|Year : 1995 | Volume
| Issue : 3 | Page : 157-158
Association of alopecia areata, vitiligo and pemphigus vulgaris
Source of Support: None, Conflict of Interest: None
A 50-year-old female had vitiligo and alopecia universalis. She developed pemphigus vulgaris with a history of recent increase in the size of the vitiligo patch. Corticosteroid therapy controlled the pemphigus vulgaris, halted the extension of the vitiligo lesion and promoted regrowth of hair. The coexistence of these three diseases in the same patient lends credence to the contention that autoimmunity plays a role in the pathogenesis of these diseases.
Keywords: Vitiligo, Alopecia areata, Pemphigus vulgaris
|How to cite this article:|
. Association of alopecia areata, vitiligo and pemphigus vulgaris. Indian J Dermatol Venereol Leprol 1995;61:157-8
| Introduction|| |
The hypothesis that immune derangements are possible causes of various diseases is largely based on the clinical observation of the co-existence of various supposedly 'autoimmune' diseases with in vitro and in vivo experimental data showing autoantibodies. Alopecia areata (AA), vitiligo and pemphigus vulgaris (PV) are diseases in whose aetiopathogenesis immunological alterations are thought to play a role.
In the present communication the unusual co-existence of AA, vitiligo and PV is reported.
| Case Report|| |
A 50-year-old female presented with the history of painful erosions in the mouth, and crusted lesions over the chin, neck and the periumbilical area since four months. At the time of admission she also had, flaccid vesiculobullous lesions over the chest and lumbar area. She had a history of vitiliginous lesions over the lower part of abdomen since the age of 25 years, which had increased in size recently. Twelve years back, after the sudden and untimely demise of her husband, within a span of 6 weeks circumscribed alopecic patches appeared on the scalp and gradually extended in area resulting in the total loss of the terminal hair of the scalp, eyebrows, axillae and pubic region.
Examination on admission revealed alopecia universalis with a depigmented patch over the right iliac region. Clear flaccid bullae intermingled with erosive and crusted lesions over the face, neck, chest and lumbar area were noted. Painful erosions were present in the oral cavity. Nikolsky's sign was positive. A Tzanck smear showed acantholytic cells. Routine haematological parameters and urine and stool examinations did not reveal any abnormalities except for a raised ESR. Blood VDRL, LE cell test, RA factor and HIV antibody test were negative. Skin biopsy from an early vesicle revealed a suprabasal cavity filled with acantholytic cells. An H & E stained section of the skin from the vitiliginous lesion revealed an inflammatory infiltrate comprising of lymphocytes and the absence of melanin pigment. A biopsied specimen from the scalp revealed peribulbar lymphocytic infiltration. A diagnosis of PV, AA and vitiligo was made and steroid therapy was started. Steroid therapy, not only helped in controlling PV but also halted the growth of vitiligo patch and resulted in cosmetically acceptable regrowth of hair over the scalp within 2 months.
| Discussion|| |
Various theories are suggested for the aetiology of AA and vitiligo. The association of AA with seemingly unrelated autoimmune disorders has led to an intense search for immune derangements. In vitiligo, an autoimmune hypothesis is suggested by its clinical association with a number of disorders considered to be autoimmune. Vitiligo seems to occur about four times as often in patients with AA as in the general population. On the other hand pemphigus is considered to be a prototype for antibody-mediated autoimmune disorders in humans, with multiple reports of the co-existence of pemphigus with autoimmune diseases as well as other disorders.,
Mehlman and Greismer noted that hair loss in AA often occurs approximately two weeks after an event of major emotional significance, as in the present case. The peribulbar lymphocytic infiltrate characteristically seen in biopsy specimens of a lesion of AA has been cited as de facto evidence of an immune aetiology. A localized lymphocytic infiltrate at the progressing edge of a lesion of vitiligo also lends support to the autoimmune pathogenesis. The response to oral steroids in the present case is also an evidence to show that immune derangement has a role to paly in causation of the above disease.
The co-existence of AA, vitiligo and PV in the present case lends credence to the contention that autoimmunity may play a role in the pathogenesis of each of these diseases.
| References|| |
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|2.||Muller S, Winkelaman R. Alopecia areata. Arch Dermatol 1963;88:290. |
|3.||Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin 1993;3:439-40. |
|4.||Koranne RV, Sehgal VN, Sachdev KG. Association of pemphigus vulgaris with vitiligo. Ind J Dermatol Venereol Leprol 1986;52:107-8. |
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