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P6702 Nicolau Balus syndrome with microcystic adnexal carcinoma Aruni Ranasinghe, MBBS, MD, Department of Dermatology, Cambridge University Hospitals NHS Trust, UK, Cambridge, United Kingdom; Ed Rytina, MD, PhD, Department of Dermatopathology, Cambridge University Hospitals NHS Trust, UK, Cambridge, United Kingdom; Jonathan Batchelor, MD, Department of Dermatology, Nottingham University Hospital, UK, Nottingham, United Kingdom; Thomas Ha, MD, Department of Dermatology, Cambridge University Hospitals NHS Trust, UK, Cambridge, United Kingdom A 13-year-old female presented with a 5-year history of skin changes on her skin and neck. Her mother gave a similar history. On examination, she had multiple erythematous hyperkeratotic granular areas on her cheek and neck consistent with atrophorderma vermiculata. She also had numerous papules on her neck and chest. A skin biopsy of these papules showed multiple cysts in the superficial dermis containing eosinophilic hyaline material lined by cuboidal cells, consistent with clear cell syringoma. Similar lesions in the patient’s mother were also proven to be syringomata on biopsy. A diagnosis of Nicolau Balus syndrome (NBS) was made and camouflage treatment given. Many years later, our patient developed apparent abscesses in the right cheek and a rapidly growing firm lesion in her left temple. A biopsy from the right cheek showed a deeply infiltrative tumor involving the papillary dermis and subcutis with prominent desmoplasia and strands of bland epithelial cells with ductal differentiation, consistent with a microcystic adnexal carcinoma (MAC). The left temple lesion was also a MAC which was excised by Mohs micrographic surgery. Her right cheek tumor was incompletely excised in 2008 with involved deep margins. The patient declined further surgery as complete excision would have caused significant tissue loss. She opted for close observation instead. NBS is a rare syndrome consisting of a triad of generalised eruptive syringomata, atrophoderma vermiculata and milia. MAC is a rare cutaneous neoplasm that usually presents as a slowly growing subcutaneous nodule in the head and neck area. Clinical and histopathologic misdiagnosis are common. MAC has an infiltrative growth pattern, often with perineural invasion. Although rare, metastases have been reported. Treatment with Mohs micrographic surgery is more likely to prevent recurrence than wide excision. To our knowledge, this is the first case of MAC that has been reported in a patient with NBS. We are also aware of only one other case of multiple MACs occurring in the same patient. Because both NBS and MAC are rare entities, it seems highly unlikely that they are unrelated in our patient. Although syringomas such as those present in NBS, have been reported to undergo malignant change, there is no documented association between syringomas and MAC. Interestingly our patient has remained free of local or distant spread of the MAC on her cheek for the past 4 years. This case highlights that conservative management is an option for MAC, although close follow up is recommended. Commercial support: None identified. P6990 Nodular amyloidosis: Clinical recognition of an unusual entity Landon Stigall, MD, Geisinger Health System, Danville, PA, United States; David Troutman, DPM, Geisinger Health System, Danville, PA, United States; Michele Maroon, MD, Geisinger Health System, Danville, PA, United States Background: Nodular amyloidosis, an abnormal protein deposition disorder, is in the spectrum of cutaneous disorders known as primary cutaneous amyloidoses, which also includes lichen and macular amyloid. Amyloidoses are classified as disorders of aberrant protein deposition, with formation of an abnormal tertiary b pleated sheet conformation in vivo, as opposed to the a helical form in normal physiologic tissues. Nodular amyloidosis is especially relevant to the clinician given its association with underlying systemic disease. Case report: Sixty-threee and 67-year-old white males, respectively, presented to the dermatology clinic at Geisinger Health System after observing red to violaceous papules and plaques on the plantar surface of their feet of several months duration. Neither patient related any symptoms to the aforementioned plaques, nor had any prior treatment been attempted. Biopsy of representative areas from both patients revealed deposition of amorphous thioflavin T positive material in the dermis, consistent with a diagnosis of nodular amyloidosis. Referral and work-up by hematology was negative for systemic disease at the time of this report. Discussion: All primary cutaneous amyloidoses are composed of a conserved amyloid P component and amyloid ground substance, and a unique protein amyloid fibril by which the subtype of amyloid can be delineated. The fibrillar component of nodular amyloidosis differs from the other cutaneous amyloidoses in that it is derived from amyloid light chains, often of the l subtype, from a suspected localized plasma cell dyscrasia. Establishing a diagnosis of purely cutaneous nodular amyloi- dosis is important for several reasons. Recent literature suggests that approximately 7% of individuals with nodular amyloidosis will progress to systemic amyloidosis, and up to 40% of patients with primary systemic amyloidosis will have cutaneous findings which are clinically and histologically indistinguishable from primary cutaneous amyloidosis. Nodular amyloidosis has also been seen in association with Sjogren syndrome, with a reported incidence as high as 25%. Conclusion: Nodular amyloidosis is an important entity to recognize in the clinical setting as it can be a harbinger of serious underlying systemic disease. Timely diagnosis of this entity enables differentiation from systemic disease, and if found, prompt institution of appropriate treatment. Commercial support: None identified. P6134 Paraneoplastic cutaneous vasculitis in multiple myeloma Julie Nguyen, MD, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States; Madison Russell, MS, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States; Pamela Allen, MD, University of Oklahoma Department of Dermatology, Oklahoma City, OK, United States A 64-year-old Hispanic male presented with a 5-year history of painful erythematous to purpuric papules and plaques. Physical examination revealed extensive palpable purpura over his trunk and extremities, with sparing of the head and neck. He was first seen at the onset of his skin lesions 5 years ago in our clinic, where a skin biopsy was done showing leukocytoclastic vasculitis (LCV). Review of systems was noncontributory. Extensive vasculitis workup at the time revealed no etiology: CBC with differential, renal and liver function tests, coagulation tests, serum electrolytes, calcium, phosphorus, complement levels, and chest radiograph were normal. ANA, ANCA, antiphospholipid antibodies, cryoglobulins, RF, HIV, hepatitis panel, and SPEP/UPEP were all negative. He was initially started on prednisone and colchicine for idiopathic cutaneous small vessel vasculitis (CSVV), but shortly after treatment initiation was suddenly lost to follow-up for several years until 1 year ago when he returned to our clinic for the same persistent skin lesions. Repeat skin biopsy again showed LCV. SPEP was repeated and this time revealed an ‘‘M’’ band with elevated IgA at 1186 mg/dL. Immunofixation showed IgA monoclonal protein with kappa light chain specificity. IgM and IgG levels were normal. Bone marrow biopsy revealed a plasma cell infiltrate of 11.3% with monoclonal gammopathy, IgA Kappa, consistent with multiple myeloma. He was diagnosed with smoldering multiple myeloma, as he was asymptomatic (no lytic bone lesions, normal calcium and creatinine levels). He is currently being followed by hematology/oncology to monitor disease progression and treatment. LCV has multiple possible etiologies and is rarely associated with multiple myeloma, as in our patient who was finally diagnosed 4 years after his skin manifestations. We report this interesting case to emphasize that onset of cutaneous vasculitis can precede years before any evidence of multiple myeloma and the importance of long-term follow-up in these patients. Simple laboratory tests, such as SPEP and serum proteins, are valuable in evaluating patients with chronic and persistent LCV or recalcitrant LCV unresponsive to conventional therapy. Commercial support: None identified. P6534 Patch test with dental screening series in oral disease Tae-Wook Kim, MD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Byung-Soo Kim, MD, PhD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Hoon-Soo Kim, MD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Hyun-Chang Ko, MD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Margaret Song, MD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Moon-Bum Kim, MD, PhD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea; Won-Jeong Kim, MD, Department of Dermatology, School of Medicine, Pusan National University, Busan, South Korea Background: The oral mucosa, including the lips, is constantly exposed to a large number of potentially irritating and allergenic substances including dental materials, but the role of contact allergy in oral disease is obscure. Objective: The aims of this study are to analyze positive patch test reaction with dental screening series in oral diseases and to evaluate clinical relevance of oral diseases with contact allergy to dental materials. Methods: Thirty-nine patients with oral disease were taken patch test with dental screening series at our hospital from February 2004 to August 2010. We reviewed symptom and sites of oral diseases, history of dental procedure and result of patch test. Results: Oral diseases included in this study were oral lichen planus (n ¼ 21; 53.8%), cheilitis (n ¼ 11; 28.2%), burning mouth syndrome (n ¼ 3; 7.7%), and others (n ¼ 4; 10.3%, recurrent aphthosis, glossodynia, stomatitis and perioral dermatitis). Twenty- nine of 39 patients (74.4%) had positive reaction to $ 1 allergen, and the number of positive patch test reactions was highest for gold sodium thiosulfate (25.6%) and nickel sulfate (25.6%), followed by potassium dichromate (23.1%), cobalt (15.4%), palladium (7.7%), mercury (5.1%), and copper (5.1%). Especially 6 of 21 patients with oral lichen planus had a symptom in adjacent area to dental materials and positive patch test reactions to dental materials containing the suspected allergen. Conclusion: We suggest that patch test with dental screening series is worth considering in oral diseases, especially given that it has clinical significance in oral lichen planus. Commercial support: None identified. AB56 JAM ACAD DERMATOL APRIL 2013

