Acitretin therapy for vulvar lichen sclerosus complicated by
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Lichen sclerosus (LS) is a chronic, idiopathic inflammatory disorder with predilection for the vulva, perineum, and perianal skin that is characterized by ivory-white scattered to confluent atrophic plaques.

A 73 year-old female presented with longstanding pruritus, weeping and irritation of the vulva, perineum, and perianal area since the 1980s. Diagnosis and treatment were delayed until 2002, when she was clinically diagnosed with LS and started on topical clobetasol dipropionate. Her course was complicated by development of vSCC. She was initially treated with partial vulvectomy. Later on she developed hyperkeratotic plaques on the vulva and perianal skin.

During the course of these treatments, the patient managed her Vulvar lichen sclerosus (vLS) with intermittent and inconsistent use of various class I-II topical corticosteroids including clobetasol dipropionate, betamethasone dipropionate, and halobetasol propionate. A biopsy demonstrated dVIN with microinvasive foci, and patient underwent total vulvectomy and further underwent CO2 laser ablation. A left anterior & posterior vulvectomy and fulguration was performed due to the presence of 1 cm raised plaque at the left anterior vulva and adjacent hypertrophic tissue.

A total laparoscopic hysterectomy with bilateral salpingo-oophorectomy and radical vulvectomy with internal reconstruction, including laparoscopic ileostomy, distal urethrectomy, cystostomy with suprapubic catheter placement, and distal vaginectomy was performed. These procedures were performed due to post-menopausal bleeding with reconstruction due to extensive scarring.

On presentation to connective tissue disease the patient noted persistent severe pruritus and irritation of the vulva despite twice daily application of both halobetasol propionate 0.05% ointment and tacrolimus 0.1% ointment. Examination revealed extensive postoperative and reconstructive changes to the vulva, with obliteration of the labia minora and clitoris, near-complete loss of the labia majora and narrowing of the vaginal introitus to less than 2 cm. Thickened, hyperkeratotic white plaques were noted along the medial aspects of the labia majora at the introitus, surrounded by background atrophic erythema and ivory white dyspigmentation.

The patient was started on acitretin 25 mg daily and her topical regimen was narrowed to halobetasol propionate 0.05% ointment. Within two months, the patient noted subjective improvement in pruritus. 5 months after initiation of acitretin, she noted alleviation of pruritus and irritation, resolution of hyperkeratotic plaques and marked improvement of atrophic plaques and erythema. Due to arthralgias and diffuse non scarring alopecia attributed to acitretin, her dose was decreased to 10 mg daily and she has remained well-controlled to date.