P6321The expression of B-defensins, cathelicidin (LL-37) in skin tumors

Kyu Uang Whang, MD, PhD, Soonchunhyang University Hospital, Seoul, SouthKorea; Sang Jin Oh, MD, Soonchunhyang University Hospital, Cheonan, Cheonan,Chungcheongnam-Do, South Korea; Seung Il Choi, MD, SoonchunhyangUniversityHostpital, Seoul, South Korea; Sung Yul Lee, MD, PhD, Soonchunhyang UniversityHostpital, Cheonan, Cheonan, Chungcheongnam-Do, South Korea; Ye Seul Kim,MD, Soonchunhyang University Hostpital, Bucheon, Bucheon, Gyenggi-Do, SouthKorea; You In Bae, MD, Soonchunhyang University Hostpital, Bucheon, Bucheon,Gyenggi-Do, South Korea; Young Lip Park, MD, PhD, Soonchunhyang UniversityHostpital, Bucheon, Bucheon, Gyenggi-Do, South Korea

Antimicrobial peptides (AMPs), which is a kind of innate immune system, areproduced by keratinocytes during differentiation. Among them, B-defensins and LL-37 are the major human antimicrobial peptides. Because B-defensins and LL-37 arecritically involved keratinocyte migration and proliferation, we hypothesized thatboth peptides also have an influence on differentiation and proliferation of skintumors originated from keratinocytes. We studied the expressions of AMPs by tissuearray techniques in benign and malignant skin tumors. The expression of AMPs wasexamined by immunohistochemical staining of 31 specimens of skin tumorsincluding 4 cases of seborrheic keratosis, 4 cases of keratoacanthomas, 4 cases ofbasal cell carcinomas (BCC), 16 cases of squamous cell carcinomas (SCC), and 3cases of malignant melanomas (MM). Immunohistochemical analysis of the skintumor samples revealed a high expression of B-defensin and cathelicidin (LL-37) inbenign skin tumors such as keratoacanthomas and seborrheic keratosis than inmalignant tumors, such as SCCs, BCCs, and MMs. Among malignant tumors, BCCsand MMs showed lower expression of AMPs than SCCs, in which their expressionwas shown to be decreased with increased dysplasia of tumor cells. These findingsshowed that AMPs have a negative association with the malignancies of the skintumors and suggested the role of AMPs in the protection of loss of differentiation anddysplasia of skin tumors.

AB8

cial support: None identified.

Commer

PD05—CLINICAL DERMATOLOGY

P6851Adult-onset Still disease in a geriatric patient: Updates and pearls indiagnosis

Rebecca Hartman, Perelman School of Medicine at the University ofPennsylvania, Philadelphia, PA, United States; Daniela Kroshinsky, MD, MPH,Department of Dermatology, Massachusetts General Hospital, Boston, MA,United States; William Tsiaras, MD, PhD, Department of Dermatology,Massachusetts General Hospital, Boston, MA, United States

Background: Adult-onset Still disease (AOSD) is a rare inflammatory disorder ofuncertain etiology typically occurring in young adults. Diagnosis is often delayed ingeriatric patients, despite classic presentations. Here, we report the delayeddiagnosis of recurrent AOSD in a 77-year-old and review the literature on diagnosticcriteria with a focus on ferritin.

Observation: A 75-year-old man was hospitalized for fevers, arthralgia, and rash. Askin biopsy specimen revealed a sparse, perivascular lymphocytic infiltrate. Hissymptoms rapidly resolved with empiric steroids, but a definitive diagnosis was notreached. Two years later, he presented with 2 weeks of mild fevers, arthralgia, andodynophagia. Serial examinations revealed an evanescent pink macular rash. Heunderwent an exhaustive evaluation for autoimmune disease, infection, andmalignancy. Laboratory findings included a leukocytosis of 18.0 with 95% neutro-phils and a ferritin of 3854. He subsequently developed hypotension unresponsiveto fluid resuscitation. His symptoms resolved with high-dose steroids, although hisferritin continued to rise, peaking at 50,129.

