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Dermatofibrosarcoma protuberans masquerading as a keloid: A diagnostic challenge
SURGERY—DERMATOLOGIC 1630 A case series on the use of topical imiquimod 5% for severe and recurrent keloid scarring Zainab Laftah, MBChB, King’s College Hospital, London, United Kingdom; Zahir Mirza, MBChB, Royal Free Hospital, London, United Kingdom; Nick Baksh, King’s College Hospital, London, United Kingdom; Atheer Ujam, MBBS, King’s College Hospital, London, United Kingdom; Kathleen Fan, MBBS, PhD, King’s College Hospital, London, United Kingdom; Saqib Bashir, MBChB, MD, King’s College Hospital, London, United Kingdom Introduction: Keloids are benign fibroproliferative tumors caused by excessive collagen deposition that can result in cosmetic deformity and distress. Despite trials of numerous treatments, no single effective regime has been identified. Imiquimod, a topical Toll-like receptor agonist, may be used on surgically excised keloid scars to reduce recurrence. It is proposed to act by enhancing cytokine production, altering gene expression, reducing collagen and glycosaminoglycan production. Aim: To determine if postoperative topical imiquimod 5% reduces keloid recurrence. Methods: We analyzed outcomes of patients with severe recurrent keloid scars treated with surgery and imiquimod via retrospective case note review. Results: In total, 19 keloids (median size 55 mm; range 10-90) were excised from 16 patients with type 6 skin of median age 25 years (range 15-53). All had intraoperative triamcinolone injection into the wound edge followed by application of imiquimod. Keloids were on various anatomic sites, with 58% located on the ear. Patients were followed up for a median of 18 months (range 9-38). Imiquimod was applied to the wound edge three times a week for a median of 2 months (range 1-6). Side effects included pain (37%), hypopigmentation (26%), erosion (21%) and bleeding (5%), which resulted in treatment cessation in 37%. 12 keloids (10 patients) had 1-2 months of imiquimod. Compared to the original, recurrence was larger in 1, half the size in 6, mild in 3, and 2 had no recurrence. Median follow-up was 17 months. 7 keloids (6 patients) had 5-6 months of imiquimod. Compared to the original, recurrence was half the size in 2, mild in 4, and 1 had no recurrence. Median followup was 20 months. A longer duration of imiquimod use resulted in reduced recurrence size even after lengthy follow-up. Our data suggest recurrence is unlikely if there has been none by 1 year. However, mild recurrence by 1 year was associated with recurrence of half the original size by 2 years. Recurrence did not depend on the site or size of the original keloid. On a satisfaction scale 65% were either ‘satisfied’ or ‘very satisfied.’
1438 Cutaneous RosaieDorfman disease treated by surgical excision and a review of the literature Anika Khan, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Esra Musbahi, MBBS, Basildon and Thurrock University Hospitals NHS Foundation Trust, Basildon, United Kingdom; Ravi Suchak, MBChB, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Rohaj K. Mehta, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Seyed Mohsen Khorshid, MD, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Peter Ozua, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom A 71-year-old female presented to the dermatology clinic with a firm, pearly, pink papule on the left cheek present for a few months, which occasionally ‘tingled.’ The clinical appearance was suggestive of a nodular basal cell carcinoma (BCC). Excision with flap reconstruction was performed. However, histology revealed cutaneous RosaieDorfman disease (RDD) as demonstrated by a dermal nodule with dense lymphoplasmacytic infiltrate with aggregates of larger cells with abundant pale staining cytoplasm and ‘emperipolesis,’ which stained positively for S100. The latter is a characteristic finding of RosaieDorfman disease whereby intact cells are phagocytosed by larger cells. Review at two and a half months postoperatively showed no recurrence. She denied being unwell. There had been no lymphadenopathy or suggestion of systemic involvement. Rosai and Dorfman described cases of sinus histiocytosis with massive lymphadenopathy in 1969 and 19721, which led to the recognition of this eponymous disease. This is a rare, benign and self-limiting condition of unknown etiology. Extranodal involvement, particularly of the skin, is seen fairly frequently in classical cases. However, isolated cutaneous lesions without lymphadenopathy are rarer still (although increasingly reported). Lesions may present as papules, nodules, and plaques, which can appear at any site, but occur particularly over the face, typically in older Caucasian women. Patients may have lesions for many months or years. Numerous treatments have been tried for purely cutaneous RDD with variable success including surgery, radiotherapy, cryotherapy, CO2 laser, steroids (topical, intralesional and oral), dapsone, acitretin, imatinib and thalidomide. There are no known definite long-term associations with systemic disease, although associated laboratory abnormalities have been found in a small number of cases such as blood cytopaenias and increased IgG. Here we present a case of a rare lesion of isolated cutaneous RosaieDorfman disease clinically resembling a BCC with a review of the literature. Commercial support: None identified.
