poroma of the forehead and face: not so uncommon presentations of a rare lesion

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P6602 Poroma of the forehead and face: Not so uncommon presentations of a rare lesion Natalie Davies, MD, East Carolina University, Greenville, NC, United States; Charles Phillips, MD, East Carolina University, Greenville, NC, United States; Daniel Friedmann, MD, East Carolina University, Greenville, NC, United States; Randall Proctor, MBA, East Carolina Univeristy, Greenville, NC, United States; Robert Schosser, MD, East Carolina University, Greenville, NC, United States; Shae Anderson, East Carolina University, Greenville, NC, United States; William Burke, MD, East Carolina University, Greenville, NC, United States Poromas are relatively uncommon, benign sweat gland tumors that account for 10% of adnexal neoplasms, but only 0.1% of all primary cutaneous lesions. These lesions are often solitary, sharply demarcated, sessile or pedunculated papules, but may also present as exophytic nodules or verrucous plaques. This diverse clinical presenta- tion, with the ability to mimic malignant cutaneous neoplasms, makes the clinical diagnosis of a poroma challenging and usually requires histopathologic confirma- tion. Poromas were originally thought to be exclusively of (intraepidermal) eccrine sweat gland origin, with a corresponding propensity for hairless, acral skin, primarily the soles and lateral aspects of feet. Yet recent studies have proposed that poromas may be derived from either eccrine or apocrine origin, supporting the prevalence of nonacral poromas. Poromas may thereby occur in any sweat glandebearing location and have been reported on the chest, back, abdomen, buttocks, pubic region, and extremities, as well as the face, eyelids, scalp, and neck. We report 2 patients with nonacral poromas. The first case, a 17-year-old African American female, presented with a sessile, friable, well-defined nodule of her left forehead, measuring 1.6 cm in diameter, that had been growing slowly in size for approximately 7 months’ time. The lesion occasionally bled easily, but was otherwise asymptomatic. The lesion was clinically consistent with a pyogenic granuloma. The second case, a 69-year-old white man with a history of 2 melanomas, presented with a solitary, pedunculated, pink, telangiectatic papule of his right lateral malar cheek, measuring 5 mm in diameter. The asymptomatic lesion had been growing slowly in size for several months and had recently become more pedun- culated in quality. Although it was clinically consistent with a benign, fibroepithelial papilloma, the lesion was biopsied with shave technique given its recent growth and the patient’s history of melanomas. The belief that poromas are predominantly found on volar acral surfaces persists despite sufficient contrary evidence in the literature. Commercial support: None identified. P6073 Postmenopausal craniofacial hyperhidrosis Layla Hanna-Bashara, MBChB, Royal Liverpool and Broadgreen University Teaching Hospitals NHS Trust, Liverpool, United Kingdom; Niall Wilson, MBChB, Royal Liverpool and Broadgreen University Teaching Hospitals NHS Trust, Liverpool, United Kingdom This case report aims to raise awareness of a new subgroup of primary focal craniofacial hyperhidrosis in postmenopausal women. We report a case of post- menopausal craniofacial hyperhidrosis in a 49-year-old woman. This patient complained of scalp and facial hyperhidrosis since the age of 40. Her hyperhidrosis was initially diagnosed as a menopausal symptom, however hyperhidrosis persisted even with HRT treatment and after her menopause which occurred at age 44. Her symptoms were provoked by heat and were socially disabling. Extensive endocrine investigations revealed no underlying cause. Her past medical history included hypothyroidism (diagnosed at age 37) and bipolar affective disorder. At time of presentation, she was taking multiple medications, some of which are known causes of hyperhidrosis. However, all medications were commenced long before her onset of hyperhidrosis. There was no family history of hyperhidrosis. We treated this patient with oral glycopyrrolate, starting at 1 mg bd and titrated up to 4 mg bd. She continues to take 4 mg bd with partial effectiveness and no side effects. In addition, we treated her with 100 units of intradermal botulinum toxin type A (BTX-A) injections to the hairline and forehead. This has effectively controlled her hyper- hidrosis and she is now symptom free. Postmenopausal craniofacial hyperhidrosis is a subgroup of primary focal hyperhidrosis (PFH). The age of onset in this subgroup is later in life than for classic PFH. It occurs in postmenopausal females, starting at the menopause and persisting. HRT has no effect on symptoms. A similar presentation has not been identified in males of the same age group. It is triggered by intense emotion, stress, and heat. The etiology and pathogenic mechanism are uncertain. The most effective treatments we have used thus far include oral glycopyrrolate and intradermal BTX-A injections. As neural activity of eccrine sweat secretion is regulated by acetylcholine, BTX-A works by competitively binding to presynaptic acetylcholine neuromuscular junction receptors. Oral glycopyrrolate is an anticho- linergic and also competitively binds postsynaptic acetylcholine receptors near eccrine sweat glands. Postmenopausal craniofacial hyperhidrosis appears to be a poorly recognized form of hyperhidrosis with major impact on social, professional, and daily activities. Treatment is challenging, but both oral glycopyrrolate and BTX-A