Cutaneous lymphangiectasia, also called as acquired lymphangioma, is a benign cutaneous disorder involving the dermal and subcutaneous lymphatic channels. It can rarely occur on the vulva. We describe a 35-year-old woman who came with multiple raised lesions over the vulva and left upper thigh of 1 year duration. She gave history of getting treated for multiple swellings that developed over right side of the neck and inguinal regions on both sides three decades ago. On local genital examination, the patient had a large polypoidal growth involving both sides of the vulva, left upper thigh, and over pubic area. Multiple linear scars were present over the upper thighs and groin bilaterally. The patient underwent simple vulvectomy and left thigh growth excision. Histopathological examination of the vulvectomy specimen confirmed our diagnosis.

Familial benign chronic pemphigus or Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by the development of recurrent blisters and erosions in the intertriginous areas. Various topical and systemic treatment options include corticosteroids, topical 5-fluorouracil, topical vitamin D analogs, topical zinc oxide, dapsone, psoralen plus ultraviolet A, systemic retinoids, cyclosporine, methotrexate, and photodynamic therapy. In recalcitrant cases, further options including, invasive methods such as grenz ray therapy, carbon dioxide laser abrasion, and erbium: YAG laser ablation, dermabrasion, electron beam therapy, botulinum toxin, and full-thickness excision of affected skin with repair by split-thickness grafting have been reported as useful in treatment of HHD. We describe a case of HHD who was treated with several treatment modalities including antibiotics, corticosteroids, and dapsone earlier and when presented to us had a severe recalcitrant disease. Thalidomide, as a modality of treatment has been successfully used in few cases earlier. Our patient responded well to thalidomide.

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QuizHow to cite this article: Saldanha CS, Shenoy MM, Shanthala PR, Amin VB. Multiple vegetating lesions over the genitalia. Indian J Dermatol Venereol Leprol 2015;81:325-6.