Conclusion: Our series has a longer duration of imiquimod therapy and follow-up than of any other published so far. Topical imiquimod appears to be an effective adjuvant treatment for reducing keloid scarring with longer duration of therapy reducing size of recurrence. Furthermore, recurrence within a year requires close monitoring and early retreatment. Commercial support: None identified.
1582 A systematic review of the uses and contraindications of epinephrine in local anesthesia in the dermatologic setting Syril Keena Que, MD, University of Connecticut Department of Dermatology, Farmington, CT, United States; James Whalen, MD, University of Connecticut Department of Dermatology, Farmington, CT, United States Background: Epinephrine is a hormone and neurotransmitter that nonselectively binds to the alpha and beta adrenergic receptors. In the context of dermatologic surgery, epinephrine can be added to a local anesthetic to decrease the amount of bleeding and prolong the duration of local anesthesia by vasoconstricting blood vessels. To our knowledge, there is no comprehensive review on the uses and relative contraindications of epinephrine in dermatologic surgery. Methods: A PubMed search of the National Library of Medicine database was performed using the following search terms: epinephrine, lidocaine, dermatologic surgery, complications, pregnancy, digital anesthesia, grafts, and flaps. We analyzed papers from dermatologic, dental, and plastic surgery literature. Results: Our study reviewed 14 papers detailing the use of epinephrine in digital or penile anesthesia, 20 papers relating to epinephrine in flaps and grafts, 7 papers about medical conditions in which epinephrine is contraindicated, 2 papers about epinephrine in the pregnant patient, and 2 papers about patients with catecholamine hypersensitivity. Contrary to conventional practice, recent studies show that the use of 0.5% lidocaine with 1:200,000 epinephrine is safe for digital or penile anesthesia and unlikely to cause necrosis. One exception is in patients with vasospastic or peripheral vascular disease. Epinephrine is also safe to use for flaps and grafts but has been reported to occasionally cause flap necrosis if too high a volume of local anesthesia is injected. Generally, the doses of epinephrine used in dermatologic surgery are safe in pregnant patients, but higher doses can cause vasoconstriction of the uterine artery and decrease placental perfusion. Caution should be taken when using epinephrine in patients with severe cardiac disease, hyperthyroidism, or pheochromocytoma. Patients with autonomic neuropathy or severe spinal cord injury may require lower doses because of catecholamine hypersensitivity, a phenomenon known as denervation hypersensitivity. The following medications may also increase sensitivity to epinephrine by interacting with or potentiating the effects of epinephrine: nonselective beta blockers, MAO inhibitors, tricyclic antidepressants, and phenothiazines. Discussion: In the scenarios mentioned above, epinephrine at a lower concentration (1:400,000 to 1:1,000,000) can still achieve effective vasoconstriction and may be safer for use in dermatologic surgery. Commercial support: None identified.
MAY 2015
1799 Dermatofibrosarcoma protuberans masquerading as a keloid: A diagnostic challenge Allyson Black, MD, University of Oklahoma, Oklahoma City, OK, United States; Victoria Antonio, University of Oklahoma, Oklahoma City, OK, United States; Travis Blalock, MD, University of Oklahoma, Oklahoma, Oklahoma City, OK, United States; Jason Stratton, MD, University of Oklahoma, Oklahoma City, OK, United States; Geeta Patel, DO, University of Oklahoma, Oklahoma City, OK, United States Dermatofibrosarcoma protuberans is an uncommon locally aggressive soft tissue sarcoma with clinical features that can resemble benign entities including keloids and dermatofibromas. We present a case of a 58-year-old African American male with a 5-year history of a slowly growing lesion on his right shoulder originally diagnosed as a keloid and treated with intralesional triamcinolone. Due to limited response from treatment and clinical concern for a soft tissue tumor, a biopsy was obtained which revealed dermatofibrosarcoma protuberans. This case highlights the importance of histologic evaluation when clinically benign entities do not respond to common therapeutic modalities. Consideration of dermatofibrosarcoma protuberans when evaluating patients with nonresponding keloids may expedite accurate diagnosis and therapy of what may be an underlying malignant neoplasm. Commercial support: None identified.
J AM ACAD DERMATOL
AB259
1438 Cutaneous RosaieDorfman disease treated by surgical excision and a review of the literature Anika Khan, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Esra Musbahi, MBBS, Basildon and Thurrock University Hospitals NHS Foundation Trust, Basildon, United Kingdom; Ravi Suchak, MBChB, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Rohaj K. Mehta, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Seyed Mohsen Khorshid, MD, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom; Peter Ozua, MBBS, Basildon and Thurrock University Hospitals NHS Trust, Basildon, United Kingdom A 71-year-old female presented to the dermatology clinic with a firm, pearly, pink papule on the left cheek present for a few months, which occasionally ‘tingled.’ The clinical appearance was suggestive of a nodular basal cell carcinoma (BCC). Excision with flap reconstruction was performed. However, histology revealed cutaneous RosaieDorfman disease (RDD) as demonstrated by a dermal nodule with dense lymphoplasmacytic infiltrate with aggregates of larger cells with abundant pale staining cytoplasm and ‘emperipolesis,’ which stained positively for S100. The latter is a characteristic finding of RosaieDorfman disease whereby intact cells are phagocytosed by larger cells. Review at two and a half months postoperatively showed no recurrence. She denied being unwell. There had been no lymphadenopathy or suggestion of systemic involvement. Rosai and Dorfman described cases of sinus histiocytosis with massive lymphadenopathy in 1969 and 19721, which led to the recognition of this eponymous disease. This is a rare, benign and self-limiting condition of unknown etiology. Extranodal involvement, particularly of the skin, is seen fairly frequently in classical cases. However, isolated cutaneous lesions without lymphadenopathy are rarer still (although increasingly reported). Lesions may present as papules, nodules, and plaques, which can appear at any site, but occur particularly over the face, typically in older Caucasian women. Patients may have lesions for many months or years. Numerous treatments have been tried for purely cutaneous RDD with variable success including surgery, radiotherapy, cryotherapy, CO2 laser, steroids (topical, intralesional and oral), dapsone, acitretin, imatinib and thalidomide. There are no known definite long-term associations with systemic disease, although associated laboratory abnormalities have been found in a small number of cases such as blood cytopaenias and increased IgG. Here we present a case of a rare lesion of isolated cutaneous RosaieDorfman disease clinically resembling a BCC with a review of the literature. Commercial support: None identified.
Conclusion: Our series has a longer duration of imiquimod therapy and follow-up than of any other published so far. Topical imiquimod appears to be an effective adjuvant treatment for reducing keloid scarring with longer duration of therapy reducing size of recurrence. Furthermore, recurrence within a year requires close monitoring and early retreatment. Commercial support: None identified.