Nicolau Balus syndrome with microcystic adnexal carcinoma

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Page 1: Nicolau Balus syndrome with microcystic adnexal carcinoma

P6702Nicolau Balus syndrome with microcystic adnexal carcinoma

Aruni Ranasinghe, MBBS, MD, Department of Dermatology, CambridgeUniversity Hospitals NHS Trust, UK, Cambridge, United Kingdom; Ed Rytina,MD, PhD, Department of Dermatopathology, Cambridge University HospitalsNHS Trust, UK, Cambridge, United Kingdom; Jonathan Batchelor, MD,Department of Dermatology, Nottingham University Hospital, UK, Nottingham,United Kingdom; Thomas Ha, MD, Department of Dermatology, CambridgeUniversity Hospitals NHS Trust, UK, Cambridge, United Kingdom

A 13-year-old female presented with a 5-year history of skin changes on her skin andneck. Her mother gave a similar history. On examination, she had multipleerythematous hyperkeratotic granular areas on her cheek and neck consistentwith atrophorderma vermiculata. She also had numerous papules on her neck andchest. A skin biopsy of these papules showedmultiple cysts in the superficial dermiscontaining eosinophilic hyaline material lined by cuboidal cells, consistent withclear cell syringoma. Similar lesions in the patient’s mother were also proven to besyringomata on biopsy. A diagnosis of Nicolau Balus syndrome (NBS) was made andcamouflage treatment given. Many years later, our patient developed apparentabscesses in the right cheek and a rapidly growing firm lesion in her left temple. Abiopsy from the right cheek showed a deeply infiltrative tumor involving thepapillary dermis and subcutis with prominent desmoplasia and strands of blandepithelial cells with ductal differentiation, consistent with a microcystic adnexalcarcinoma (MAC). The left temple lesionwas also aMACwhichwas excised byMohsmicrographic surgery. Her right cheek tumor was incompletely excised in 2008withinvolved deep margins. The patient declined further surgery as complete excisionwould have caused significant tissue loss. She opted for close observation instead.NBS is a rare syndrome consisting of a triad of generalised eruptive syringomata,atrophoderma vermiculata and milia. MAC is a rare cutaneous neoplasm that usuallypresents as a slowly growing subcutaneous nodule in the head and neck area.Clinical and histopathologic misdiagnosis are common. MAC has an infiltrativegrowth pattern, often with perineural invasion. Although rare, metastases have beenreported. Treatment with Mohs micrographic surgery is more likely to preventrecurrence than wide excision. To our knowledge, this is the first case of MAC thathas been reported in a patient with NBS. We are also aware of only one other case ofmultiple MACs occurring in the same patient. Because both NBS and MAC are rareentities, it seems highly unlikely that they are unrelated in our patient. Althoughsyringomas such as those present in NBS, have been reported to undergo malignantchange, there is no documented association between syringomas and MAC.Interestingly our patient has remained free of local or distant spread of the MACon her cheek for the past 4 years. This case highlights that conservativemanagementis an option for MAC, although close follow up is recommended.

AB56

cial support: None identified.

Commer

P6990Nodular amyloidosis: Clinical recognition of an unusual entity

Landon Stigall, MD, Geisinger Health System, Danville, PA, United States; DavidTroutman, DPM, Geisinger Health System, Danville, PA, United States; MicheleMaroon, MD, Geisinger Health System, Danville, PA, United States

Background: Nodular amyloidosis, an abnormal protein deposition disorder, is in thespectrum of cutaneous disorders known as primary cutaneous amyloidoses, whichalso includes lichen and macular amyloid. Amyloidoses are classified as disorders ofaberrant protein deposition, with formation of an abnormal tertiary b pleated sheetconformation in vivo, as opposed to the a helical form in normal physiologic tissues.Nodular amyloidosis is especially relevant to the clinician given its association withunderlying systemic disease.

Case report: Sixty-threee and 67-year-old white males, respectively, presented to thedermatology clinic at Geisinger Health System after observing red to violaceouspapules and plaques on the plantar surface of their feet of several months duration.Neither patient related any symptoms to the aforementioned plaques, nor had anyprior treatment been attempted. Biopsy of representative areas from both patientsrevealed deposition of amorphous thioflavin T positive material in the dermis,consistent with a diagnosis of nodular amyloidosis. Referral and work-up byhematology was negative for systemic disease at the time of this report.

Discussion: All primary cutaneous amyloidoses are composed of a conservedamyloid P component and amyloid ground substance, and a unique protein amyloidfibril by which the subtype of amyloid can be delineated. The fibrillar component ofnodular amyloidosis differs from the other cutaneous amyloidoses in that it isderived from amyloid light chains, often of the l subtype, from a suspected localizedplasma cell dyscrasia. Establishing a diagnosis of purely cutaneous nodular amyloi-dosis is important for several reasons. Recent literature suggests that approximately7% of individuals with nodular amyloidosis will progress to systemic amyloidosis,and up to 40% of patients with primary systemic amyloidosis will have cutaneousfindings which are clinically and histologically indistinguishable from primarycutaneous amyloidosis. Nodular amyloidosis has also been seen in association withSj€ogren syndrome, with a reported incidence as high as 25%.

Conclusion: Nodular amyloidosis is an important entity to recognize in the clinicalsetting as it can be a harbinger of serious underlying systemic disease. Timelydiagnosis of this entity enables differentiation from systemic disease, and if found,prompt institution of appropriate treatment.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6134Paraneoplastic cutaneous vasculitis in multiple myeloma

Julie Nguyen, MD, University of Oklahoma Department of Dermatology,Oklahoma City, OK, United States; Madison Russell, MS, University ofOklahoma Department of Dermatology, Oklahoma City, OK, United States;Pamela Allen, MD, University of Oklahoma Department of Dermatology,Oklahoma City, OK, United States