are potentially useful therapeutic options. Commercial support: None identified. P6317 Pregnancy-associated hyperkeratosis of the nipple: A study of 25 cases H. William Higgins II, MD, Brown University, Providence, RI, United States; George Kroumpouzos, MD, PhD, Brown University, Providence, RI, United States; Jennifer Jenkins, MD, MPH, Miraca Life Sciences/Tufts Medical Center, Newton, MA, United States; Thomas Horn, MD, MBA, Massachusetts General Hospital/Harvard Medical School, Boston, MA, United States Background: Reported physiologic nipple changes in pregnancy do not include hyperkeratosis, and are expected to resolve or improve postpartum. Hyperkeratosis of the nipple and/or areola can develop in the context of inflammatory diseases, such as atopic dermatitis, in acanthosis nigricans, as an extension of epidermal nevus, following estrogen treatment, and/or in nevoid hyperkeratosis of the nipple and areola. Objective: We sought to perform a clinicopathologic analysis of cases of pregnancy- associated nipple hyperkeratosis. Methods: Clinical and histopathologic data were obtained from a chart review of cases that were seen over a 5-year period (2007-2012) in a health care system. Clinical documentation included photography of lesions, medical, family and obstetric history, postpartum course, and patient’s rating of response to treatment. Results: Twenty-five cases of pregnancy-associated nipple hyperkeratosis are reported. The lesions were bilateral and involved predominantly the top of the nipple. There was symptomatic aggravation, including pain, tenderness, pruritus, and/or discomfort with breastfeeding, in 17 patients (68%). Nine patients (36%) experienced symptomatic aggravation only during breastfeeding or pregnancy. The lesions persisted postpartum in 22 patients (88%). Histopathologic features were conspicuous orthokeratotic hyperkeratosis, with papillomatosis and acanthosis being mild or absent. Treatment was challenging, because emollients and topical medications provided only mild to moderate response. Conclusions: Pregnancy-associated hyperkeratosis of the nipple can be sympto- matic and persist postpartum. It may represent a physiologic change of pregnancy. The characteristic clinicopathologic features of this disorder allow differentiation from nevoid hyperkeratosis of the nipple and areola. We suggest that this distinctive clinicopathologic entity be called pregnancy-associated hyperkeratosis of the nipple. Commercial support: None identified. P6687 Prevalence and incidence of hidradenitis suppurativa in the United States: Evidence from large administrative claims databases Murali Sundaram, Global Health Economics and Outcomes Research, Abbott Laboratories, Abbott Park, IL, United States; Amy Styles, Analysis Group, Inc, Boston, MA, United States; Annie Gu erin, Analysis Group, Inc, Boston, MA, United States; Cl emence Aberki, Analysis Group, Inc, Boston, MA, United States; Martin Okun, Global Pharmaceutical Research and Development, Abbott Laboratories, Abbott Park, IL, United States; Oscar Hayes, Global Health Economics and Outcomes Research, Abbott Laboratories, Abbott Park, IL, United States; Parvez Mulani, Global Health Economics and Outcomes Research, Abbott Laboratories, Abbott Park, IL, United States Background: Estimated prevalence of hidradenitis suppurativa (HS) has been reported to range from 0.00033% to 4% in various countries, with recent studies presenting self-reported prevalence of approximately 1%. However, source popu- lations and HS patient ascertainment have been inconsistent, and no previous studies estimated prevalence of diagnosed HS using a large administrative database. The objective of this study is to estimate the prevalence and incidence of diagnosed HS in the United States from large administrative claims databases. Methods: Using the Ingenix Employer Solutions database (1999-2009), prevalent HS cases were defined as adults ( $ 18 years of age) who were continuously enrolled in a health care plan during 2009 and who received a diagnosis of HS (ICD-9 code 705.83) before 2010. Incident HS cases were defined as adults continuously enrolled in a health care plan during 2008 and 2009 who received their first diagnosis of HS in 2009. A sensitivity analysis of prevalent and incident diagnosed HS cases was performed using the Thomson Reuters MarketScan database (2002-2009). Results: Estimated prevalence of diagnosed HS in this administrative claims database was 0.23%. A sensitivity analysis with a shorter time period of data resulted in a similar prevalence estimate of 0.17%. Of the 7162 prevalent HS cases, 4849 were women and 2313 were men, resulting in a sex ratio of 2.1:1. Estimated incidence of diagnosed HS was 0.039% and the sensitivity analysis resulted in a similar finding of 0.062%. Conclusion: The prevalence of diagnosed HS cases estimated from large adminis- trative claims databases are much smaller than prevalence estimates reported in the literature based on self-diagnosis. If the true prevalence of HS is 1%, our findings would indicate that only 23% of all HS patients are diagnosed, suggesting that the majority of HS cases remain undiagnosed. Underdiagnosis may result from patients not presenting to physicians, or it may be a matter of misdiagnosis. It is also likely that the 0.19% estimated prevalence captures more severe cases and that some less severe cases remain undiagnosed. These findings suggest a need for increased education for both patients and physicians on the disease and on recommendations for HS diagnosis. This study was funded by Abbott Laboratories. AB58 JAM ACAD DERMATOL APRIL 2013

