Download pdf - Successful treatment of ulcerated infantile hemangioma associated with PELVIS syndrome using oral propanolol

P7089Successful treatment of ulcerated infantile hemangioma associated withPELVIS syndrome using oral propanolol

Shawn Kwatra, MD, Wake Forest University School of Medicine, Winston-Salem,NC, United States; Alexander Powers, MD, Wake Forest University School ofMedicine, Winston-Salem, NC, United States; Anthony Atala, MD, Wake ForestUniversity School of Medicine, Winston-Salem, NC, United States; GilYosipovitch, MD, Wake Forest University School of Medicine, Winston-Salem,NC, United States; Shivani Kaushik, MD, Wake Forest University School ofMedicine, Winston-Salem, NC, United States; Thomas Mclean, MD, Wake ForestUniversity School of Medicne, Winston-Salem, NC, United States

PELVIS syndrome is a rare form of segmental hemangioma associated with genitaland systemic abnormalities. We report the case of a male infant with ulceratedsegmental hemangiomas in the perineal area misdiagnosed at birth as diaper rash.Presence of atypical genital features such as hypospadias and bifid scrotum noted atbirth led to further investigations which revealed the presence of spinal cord andvesicorenal abnormalities. Oral propranolol therapy was started, which graduallydecreased the size and ulceration of the hemangioma. Multistep surgical repair wasperformed for the external genital malformations and tethered spinal cord. Thisreport emphasize the importance of careful examination and correct diagnosis ofsuspicious lesions as these can be associated with other systemic anomalies that maylead to long-term complications. We also confirm the successful use of oralpropranolol in treating ulcerated segmental hemangiomas in PELVIS syndrome.

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P6297Trends in the presence or absence of transaminitis and/or leukopenia inpediatric patients treated with methotrexate for inflammatory skindisease

G. Michael Lewitt, MD, St. Louis University, St. Louis, MO, United States; ElaineSiegfried, MD, Cardinal Glennon Children’s Hospital, Saint Louis, MO, UnitedStates

Methotrexate (MTX) was approved by the US FDA in 1953. It has 12 labeledindications and numerous off-label uses. The medicine is inexpensive, convenient,and versatile; it has been the standard of care for 50 years for psoriasis, rheumatoidarthritis, and cancer despite 12 black box warnings. Safety of low-dose MTX is well-established in children with JIA. There is no standard for assessing laboratoryparameters or making dose adjustments based on laboratory abnormalities.Transient elevations in LFTs and decreases in WBC are common in children. Thisretrospective chart review sought to define the incidence of laboratory abnormal-ities among pediatric dermatology patients taking MTX, including anemia, leuko-penia, and transaminitis. We evaluated the impact of dose adjustments on laboratoryabnormalities. Inclusion criteria: Evaluation in the pediatric dermatology clinic from1/1/05 to 10/31/11; Rx MTX. We collected data including MTX dose, doseadjustments, and laboratory values.

Results: n ¼ 59 subjects: 47% M; 53% F. Ethnicity: 66.1% white, 30.5% AfricanAmerican. Age (yrs): 0-3 e 18.6%; 4-6 e 27.1%; 7-12 e 40.7%; and 13-18 e 13.6%.Dose adjustments: 37 cessations reported amongst 33/59 pts (56%); 73% cessationsreported discontinued for reasons other than medication-related complications.15% discontinued for transaminitis. 29/33 pts had cessations for 1 reason; 4/33 ptshad cessations for 2 reasons. There were 27 dose reductions amongst 26/59 pts(44%). 12% of pts had dose reduction for transaminitis. Themost common reason fordose reduction was improvement; the 2nd most common was for adverse effectsunrelated to lab abnormalities. 71% of pts had a laboratory abnormality. No dosechanges were made for anemia. In 11/59 pts, laboratory abnormalities prompted:MTX discontinuation: 3; MTX dose reduction: 2; both dose reduction + discontin-uation: 6. A difference in the magnitude of the transaminitis in pts plays a major rolein the respective changes in therapy. 46% of pts had elevated transaminases. 16/27(59%) had no change in dose and 81% of these pts normalized on subsequentlaboratory draw versus 11/27 (41%) had dose reduction/discontinuation, and 82% ofthese pts normalized on the subsequent laboratory draw. MTX dose reduction/dis-continuation was for reasons other than laboratory abnormalities in the majority ofcases. The degree of laboratory abnormality varied directly with the likelihood ofdecreasing or discontinuing the drug. For transaminitis, # 2 times the upper limit ofnormal dose reduction or cessation does not impact the change in laboratory valuesback to normal.