A 64-year-old Hispanic male presented with a 5-year history of painful erythematousto purpuric papules and plaques. Physical examination revealed extensive palpablepurpura over his trunk and extremities, with sparing of the head and neck. He wasfirst seen at the onset of his skin lesions 5 years ago in our clinic, where a skin biopsywas done showing leukocytoclastic vasculitis (LCV). Review of systems wasnoncontributory. Extensive vasculitis workup at the time revealed no etiology:CBC with differential, renal and liver function tests, coagulation tests, serumelectrolytes, calcium, phosphorus, complement levels, and chest radiograph werenormal. ANA, ANCA, antiphospholipid antibodies, cryoglobulins, RF, HIV, hepatitispanel, and SPEP/UPEP were all negative. He was initially started on prednisone andcolchicine for idiopathic cutaneous small vessel vasculitis (CSVV), but shortly aftertreatment initiation was suddenly lost to follow-up for several years until 1 year agowhen he returned to our clinic for the same persistent skin lesions. Repeat skinbiopsy again showed LCV. SPEP was repeated and this time revealed an ‘‘M’’ bandwith elevated IgA at 1186 mg/dL. Immunofixation showed IgA monoclonal proteinwith kappa light chain specificity. IgM and IgG levels were normal. Bone marrowbiopsy revealed a plasma cell infiltrate of 11.3% with monoclonal gammopathy, IgAKappa, consistent with multiple myeloma. He was diagnosed with smolderingmultiple myeloma, as he was asymptomatic (no lytic bone lesions, normal calciumand creatinine levels). He is currently being followed by hematology/oncology tomonitor disease progression and treatment. LCV hasmultiple possible etiologies andis rarely associated with multiple myeloma, as in our patient who was finallydiagnosed 4 years after his skin manifestations. We report this interesting case toemphasize that onset of cutaneous vasculitis can precede years before any evidenceof multiple myeloma and the importance of long-term follow-up in these patients.Simple laboratory tests, such as SPEP and serum proteins, are valuable in evaluatingpatients with chronic and persistent LCV or recalcitrant LCV unresponsive toconventional therapy.

cial support: None identified.

Commer

P6534Patch test with dental screening series in oral disease

Tae-Wook Kim, MD, Department of Dermatology, School of Medicine, PusanNational University, Busan, South Korea; Byung-Soo Kim, MD, PhD, Departmentof Dermatology, School of Medicine, Pusan National University, Busan, SouthKorea; Hoon-Soo Kim, MD, Department of Dermatology, School of Medicine,Pusan National University, Busan, South Korea; Hyun-Chang Ko, MD, Departmentof Dermatology, School of Medicine, Pusan National University, Busan, SouthKorea; Margaret Song, MD, Department of Dermatology, School of Medicine,Pusan National University, Busan, South Korea; Moon-Bum Kim, MD, PhD,Department of Dermatology, School of Medicine, Pusan National University,Busan, South Korea; Won-Jeong Kim, MD, Department of Dermatology, School ofMedicine, Pusan National University, Busan, South Korea

Background: The oral mucosa, including the lips, is constantly exposed to a largenumber of potentially irritating and allergenic substances including dental materials,but the role of contact allergy in oral disease is obscure.

Objective: The aims of this study are to analyze positive patch test reaction withdental screening series in oral diseases and to evaluate clinical relevance of oraldiseases with contact allergy to dental materials.

Methods: Thirty-nine patients with oral disease were taken patch test with dentalscreening series at our hospital from February 2004 to August 2010. We reviewedsymptom and sites of oral diseases, history of dental procedure and result of patchtest.

Results: Oral diseases included in this study were oral lichen planus (n¼ 21; 53.8%),cheilitis (n¼ 11; 28.2%), burning mouth syndrome (n¼ 3; 7.7%), and others (n¼ 4;10.3%, recurrent aphthosis, glossodynia, stomatitis and perioral dermatitis). Twenty-nine of 39 patients (74.4%) had positive reaction to $ 1 allergen, and the number ofpositive patch test reactions was highest for gold sodium thiosulfate (25.6%) andnickel sulfate (25.6%), followed by potassium dichromate (23.1%), cobalt (15.4%),palladium (7.7%), mercury (5.1%), and copper (5.1%). Especially 6 of 21 patientswith oral lichen planus had a symptom in adjacent area to dental materials andpositive patch test reactions to dental materials containing the suspected allergen.

Conclusion: We suggest that patch test with dental screening series is worthconsidering in oral diseases, especially given that it has clinical significance in orallichen planus.

cial support: None identified.

Commer

APRIL 2013