P6433Propanolol for a pelvis syndrome-associated hemangioma

Shivani Bashiyan, MBBS, MD, Wake Forest University School of Medicine,Winston-Salem, NC, United States; Gil Yosipovitch, MD, Wake Forest UniversitySchool of Medicine, Winston-Salem, NC, United States; Shawn Kwatra, MD, WakeForest University School of Medicine, Winston-Salem, NC, United States

PELVIS syndrome is a rare form of segmental hemangioma associated with genitaland systemic abnormalities. In 2006 Girard et al proposed the acronym PELVIS to tietogether the syndrome composed of the following abnormalities: perineal heman-gioma, external genital malformations, lipomyelomeningocele, vesicorenal abnor-malities, imperforate anus and skin tag. We report the case of a male infant withulcerated segmental hemangiomas in the perineal area misdiagnosed at birth asdiaper rash. Atypical external genitalia was noticed at birth. Examination revealed abifid scrotum, a midshaft hypospadias with chordee, and leftward deviation of thepenis with abnormal foreskin. No vascular lesions were noted at this time. Thepatient eventually presented at 3 months of age with a large, painful vascular lesionin the perineal area extending anteriorly to the genitals, bifurcating the scrotum andrunning posteriorly to the sacral area and buttocks with ulceration in parts. Thislesion started at birth as a red patch in the sacral area and was misdiagnosed andtreated as a diaper rash. Magnetic resonance imaging of the lumbar spine revealedthe presence of spinal cord and vesicorenal abnormalities. Oral propranolol therapywas started, which gradually decreased the size and ulceration of the hemangioma.Multistep surgical repair was performed for the external genital malformations andtethered spinal cord. This report emphasizes the importance of careful examinationand correct diagnosis of suspicious lesions at birth as these can be associated withother systemic anomalies that may lead to long-term complications. Approximately10% of infantile hemangiomas are located in the perineal area. Hemangiomasoccurring at this site are more prone to ulceration due to irritation from stool, urine,friction and maceration. The first case of perineal hemangioma associated withsystemic and genital anomalies was reported in 1986 by Goldberg et al, and only ahandful of cases have been reported since this time. We also confirm the successfuluse of oral propranolol in treating ulcerated segmental hemangiomas in PELVISsyndrome.

APRIL 20

cial support: None identified.

Commer

P6302Recognizing and managing eczematous id reactions to molluscum con-tagiosum virus in children

Elena Netchiporouk, MD, Elena Netchiporouk, Laval, Quebec, Canada; BernardCohen, MD, Bernard A. Cohen, Baltimore, MD, United States

Molluscum contagiosum (MC) is a self-limiting cutaneous viral eruption that is verycommon in children. MC infection can trigger an eczematous reaction aroundmolluscum papules known as a hypersensitivity or an id reaction. In addition, ahypersensitivity reaction can occasionally occur at sites distant from the primarymolluscum papules. These eczematous reactions are often asymptomatic orminimally pruritic. We believe that id reactions represent an immunologicallymediated host response to MC virus and a harbinger of regression. Therefore, thesereactions often do not require treatment other than emollients. Moreover, topicalsteroids or immunomodulators may suppress this process and potentiate the spreadof the primary MC infection. However, in symptomatic patients, treatment shouldnot be withheld and short-course treatments of topical corticosteroids may be used.In this case series, we describe 3 cases of hypersensitivity reactions in otherwisehealthy children with MC. We hope that our report will make clinicians more awareof this common eczematous response to MC and will improve the management andcounseling of these patients and their parents.

cial support: None identified.

Commer

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P6883Skin physiology and surface isotropy investigations in young children ofdifferent age groups compared to adults

Joachim Fluhr, Charite University Clinic, Berlin, Germany; Caroline Baudouin,Laboratoires Expanscience, Epernon, France; Clarence de Belilovsky,Laboratoires Expanscience, Epernon, France; Jean-Pierre Hachem, VrijeUniversiteit Brussel, Brussel, Belgium; Nadege Lachmann, LaboratoiresExpanscience, Epernon, France; Philippe Msika, Laboratoires Expanscience,Epernon, France; Razvigor Darlenski, Hopital Tokuda, Sofia, Bulgaria

Background: Postnatal life is a time period of active functional maturation andcutaneous adaptation to the dry extrauterine environment. Two studies wereperformed in order to investigate noninvasively physiologic skin parameters,biochemical skin composition, and skin surface maturation for characterizingneonatal skin in comparison to different children age groups and adult’s skin.