1582 A systematic review of the uses and contraindications of epinephrine in local anesthesia in the dermatologic setting Syril Keena Que, MD, University of Connecticut Department of Dermatology, Farmington, CT, United States; James Whalen, MD, University of Connecticut Department of Dermatology, Farmington, CT, United States Background: Epinephrine is a hormone and neurotransmitter that nonselectively binds to the alpha and beta adrenergic receptors. In the context of dermatologic surgery, epinephrine can be added to a local anesthetic to decrease the amount of bleeding and prolong the duration of local anesthesia by vasoconstricting blood vessels. To our knowledge, there is no comprehensive review on the uses and relative contraindications of epinephrine in dermatologic surgery. Methods: A PubMed search of the National Library of Medicine database was performed using the following search terms: epinephrine, lidocaine, dermatologic surgery, complications, pregnancy, digital anesthesia, grafts, and flaps. We analyzed papers from dermatologic, dental, and plastic surgery literature. Results: Our study reviewed 14 papers detailing the use of epinephrine in digital or penile anesthesia, 20 papers relating to epinephrine in flaps and grafts, 7 papers about medical conditions in which epinephrine is contraindicated, 2 papers about epinephrine in the pregnant patient, and 2 papers about patients with catecholamine hypersensitivity. Contrary to conventional practice, recent studies show that the use of 0.5% lidocaine with 1:200,000 epinephrine is safe for digital or penile anesthesia and unlikely to cause necrosis. One exception is in patients with vasospastic or peripheral vascular disease. Epinephrine is also safe to use for flaps and grafts but has been reported to occasionally cause flap necrosis if too high a volume of local anesthesia is injected. Generally, the doses of epinephrine used in dermatologic surgery are safe in pregnant patients, but higher doses can cause vasoconstriction of the uterine artery and decrease placental perfusion. Caution should be taken when using epinephrine in patients with severe cardiac disease, hyperthyroidism, or pheochromocytoma. Patients with autonomic neuropathy or severe spinal cord injury may require lower doses because of catecholamine hypersensitivity, a phenomenon known as denervation hypersensitivity. The following medications may also increase sensitivity to epinephrine by interacting with or potentiating the effects of epinephrine: nonselective beta blockers, MAO inhibitors, tricyclic antidepressants, and phenothiazines. Discussion: In the scenarios mentioned above, epinephrine at a lower concentration (1:400,000 to 1:1,000,000) can still achieve effective vasoconstriction and may be safer for use in dermatologic surgery. Commercial support: None identified.
MAY 2015
1799 Dermatofibrosarcoma protuberans masquerading as a keloid: A diagnostic challenge Allyson Black, MD, University of Oklahoma, Oklahoma City, OK, United States; Victoria Antonio, University of Oklahoma, Oklahoma City, OK, United States; Travis Blalock, MD, University of Oklahoma, Oklahoma, Oklahoma City, OK, United States; Jason Stratton, MD, University of Oklahoma, Oklahoma City, OK, United States; Geeta Patel, DO, University of Oklahoma, Oklahoma City, OK, United States Dermatofibrosarcoma protuberans is an uncommon locally aggressive soft tissue sarcoma with clinical features that can resemble benign entities including keloids and dermatofibromas. We present a case of a 58-year-old African American male with a 5-year history of a slowly growing lesion on his right shoulder originally diagnosed as a keloid and treated with intralesional triamcinolone. Due to limited response from treatment and clinical concern for a soft tissue tumor, a biopsy was obtained which revealed dermatofibrosarcoma protuberans. This case highlights the importance of histologic evaluation when clinically benign entities do not respond to common therapeutic modalities. Consideration of dermatofibrosarcoma protuberans when evaluating patients with nonresponding keloids may expedite accurate diagnosis and therapy of what may be an underlying malignant neoplasm. Commercial support: None identified.
J AM ACAD DERMATOL
AB259