Infantile hemangiomas treated with propranolol: Description of thePhoenix Children’s Hospital experience

(Poster reference number 5079)Larisa Speetzen, MD, Mayo Clinic, Scottsdale, AZ, United States; Harper Price,MD, Phoenix Children’s Hospital, Phoenix, AZ, United States; Judy O’Haver, PhD,CNP, Phoenix Children’s Hospital, Phoenix, AZ, United States; Kellie Reinesch,RN, Phoenix Children’s Hospital, Phoenix, AZ, United States; Nirav Patel,Phoenix Children’s Hospital, Phoenix, AZ, United States

Background: Since the case study describing the use of propranolol to arrest thegrowth of hemangiomas, pediatric dermatologists have used this medication as asteroid-sparing agent and monotherapy for the treatment of infantile hemangiomas(IH). However, an established protocol for administration and monitoring of thismedication has yet to be agreed upon.

Purpose: To describe the treatment of IHswith propranolol including complicationsand efficacy in one pediatric dermatology clinic.

Methods: A retrospective chart review was conducted on all children who attendedclinic from July 1, 2008 to April 1, 2011 and met the following criteria: (1) clinicallydiagnosed IH(s); (2) propranolol administered for treatment of the hemangioma(s).

Treatment regimen: Demographic data, a detailed health history and physicalexamination were performed. Depending on the clinical circumstance, patientswere treated with propranolol, oral prednisolone, intralesional triamcinolone,and/or pulsed dye laser. Patients treated with propranolol were followed accordingto clinic protocol. Hemangiomas were evaluated by the provider and parentalimpressions were noted. Adverse events were also documented.

Results: Preliminary analysis of this data identified 176 patients who met theinclusion criteria. 47 infants were treated concurrently with both oral prednisoneand propranolol. Propranolol was initiated during an inpatient hospitalization in 7infants. One child was hospitalized for an RSV infection that occurred during thetreatment course with propranolol. There were no other hospitalizations forcomplications of propranolol. As instructed, parents held doses of propranolol in32 patients at one or more times in the treatment course for upper respiratorysymptoms, fever, or other illness. Seven patients had propranolol discontinued dueto adverse events; 5 for persistent respiratory symptoms, 1 for questioned drug rash,and 1 due to parental concern for fatigue. The hemangiomas were improved in 160of the 176 patients, follow up was insufficient to determine improvement in 11patients, no improvement was seen in 5 patients.

Conclusion: Propranolol is an efficacious and safe treatment for infants withhemangiomas. Our large series highlights the feasibility of administering propran-olol in the outpatient setting with careful clinical follow-up, blood pressure andheart rate monitoring aswell as specific screening and education regarding potentialside effects.

AB164

cial support: None identified.

Commer

Juvenile amyopathic dermatomyositis

(Poster reference number 5406)Josep Pujol-Montcusi, MD, Dermatology, Hospital Univeristari Joan XXIII,Tarragona, Spain; Antoni Ravent�os-Estell�e, MD, Pathology, Hospital UniveristariJoan XXIII, Tarragona, Spain; Laia Pastor-Jan�e, MD, Dermatology, HospitalUniveristari Joan XXIII, Tarragona, Spain; Maria Sim�o-Esqu�e, MD, Dermatology,Hospital Univeristari Joan XXIII, Tarragona, Spain; Maria Luisa Diaz-Fernandez,MD, Pathology, Hospital Univeristari Joan XXIII, Tarragona, Spain; Pilar Tur�egano-Fuentes, MD, Dermatology, Hospital Univeristari Joan XXIII, Tarragona, Spain

Case report: An 11-year old girl with no relevant medical history consulted forcutaneous lesions that corresponded to Gottron papules over the extensor aspectsof her hands and elbows, and mild erythematous and desquamative plaques on hernose and ears. Laboratory tests including CK, aldolase, and LDH were normal, ANAwere positive (1/160), and ENA, anti-Jo, anti-PM1were negative. The histopathologyof a papule showed lymphocytic perivascular dermatitis. Direct immunofluores-cence was negative. Electromyography, electrocardiogram, chest X-ray, and longbones X-ray were normal. Cutaneous lesions improved substantially but slowly withtopical steroids and tacrolimus 0.1% ointment. Dermatology and pediatric follow-upduring 2 years didn’t show any relevant muscular or systemic complication.

Discussion: Amyopathic JDM is a very uncommon variant of JDM, characterized bytypical cutaneous features of dermatomyositis, in the absence of clinical orlaboratory signs of muscle disease for more than 6 months. Gerami et al reviewedof 68 cases of clinically amyopathic juvenile dermatomyositis. They include twosubphenotypes: amyopatic and hypomyopathic DMJ. In this review, ANA wasspecifically mentioned in 19 cases and they were positive in 53%. The vast majorityof patients with juvenile-onset clinically amyopathic. DMJ had a good outcome withvery few complicationswhich are typically associated with juvenile or adult classicaldermatomyositis. Overall, 74% of the 68 patients presented isolated cutaneousdermatomyositis for at least 6 months and did not develop any clinical evidence ofmuscle disease over many years of follow-up. We present a case of an 11-year-old girldiagnosed of amyophatic JDM. According to previous reports, our case presented anexcellent evolution without any signs of muscular or systemic complication after 2years of follow-up. Children with clinically amyopathic JDM usually have goodprognosis and, therefore, symptomatic treatment of cutaneous involvement andclose clinical monitoring may be an alternative to aggressive immunosuppression.

cial support: None identified.