Methods: The first study was performed in healthy volunteers (n ¼ 108) of 6 agegroups (n ¼ 18/group: newborns (1-15days), five-week, six-month, 1-2yrs, 4-5yrsand adults 20-35yrs). Biophysical parameters (TEWL, capacitance and surface pH)were measured on the volar forearms. In six subjects of each age group water- andbulk NMF-profiles were assessed with in vivo Raman spectroscopy (RS). D-squameswere collected (n ¼ 6/group) and a clinical score of surface maturation wasdeveloped based on scanning electron microscopy (SEM) pictures: the electronmicroscopy isotropy (EMI) score. In the second study, D-squames were collected on30 volunteers of the same age groups (excepted newborns) (n ¼ 6/group). SEMpictures were scored with the EMI score to validate surface maturation with age.

Results: The newborns’ skin is quickly adjusting to the challenging postpartumenvironmental conditions. However, some functions such as SC water-bindingproperties continue to develop even beyond the neonatal period with an impact onskin surface organization. Semiquantitative analysis of skin surface isotropy based onthe EMI score show a clear correlation between the age and the score.

Conclusion: Neonatal and infant skin is mainly competent under basal conditionsexcept SC water content and NMF components regulating the SC hydration level. Amaturation of the skin surface is also observed with EMI scores probably related tothe organization of the skin microanatomy after birth.

cial support: None identified.

Commer

P7024Subcutaneous histiocytoid Sweet syndrome in an adolescent with Crohn’sdisease

Marta Gonz�alez-Sab�ın, MD, MD, Complejo Hospitalario Universitario A Coru~na, ACoru~na, Spain; Ana Moreno-�Alvarez, PhD, PhD, Complejo HospitalarioUniversitario A Coru~na, A Coru~na, Spain; Eduardo Fonseca-Capdevila, PhD,PhD, Complejo Hospitalario Universitario A Coru~na, A Coru~na, Spain; RaquelIglesias-Conde, MD, MD, Complejo Hospitalario Universitario A Coru~na, ACoru~na, Spain; Roberto �Alvarez-Rodr�ıguez, PhD, PhD, Complejo HospitalarioUniversitario A Coru~na, A Coru~na, Spain; Rosa Mar�ıa Fern�andez-Torres, PhD, PhD,Complejo Hospitalario Universitario A Coru~na, A Coru~na, Spain, Susana Castro-Aguiar, PhD, PhD, Complejo Hospitalario Universitario A Coru~na, A Coru~na,Spain

Background: To describe one case of histiocytoid Sweet syndrome, a histopatho-logic variant of Sweet syndrome, in a young patient with Crohn’s disease.

Methods: We present a 14-year-old male with history of hay fever and Crohn’sdisease since he was 13 treated with infliximab and azathioprine. Despite treatmentthe patient developed an intense outbreak of Crohn’s disease with an abscess in thepresacral area, starting treatment with adalimumab, teicoplanin, meropenem andmetronidazole. He was admitted to for general malaise, abdominal pain, rector-rhagia, fever and appearance since 2 days ago of urticarial papules and nodules onthe legs, which were painful on palpation.

Results: The histologic features include diffuse infiltrates into the deeper dermisextending into the subcutaneous tissue composed of neutrophils and cells ofmonocytic-histiocytic appearance. The infiltrating cells were in part immature withround, oval or indented nuclei. Immunohistochemical study of these immature cellsshowed positive staining for myeloperoxidase. Routine blood cell count showedanemia (hemoglobin, 112 g/L) with elevation of white blood cells (18.85 3 109/L)and neutrophilia (76.4%). Other laboratory evaluations showed elevated erythro-cyte sedimentation rate (101 mm/hr) and C-reactive protein. The remainingdiagnostic tests showed no abnormalities. Treatment with methylprednisolonewas started with disappearance of fever in 24 hours and progressive improvement ofskin lesions.

Discussion: Histiocytoid Sweet syndrome represents a histopathologic variant ofSweet syndrome, characterized by an infiltrate composed mainly of immaturegranulocytes, which can be misinterpreted as histiocytes. This histopathologicvariant should not be mistaken with leukemia cutis or other inflammatory derma-toses that are histopathologically characterized by histiocytes interstitially arrangedbetween collagen bundles of the dermis. Sweet syndrome is an unusual extra-intestinal manifestation of Crohn’s disease, especially the subcutaneous histiocyticvariant. In the few cases reported, it is described in patients with underlyinghematologic disorders or caused by several drugs, but very rarely in patients withinflammatory bowel disease.

Conclusions: To our knowledge, this is the first case of subcutaneous histiocytoidSweet syndrome in a pediatric patient.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB177