Disseminated extragenital bullous lichen sclerosus
A 61-year-old Caucasian female was seen with a several year history of large pruritic plaques on the trunk and proximal extremities that proved to be lichen sclerosus et atrophicus on biopsy. Over the preceding months many of the truncal lesions developed painful bullae. Multiple therapies including ultra-potent topical steroids, topical vitamin D derivative (Dovonex), narrowband ultraviolet (UV) B, and hydroxychloroquine had failed to alleviate her symptoms or halt the progression of her dermatitis in the past.\nPhysical examination revealed several hypopigmented atrophic plaques, ranging in size from 5 cm to 10 cm, consistent with LS lesions that were widely distributed over the trunk, arms, forearms, and medial thighs. Hemorrhagic bullae were present within pre-existing atrophic lesions involving her abdomen, left flank, bilateral medial arms and antecubital fossae []. No oral or genital lesions were noted. The remainder of the physical examination was unremarkable.\nBiopsies of the bullous lesions on the left flank were obtained for routine histological examination and immunofluorescence staining. Hematoxylin and eosin staining demonstrated features consistent with bullous LS including thinning of the epidermis with compact hyperkeratosis, degeneration of the basal layer, edema with the early stages of a subepidermal blister, and a band of homogenized collagen in the dermis []. Direct immunofluorescence staining was negative.\nThe patient was treated with topical calcitriol 3 mcg/g ointment and topical triamcinolone 0.1% cream daily in combination with oral acitretin 25 mg daily and responded with dramatic improvement of her lesions and level of pain. Future therapy considerations include high intensity ultraviolet A1 and immunosuppressive therapy with prednisone or methotrexate.