nicolau balus syndrome with microcystic adnexal carcinoma
TRANSCRIPT
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.
CommerP6990Nodular 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.
CommerJ 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.
CommerP6534Patch 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.
CommerAPRIL 2013