Discussion: The diagnosis in our patient was difficult because of his age; only 4 casesof AOSD have been recorded in the literature in patients aged 75 years and older.This delay in diagnosis likely contributed to his episode’s severity. Although theYamaguchi diagnostic criteria have high sensitivity and specificity values, thediagnosis of AOSD is one of exclusion, causing unnecessary diagnostic interventionsand delays. There is no definitive laboratory test. Nonspecific hyperferritinemia,atypical for other autoimmune diseases, occurs in[70% of patients, correlatingwithdisease activity and prognosis. More specific tests for AOSD include a very highferritin ([1000) and a low fraction of glycosylated ferritin. The latter finding isattributed to release of intracellular non-glycosylated ferritin or rapid ferritinsynthesis outpacing glycosylation capacity. Fautrel et al have proposed noveldiagnostic criteria that include a low fraction of glycosylated ferritin as a majorcriterion. These criteria have a specificity of 98% in the absence of exclusion criteria,allowing earlier diagnosis. Such criteria and tests may be particularly valuable foratypical cases, including elderly patients, to facilitate rapid diagnosis and appropri-ate intervention.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6570Alitretinoin in the treatment of hidradenitis suppurativa: An insight

Samuel Menon, MBBS, Princess Alexandra Hospital, Essex, United Kingdom;Roberto Verdolini, MD, Princess Alexandra Hospital, Essex, United Kingdom

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease affecting theapocrine glandular zones with preferential involvement of the axillae, groin, andperineum. Its pathogenesis is thought to originate with follicular alterationleading to occlusion. Subsequently there ensues inflammation with acuteabscesses, fistula formation, and scarring. As the disease advances, pain becomesa persistent symptom. Indeed, the psychological impact of the disease issignificant, with depression scores being both higher than for any otherdermatological condition, and significantly higher than controls. The psycho-physical morbidity of HS is not in doubt, but as yet treatment modalities are notideal. Currently, the cornerstones of management are antibiotics, retinoids, andsurgery, with antiandrogens, corticosteroids, sulfones, antiinflammatories, andimmunomodulators also in the dermatologist’s arsenal. Acitretin has thus far beenthe systemic retinoid of choice, achieving a 6-month remission rate of 60% to 75%after 1 course. However, its teratogenicity profile is poor, with women ofchildbearing potential being advised to use contraception for 3 years posttreat-ment. Given that HS affects women more frequently than men (female:male ratio,3:1), this problem is particularly pertinent. Alitretinoin, on the other hand, is afirst-generation retinoid presently employed for severe chronic hand eczema.Unlike acitretin, it has a much smaller washout period: women need only usecontraception for 6 weeks posttreatment. This study demonstrates the efficacy ofalitretinoin in the treatment of HS, as measured by Dermatology Quality of LifeIndex (DLQI) and Sartorious scoring. Four patients between 30 and 75 years ofage were recruited into the study. Average depression scores as measured by theDLQI at week 0, 12, and 24 were 19.7, 8.0, and 6.7, respectively, achieving anaverage reduction of 13 at week 24. Indeed, similar results were achieved usingthe Sartorious scale, with an average reduction of 15.7. These preliminary resultsare encouraging and a larger study achieving statistical significance is the nextstep. In conclusion, the results provide support for the efficacy of alitretinoin ascompared with acitretin, but the fact that alitretinoin offers significant reductionsin risk of teratogenicity makes it the retinoid of choice for this debilitatingcondition.

cial support: None identified.

Commer

P5950Alternative treatments of vitamin D deficiency

Morten Bogh, MD, PhD, Department of Dermatology, Lund University, Malm€oHospital, Malm€o, Sweden; �Ake Svensson, MD, PhD, Department of Dermatology.Lund University, Malm€o Hospital, Malm€o, Sweden; Bo Ljunggren, MD, PhD,Department of Dermatology, Lund University, Malm€o Hospital, Malm€o, Sweden;Jill Gullstrand, MD, M€ollev�angen General Practice, Malm€o, Sweden; MozhganDorkhan, MD, PhD, Deparment of Clinical Sciences, Division of Diabetes andEndocrinology, Lund University, Malm€o Hospital, Malm€o, Sweden

The objective was to compare the efficiency of full body narrowband ultraviolet-B (NB-UVB) exposure 3 times per week with a daily oral intake of 1600 IU (40�g) vitamin D3 during a winter period of 6 weeks in Sweden. The participantswere randomized into 2 groups, 1 receiving NB-UVB exposure, and the otherreceiving vitamin D3 supplements. Thirty-two participants completed the studyperiod, 16 in each group. We found a significant difference in the 25-hydroxyvitamin D3 (25 (OH)D3) increase among the 2 groups after 6 weeks ofintervention (P \ .05). The 25 (OH)D3 level (mean) increased from 19.2 nmol/Lto 75 nmol/L in the NB-UVB treated group, and from 23.3 nmol/L to 60.6 nmol/Lin the oral vitamin D3etreated group. In conclusion, exposure to NB-UVB can bea useful alternative treatment of vitamin D deficiency for those with malabsorp-tion diseases, restricted outdoor access, noncompliant patients, and when thereis a need for rapid substitution.

cial support: None identified.

Commer

APRIL 2013