Commer

J AM ACAD DERMATOL

KippeleTrenaunay syndrome: Presentation of typical and exuberantclinical case

(Poster reference number 4968)Carla Di Gi�acomo, MD, Policlinica Geral do Rio de Janeiro, Rio de Janeiro, Brazil;Ana Maria Mosca, MD, Policlinica Geral do Rio de Janeiro, Rio de Janeiro, Brazil;Flavia Schueler, MD, Policlinica Geral do Rio de Janeiro, Rio de Janeiro, Brazil;Leticia Rautha, MD, Policlinica Geral do Rio de Janeiro, Rio de Janeiro, Brazil;Luciana Klein, MD, Policlinica Geral do Rio de Janeiro, Rio de Janeiro, Brazil

Background: KippeleTrenaunay syndrome is characterized by the triad of port-winestain, varicose veins with or without venous malformations, bone and soft tissuehypertrophy. It occurs in only one extremity causing hypertrophy. No gender orethnicity predilection, and appears at birth or during childhood, and it’s morecommon to appear around 10 years of age. The etiopathogenesis of this syndrome isnot well established, with different theories. Presentation purposes: Rare syndrome,typical and exuberant clinical case.

Presentation: Four-years-old child, presenting wine-stain in right hand since birth,showing on first finger and palm hand area. The injury turned swollen and varicosecompromising the movements. There is hypertrophy on the affected area.Hemangiomatous aspect injury, similar to port-wine stain. X-ray showing soft tissuehypertrophy, characterizing the syndrome of KippeleTrenaunay.

Argument: KippeleTrenaunay Syndrome was first described in 1900 as a triad thatconsist in cutaneous hemangioma, bone and soft tissue hypertrophy and venousdilatation. Affects only one body segment. Some authors believe that all the venouschanges are consequences from profound venous obstruction or profound veinsatresia, causing edema and hypertrophy in the affected site. Most of the patientshave all three syndrome signs, and hemangioma is the first sign to appear. Thehemangioma can be limited in an area or can expand to deeper layers of skin,muscles, bones and internal organs, aggravating the prognosis. The varicose can bewatched during first years of childhood. The main differential diagnosis is theKippeleTrenaunay -Weber Syndrome, where there is an arteriovenous malforma-tion. There are no healing treatments, but only to improve patient’s life quality andreduce injuries consequences. For port-wine stain, the treatment is pulsed dye laserwith better results in recent stains. In this case shown, the expectant treatment wasadopted with regular monitoring. An elastic sleeve was indicated for this patient tocontain the varicosity and to improve the local blood circulation, decreasing theedema of the end affected, with good results.

cial support: None identified.

Commer

Loose anagen hair syndrome in two patients with epidermolysis simplex,DowlingeMeara type

(Poster reference number 5352)Jennifer Crombie, University of Masschusetts Medical School, Worcester, MA,United States; Julie Fenner, MD, University of Massachusetts Medical School,UMass Memorial Healthcare, Worcester, MA, United States; Karen Wiss, MD,University of Massachusetts Medical School, UMass Memorial Healthcare,Worcester, MA, United States; Sheila Greenlaw, MD, University ofMassachusetts Medical School, UMass Memorial Healthcare, Worcester, MA,United States

We describe two patients with known epidermolysis bullosa simplex,DowlingeMeara type (EBS-DM), who were also found to have loose anagen hairsyndrome (LAHS). The parents of a 7-year-old female diagnosed with EBS-DM at birthobserved that her hair grew slowly and that she infrequently required haircuts.Forceful hair extraction performed on the left parietal scalp caused minimaldiscomfort. Microscopic analysis demonstrated dystrophic anagen hairs withmisshapen hair bulbs and ruffled cuticles characteristic of LAHS. The secondpatient, a 32-month-old female, was also diagnosed with EBS-DM at birth. Herparents noted that her hair fell out easily and that clumps of hair could be pulledpainlessly from her scalp. She had fine, blonde hair and seldom required haircuts.Forceful hair extraction revealed anagen hairs with ruffled cuticles on the hair shaftsjust proximal to the bulb. EBS-DM, a severe form of the inherited blistering disorderEBS marked by widespread herpetiform blistering in infancy, is usually caused bydefects in epidermal keratins 5 or 14. With genotype analysis, these patients had thesame keratin 14 mutation, N123S. The genetic basis for LAHS, characterized byabnormal anagen hairs that are easily and painlessly removed from the scalp, has notbeen fully elucidated but a variety of epithelial keratin abnormalities have beenimplicated. The presence of EBS and LAHS in two patients with the same keratin 14mutation raises the possibility that the common defect contributes to the patho-genesis of both disorders.

cial support: None identified.

Commer

APRIL 2012