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J AM ACAD DERMATOL

P7093Verrucous hemangioma leg: A challenging birthmark

Toby Nelson, MBBS, The James Cook University Hospital, Middlesbrough, UnitedKingdom; Sivakumar Natarajan, MD, The James Cook University Hospital,Middlesbrough, United Kingdom

Verrucous hemangioma (VH) is a congenital vascular malformation within thedermis and subcutaneous tissue associated with reactive acanthosis, papillomatosis,and hyperkeratosis. Presence from birth with a predilection for lower limbs iscommon. The lesion(s) grow with the patient, developing a warty surface later inlife. Careful clinical examination and imaging are necessary to delineate the depth ofthe lesion. Surgery remains the mainstay of treatment.We report a case of a 6-month-old boy who was referred with a congenital asymptomatic black-blue birthmark. Onexamination, there were large, linear, bizarre-shaped, purple plaques with areas ofhyperkeratosis on the left medial thigh and shin. A bluish hue was noted at the edge,suggesting deeper dermal involvement. The lesions failed to empty on pressure.Dermatoscopy demonstrated vascular lacunae of varying sizes centrally withuniform pseudopod vascular projections at the periphery. A clinical diagnosis ofVHwas made based on the presence at birth, location and colour. Investigation withultrasound showed the vascular lesion extended into the subcutaneous tissue to adepth of 1 cm, but did not involve underlying muscle. MRI has been deferred untillater life at the family’s request. Challenges posed by VH include recognising this rarevascular malformation and determining the optimum time for surgical intervention.In this case, the size and extent of the lesions pose further challenges. The specificityof the novel dermatoscopic findings of vascular lacunae with uniform pseudopodvascular projections need to be verifid in other cases.

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PHARMACOLOGY

P6227Association of 5-a reductase inhibitors and sexual dysfunction: A RADARreport

Victoria Godinez-Puig, MD, Northwestern University Feinberg School ofMedicine, Chicago, IL, United States; Beatrice Nardone, MD, NorthwesternUniversity Feinberg School of Medicine, Chicago, IL, United States; LaurenBecker, MD, Northwestern University Feinberg School of Medicine, Chicago, IL,United States; Maria Colavincenzo, MD, Northwestern University FeinbergSchool of Medicine, Chicago, IL, United States; Steven Belknap, MD,Northwestern University Feinberg School of Medicine, Chicago, IL, UnitedStates; Vishvas Garg, College of Pharmacy, University of New Mexico,Albuquerque, NM, United States

Background: Finasteride (F) and dutasteride (D) are 5-a reductase inhibitors (5-aRIs)that reduce systemic and local dihydrotestosterone and are associated with sexualadverse experiences (SAEs) in men, including decreased libido, ejaculation disor-ders, and erectile dysfunction. F 1 mg is labeled for treatment of male pattern hairloss; both F 5 mg and D 0.5 mg are labeled for treatment of benign prostatichyperplasia. SAEs have been reported to persist beyond discontinuation of F. Here,we assess and further characterize 5-aRI associated SAEs in the database of the FDAAdverse Event Reporting System (AERS).

Methods: We calculated proportional reporting ratios (PRRs) and empiric Bayesgeometric means (EBGM) for 5-aRI-associated SAEs in the FDA AERS database.Search terms included but were not limited to decreased libido, ejaculationdisorders, and erectile dysfunction.

Results: Through December 2011, 431 reports of F 1 mg-associated SAE, 1089reports of F 5 mg-associated SAE and 387 reports of D-associated SAE wereidentified. The mean age for SAE after F 1 mg, F 5 mg, and D exposure was 36.56 11.6, 64.46 11.7, and 66.56 10.0 years, respectively. For F 1 mg-associated SAE,PRR¼ 32.09 (95% CI 29.20-35.25; P¼.001) and EBGM¼ 31.21 (95% CI 27.96-34.45;P¼.001). For F 5mg-associated SAE, PRR¼ 21.88 (95% CI 20.64-23.19; P¼.001) andEBGM ¼ 20.71 (95% CI 19.49-21.96; P ¼ .001). For D-associated SAE, PRR ¼ 17.77(95% CI 16.09-19.61; P ¼ .001) and EBGM ¼ 17.04 (95% CI 15.23-18.73; P ¼ .001).Decreased semen volume (F 1 mg ¼ 6.7%, F 5 mg ¼ 1.1%, and D ¼ 13.2%) andtesticular pain (F 1 mg ¼ 9.5%, F 5 mg ¼ 1.0%, and D ¼ 1.0%) were also reported.Furthermore, depression (F 1 mg¼ 17.2%, F 5 mg¼ 3.0%, and D¼ 2.6%), anxiety (F1 mg¼ 14.9%, F 5 mg¼ 2.4%, and D¼ 1.3%) and suicidal ideation (F 1 mg¼ 3.3%, F5 mg ¼ 1.0%, and D ¼ 1.0%) were also reported.

Conclusion: We identified a highly significant association between 5-aRI exposureand SAE in the FDA AERS database. The strong stafety signal for F 1mg is of specialconcern, given the particularly young mean age of these patients. The strong safetysignals for all three 5-aRI products and reports of persistance beyond discontinu-ation of F 5 mg and F 1 mg require further investigation.

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APRIL 2013