Primary Localized Cutaneous Lichen Amyloidosus of the vulva

A woman, age 72, gravida 5, para 5, was hospitalized for a vulvar biopsy. Her past history revealed a generally healthy woman, menopausal from the age of 53, who had a vaginal hysterectomy and repair at the age of 68 because of genital prolapse. One year before her present admission, the woman started complaining of pruritus vulvae, which progressively worsened. On admission, general physical examination revealed no outstanding pathologic findings; gynecologic examination revealed advanced atrophy of the external genitalia and the vaginal wall. The clitoris and the small labia were atrophic, scarred, and only slightly elevated off the surface (Fig. 1). The vulvar skin was shiny with scattered white patches that were not elevated and had no shiny borders. The rectal examination was normal, and inguinal lymph nodes were not palpable. Laboratory tests revealed the following: sedimentation rate 15/26, hemoglobin 15 gidl, WBC 6400/mm 3 (with normal differential count), thrombocytes 150,000/mm‐ 3 , serum alkaline phosphatase 100 mu/ml (normal 30–85), uric acid 7 mg/dl (upper normal value 6.4). Serum electrolytes, blood urea nitrogen, glucose, cholesterol, creatinine, total proteins, albumin, glutamic ox‐aloacetic transaminase, lactic dehydrogenase, creatine phosphokinase, diastase, urine analysis, chest x‐ray, and electrocardiogram were normal. Vulvar biopsy was per‐formed under general anesthesia. Histologic examination of vulvar skin was as follows: the epidermis was hyperkeratotic with granulosis and acanthosis (Fig. 2). There were enlarged blood vessels in the dermis with thickened and hyalinized walls and foci of infiltration of round cells. The papillaris and reticularis revealed amorphic areas that stained positively with Congo red (Fig. 3). The histologic diagnosis was amy‐loidosis of the vulvar skin. After the histologic report was received, further investiga‐tions were done, as follows: urine Bence Jones protein was negative, serum protein electrophoresis and immunoelec‐trophoresis were normal, and no free light chains were found in the plasma. Serum antinuclear factor and tubercu‐lin skin test were negative. The patient refused to undergo rectal biopsy. She was initially treated with local application of testosterone ointment. After 2 months of treatment, the itching worsened and we started with applications of iso‐conazole nitrate and diflucortolone valerate. This treatment was followed by prompt relief of symptoms, although the gross appearance of the lesions had not changed. One year later, the patient feels well and refuses repeat biopsy.