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P6602Poroma of the forehead and face: Not so uncommon presentations of arare lesion

Natalie Davies, MD, East Carolina University, Greenville, NC, United States;Charles Phillips, MD, East Carolina University, Greenville, NC, United States;Daniel Friedmann, MD, East Carolina University, Greenville, NC, United States;Randall Proctor, MBA, East Carolina Univeristy, Greenville, NC, United States;Robert Schosser, MD, East Carolina University, Greenville, NC, United States;Shae Anderson, East Carolina University, Greenville, NC, United States; WilliamBurke, MD, East Carolina University, Greenville, NC, United States

Poromas are relatively uncommon, benign sweat gland tumors that account for 10%of adnexal neoplasms, but only 0.1% of all primary cutaneous lesions. These lesionsare often solitary, sharply demarcated, sessile or pedunculated papules, but may alsopresent as exophytic nodules or verrucous plaques. This diverse clinical presenta-tion, with the ability to mimic malignant cutaneous neoplasms, makes the clinicaldiagnosis of a poroma challenging and usually requires histopathologic confirma-tion. Poromas were originally thought to be exclusively of (intraepidermal) eccrinesweat gland origin, with a corresponding propensity for hairless, acral skin,primarily the soles and lateral aspects of feet. Yet recent studies have proposedthat poromas may be derived from either eccrine or apocrine origin, supporting theprevalence of nonacral poromas. Poromas may thereby occur in any sweatglandebearing location and have been reported on the chest, back, abdomen,buttocks, pubic region, and extremities, as well as the face, eyelids, scalp, and neck.We report 2 patients with nonacral poromas. The first case, a 17-year-old AfricanAmerican female, presented with a sessile, friable, well-defined nodule of her leftforehead, measuring 1.6 cm in diameter, that had been growing slowly in size forapproximately 7 months’ time. The lesion occasionally bled easily, but wasotherwise asymptomatic. The lesion was clinically consistent with a pyogenicgranuloma. The second case, a 69-year-old white manwith a history of 2melanomas,presented with a solitary, pedunculated, pink, telangiectatic papule of his rightlateral malar cheek, measuring 5mm in diameter. The asymptomatic lesion had beengrowing slowly in size for several months and had recently become more pedun-culated in quality. Although it was clinically consistent with a benign, fibroepithelialpapilloma, the lesionwas biopsied with shave technique given its recent growth andthe patient’s history of melanomas. The belief that poromas are predominantlyfound on volar acral surfaces persists despite sufficient contrary evidence in theliterature.

