Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.

IMPORTANCE: Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. OBJECTIVE: To develop recommendations for the care of adults with EMPD. EVIDENCE REVIEW: A systematic review of the literature on EMPD from January 1990 to September 18, 2019, was conducted using MEDLINE, Embase, Web of Science Core Collection, and Cochrane Libraries. Analysis included 483 studies. A multidisciplinary expert panel evaluation of the findings led to the development of clinical care recommendations for EMPD. FINDINGS: The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years. CONCLUSIONS AND RELEVANCE: Clinical practice guidelines for EMPD provide guidance regarding recommended diagnostic approaches, differentiation between invasive and noninvasive disease, and use of surgical vs nonsurgical treatments. Prospective registries may further improve our understanding of the natural history of the disease in primary vs secondary EMPD, clarify features of high-risk tumors, and identify superior management approaches.

Adalimumab for treatment of severe ulcerative sarcoidosis.

Ulcerative sarcoidosis is a rare variant of cutaneous sarcoidosis that may present as ulceration with necrotic yellow plaques on the lower extremities, face, arms, trunk, or genital area. Adalimumab, a human monoclonal anti-TNF antibody, is an emerging treatment for recalcitrant cutaneous sarcoidosis. We describe severe ulcerative sarcoidosis in a 60-year-old woman with chronic ulcerative necrobiosis lipoidica-like plaques on her left arm for over 20 years. Her condition had not responded to previous treatments with hydroxychloroquine, methotrexate, and sulfasalazine. After a four-month course of adalimumab therapy in addition to pentoxifylline and prednisone with taper, the patient had significant improvement in her skin disease.

High-Potency Topical Steroid Treatment of Multiple Keratoacanthomas Associated With Prurigo Nodularis.

Patients who present with multiple keratoacanthomas (KAs) associated with prurigo nodularis often pose a treatment challenge. These lesions often require aggressive treatment, such as Mohs micrographic surgery, surgical excision, electrodesiccation and curettage, intralesional steroid injection, and long-term acitretin. 5-Fluorouracil (5-FU) cream 5% has been shown to be effective; however, topical options are limited when 5-FU fails. We have found success using a high-potency topical steroid under occlusion, resulting in resolution of KAs and prurigo nodules.

Use of an adherent fenestrated drape to anchor an adjacent vertical sterile surgical towel.

Vertical surgical sites or those on reclining patients often present a challenge when establishing and securing a sterile field. The drape or towel most proximal to the physician is often vertically oriented. The forces of gravity and movements of surgery can shift or detach this vertical drape. Sterile clamps are not always available or are needed for securing other instruments. We present a method to secure this vulnerable drape using a central fenestrated adherent drape.