008. p. 459-68.  Back to cited text no. 3
4.Amagai MP. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. Vol. 1., 2 nd ed. Mosby Elsevier Publishers; 2008. p. 419-28.  Back to cited text no. 4
5.Ingen-Housz-Oro S, Alexandre M, Le Roux-Villet C, Picard-Dahan C, Tancrède-Bohin E, Wallet-Faber N, et al. Pemphigus in elderly adults: Clinical presentation, treatment, and prognosis. J Am Geriatr Soc 2012;60:1185-7.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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