AB58

cial support: None identified.

Commer

P6073Postmenopausal craniofacial hyperhidrosis

Layla Hanna-Bashara, MBChB, Royal Liverpool and Broadgreen UniversityTeaching Hospitals NHS Trust, Liverpool, United Kingdom; Niall Wilson,MBChB, Royal Liverpool and Broadgreen University Teaching Hospitals NHSTrust, Liverpool, United Kingdom

This case report aims to raise awareness of a new subgroup of primary focalcraniofacial hyperhidrosis in postmenopausal women. We report a case of post-menopausal craniofacial hyperhidrosis in a 49-year-old woman. This patientcomplained of scalp and facial hyperhidrosis since the age of 40. Her hyperhidrosiswas initially diagnosed as a menopausal symptom, however hyperhidrosis persistedeven with HRT treatment and after her menopause which occurred at age 44. Hersymptoms were provoked by heat and were socially disabling. Extensive endocrineinvestigations revealed no underlying cause. Her past medical history includedhypothyroidism (diagnosed at age 37) and bipolar affective disorder. At time ofpresentation, shewas takingmultiple medications, some ofwhich are known causesof hyperhidrosis. However, all medications were commenced long before her onsetof hyperhidrosis. There was no family history of hyperhidrosis. We treated thispatient with oral glycopyrrolate, starting at 1 mg bd and titrated up to 4 mg bd. Shecontinues to take 4 mg bd with partial effectiveness and no side effects. In addition,we treated her with 100 units of intradermal botulinum toxin type A (BTX-A)injections to the hairline and forehead. This has effectively controlled her hyper-hidrosis and she is now symptom free. Postmenopausal craniofacial hyperhidrosis isa subgroup of primary focal hyperhidrosis (PFH). The age of onset in this subgroup islater in life than for classic PFH. It occurs in postmenopausal females, starting at themenopause and persisting. HRT has no effect on symptoms. A similar presentationhas not been identified in males of the same age group. It is triggered by intenseemotion, stress, and heat. The etiology and pathogenic mechanism are uncertain.The most effective treatments we have used thus far include oral glycopyrrolate andintradermal BTX-A injections. As neural activity of eccrine sweat secretion isregulated by acetylcholine, BTX-A works by competitively binding to presynapticacetylcholine neuromuscular junction receptors. Oral glycopyrrolate is an anticho-linergic and also competitively binds postsynaptic acetylcholine receptors neareccrine sweat glands. Postmenopausal craniofacial hyperhidrosis appears to be apoorly recognized form of hyperhidrosis with major impact on social, professional,and daily activities. Treatment is challenging, but both oral glycopyrrolate and BTX-Aare potentially useful therapeutic options.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6317Pregnancy-associated hyperkeratosis of the nipple: A study of 25 cases

H. William Higgins II, MD, Brown University, Providence, RI, United States;George Kroumpouzos, MD, PhD, Brown University, Providence, RI, UnitedStates; Jennifer Jenkins, MD, MPH, Miraca Life Sciences/Tufts Medical Center,Newton, MA, United States; Thomas Horn, MD, MBA, Massachusetts GeneralHospital/Harvard Medical School, Boston, MA, United States

Background: Reported physiologic nipple changes in pregnancy do not includehyperkeratosis, and are expected to resolve or improve postpartum. Hyperkeratosisof the nipple and/or areola can develop in the context of inflammatory diseases,such as atopic dermatitis, in acanthosis nigricans, as an extension of epidermalnevus, following estrogen treatment, and/or in nevoid hyperkeratosis of the nippleand areola.

Objective: We sought to perform a clinicopathologic analysis of cases of pregnancy-associated nipple hyperkeratosis.

Methods: Clinical and histopathologic data were obtained from a chart review ofcases that were seen over a 5-year period (2007-2012) in a health care system.Clinical documentation included photography of lesions, medical, family andobstetric history, postpartum course, and patient’s rating of response to treatment.

Results: Twenty-five cases of pregnancy-associated nipple hyperkeratosis arereported. The lesions were bilateral and involved predominantly the top of thenipple. There was symptomatic aggravation, including pain, tenderness, pruritus,and/or discomfort with breastfeeding, in 17 patients (68%). Nine patients (36%)experienced symptomatic aggravation only during breastfeeding or pregnancy. Thelesions persisted postpartum in 22 patients (88%). Histopathologic features wereconspicuous orthokeratotic hyperkeratosis, with papillomatosis and acanthosisbeing mild or absent. Treatment was challenging, because emollients and topicalmedications provided only mild to moderate response.

Conclusions: Pregnancy-associated hyperkeratosis of the nipple can be sympto-matic and persist postpartum. It may represent a physiologic change of pregnancy.The characteristic clinicopathologic features of this disorder allow differentiationfrom nevoid hyperkeratosis of the nipple and areola. We suggest that this distinctiveclinicopathologic entity be called pregnancy-associated hyperkeratosis of thenipple.

cial support: None identified.

Commer

P6687Prevalence and incidence of hidradenitis suppurativa in the United States:Evidence from large administrative claims databases

Murali Sundaram, Global Health Economics and Outcomes Research, AbbottLaboratories, Abbott Park, IL, United States; Amy Styles, Analysis Group, Inc,Boston, MA, United States; Annie Gu�erin, Analysis Group, Inc, Boston, MA,United States; Cl�emence Aberki, Analysis Group, Inc, Boston, MA, United States;Martin Okun, Global Pharmaceutical Research and Development, AbbottLaboratories, Abbott Park, IL, United States; Oscar Hayes, Global HealthEconomics and Outcomes Research, Abbott Laboratories, Abbott Park, IL,United States; Parvez Mulani, Global Health Economics and OutcomesResearch, Abbott Laboratories, Abbott Park, IL, United States

Background: Estimated prevalence of hidradenitis suppurativa (HS) has beenreported to range from 0.00033% to 4% in various countries, with recent studiespresenting self-reported prevalence of approximately 1%. However, source popu-lations and HS patient ascertainment have been inconsistent, and no previousstudies estimated prevalence of diagnosed HS using a large administrative database.The objective of this study is to estimate the prevalence and incidence of diagnosedHS in the United States from large administrative claims databases.

Methods: Using the Ingenix Employer Solutions database (1999-2009), prevalent HScaseswere defined as adults ($ 18 years of age)whowere continuously enrolled in ahealth care plan during 2009 and who received a diagnosis of HS (ICD-9 code705.83) before 2010. Incident HS cases were defined as adults continuously enrolledin a health care plan during 2008 and 2009who received their first diagnosis of HS in2009. A sensitivity analysis of prevalent and incident diagnosed HS cases wasperformed using the Thomson Reuters MarketScan database (2002-2009).

Results: Estimated prevalence of diagnosed HS in this administrative claims databasewas 0.23%. A sensitivity analysis with a shorter time period of data resulted in asimilar prevalence estimate of 0.17%. Of the 7162 prevalent HS cases, 4849 werewomen and 2313 were men, resulting in a sex ratio of 2.1:1. Estimated incidence ofdiagnosed HS was 0.039% and the sensitivity analysis resulted in a similar finding of0.062%.

Conclusion: The prevalence of diagnosed HS cases estimated from large adminis-trative claims databases are much smaller than prevalence estimates reported in theliterature based on self-diagnosis. If the true prevalence of HS is 1%, our findingswould indicate that only 23% of all HS patients are diagnosed, suggesting that themajority of HS cases remain undiagnosed. Underdiagnosis may result from patientsnot presenting to physicians, or it may be a matter of misdiagnosis. It is also likelythat the 0.19% estimated prevalence captures more severe cases and that some lesssevere cases remain undiagnosed. These findings suggest a need for increasededucation for both patients and physicians on the disease and on recommendationsfor HS diagnosis.

y was funded by Abbott Laboratories.

This stud

APRIL 2013