P255 Prevention of solar urticaria using a broadspectrum sunscreen and determination of a solar urticaria protecting factor (SUPF)

Posters -

s211

Photobiology

Photobiology

We discuss, through a review of the literature, the clinical features and diagnosis of this rare syndrome. I P252 Visualization of transglutaminase deficiency in lamellar ichthyosis

1

M. Rhagunath ’ , F. Velten ‘,‘, H.Ch. Hennies3, A. Reis3, PM. Steinert4, H. Traupe’ . ‘Department of Dermatology, Miinster; 31nst. of Human Genetics, Virchow Hospital, Berlin, Germany, 2Lab mixte, Jouy-en Josa, France, USA

INRA-CEA de radiobiologie 4L.ab. of Skin Biology, NIH,

appliquee Bethesda,

I P253 Proteus syndrome: A case report S. Gtindtiz, T. Uygur Derin, T. Mansur, M. Gorgec. Numune

prevents photo-provocation - Induced polymorphous light eruption (PLE) and keratinocyte (KC) ICAM- expression H. Stege’, C. Ahrens’, C. Billmann-Eberwein’, T. Ruzicka’, A. Rougier2, J. Krutmann ’ ‘Department of Dermatology, University of DusseldorJ; Germany, 2L.a Roche-Posay Pharmaceutical

In some families autosomal recessive lamellar ichthyosis (ARLI) could be shown to be due to a deficiency of transglutaminase I which resides on the long arm of chromosome 14, while in other families a disease locus on chromosome 2q 33-35 could be identified. For genetic counselling and in view of future corrective gene therapy a biochemical characterization of individual patients with lamellar ichthyosis is therefore desirable. However, currently the biochemical assay is tedious and dependent on the successful cultivation of ARLI keratinocytes. We therefore developed a histochemical assay that allows to visualize activity and spatial expression of transglutaminase 1 in skin using covalent incorporation of biotinylated donor substrates such as biotinyl-monodansylcadaverine. Among 15 patients with lamellar ichthyosis studied we found four with deficient transglutaminase 1 activity. Molecular studies confirmed only in these four patients respective mutations of transglutaminase 1. We conclude that a considerable proportion of patients with lamellar ichthyosis have a normal transglutaminase 1 activity.

Haydarpaga

Pretreatment of human skin with a I P254 sunscreen or dihydroxy-acetone (DHA)

Hospital,

Istanbul,

Turkey

The term “Proteus syndrome” was first used in 1983 to describe a disorder of skeletal hamartomatous and other mesodennal malformations. Major clinical findings, defined as those findings seen in more than half of the cases, include hemihypertrophy, macrodactyly, exostoses, epidetmal nevi, characteristic cerebriform masses involving the plantar or palmar surfaces, a variety of subcutaneous masses and scoliosis. In this report, we present a case of Proteus syndrome in a 13 year-old female patient with the following clinical features: bilateral macrodactyly and bilateral gigantism of feet, linear epidermal nevus, abdominal subcutaneous lipomatous masses. We discuss, through a review of the literature, the clinical features, the diagnosis and the management of this rare syndrome.

Laboratories,

Courbevoie,

France

PLE represents an abnormal response of human skin to UVAradiation (UVAR), which is characterized by the expression of proinflammatory molecules such as ICAM- 1 on the surface of KC. We have assessed whether the induction of KC ICAMexpression and the development of skin lesions in PLE patients may be affected by topical application of a UVA/UVB filtering sunscreen (Eusolex 6300 + TiOz + Parsol 1789 + Mexoryl SX; Anthelios L) or pretreatment with DHA (Autohelios). Skin lesions were induced by simultaneously using 2 different protocols: (i) exposure of previously affected skin to 3 x 100 J/cm’ UVA or (ii) to 20, 20, 30, 40, and 60 J/cm2 UVA for 5 days. We found that Anthelios L was highly effective in protecting against UVAR-induced skin lesions in 900 patients regardless of the provocation protocol used. A strong KC ICAMexpression was observed in UVA-irradiated, but not in sunscreen pretreated + UVA-irradiated, or normal control skin. Pretreatment of skin areas with DHA also provided protection against UVAR-induced PLE lesions in 5/8 patients. These studies indicate that both the broad spectrum sunscreen and, to a lesser extent, the DHA formulation tested, provide significant protection against the induction of skin lesions in PLE. Moreover, KC ICAM- expression and development of skin lesions in PLE patients are closely linked and may be causally related. I P255 Prevention of solar urticaria using a

broadspectrum sunscreen and determination of a solar urticaria protecting factor (SUPF)

J.L. Peyron ’ , D. Moyal 2, A. Rougier 3, C. Hourseau 2. ’ Departement of Dermatology, Saint Eloi Hospital, Montpellier; Pharmaceutical

21’Oreal Research, Clichy; 3La Roche-Posay Laboratories, Courbevoie, France

Solar urticaria (SU) is a rare photodennatosis whose preventive treatment remains difficult. An alternative would of the use of sunscreen products. Unfortunately, up to now, most them were found poorly effective as SU is known to be elicited mainly by UVA. We determined the Solar Urticaria Protection Factor (SUPF) using a broadspectrum sunscreen containing UVB (Eusolex 6300 + TiOz) and UVA (Pars01 1789 + Mexoryl SX) filters (SPF (UVB) = 60; IPD/PPD (UVA) = 55/12). 10 patients susceptible to SU were investigated. The action spectrum of SU was determined in each patient following irradiation with a xenon arc solar simulator. Spectral bands used were: 360 f 10 nm (long UVA), 335 f 10 nm (short UVA), 310 f 10 nm

s212

Posters - Photobiology

(UVB). The Minimal Urticarial Dose (MUD) was assessed for each patient in his triggering spectral band. SUPF was then determined after topical application of the sunscreen formulation or its vehicle according to an arythmetical ratio of 2 MUD. Clinical assessment of erythema and swelling was performed in the early minutes following each irradiation dose. 6 patients responded to UVA (long UVA: 3; short UVA: 1; both: 2). 1 to UVB and 3 to UVA + UVB. The MUD ranged from 80 mJ to 1 J/cm* in the long UVA, from 40 mJ to 2 J/cm* in the short UVA and from 40 to 60 m J/cm2 in the UVB. The SUPF of the vehicle ranged from 2 to 4, whereas that of the sunscreen formulation reached respectively 90 and 50 in the long UVA and in the short UVA, and 133 in the UVB. These results demonstrate that SU can be effectively prevented when using a highly protective UVB and UVA sunscreen. I P256 Treatment of psoriasis resistant to other

modalities by topical photodynamic

therapy

C. Beier, W.-H. Boehncke, T. Schmitt, A. Rtick’ , R. Kaufmann. Dpt. of Dermatology, Frankfurt University; ‘Inst.

of Laser

Technologies,

Ulm

University,

Germany

Photodynamic therapy (PDT) combines the application of photosensitizers absorbing light in the visible spectral region and irradiation with light of corresponding wavelengths. We here report on a patient with chronic plaque-stage psoriasis treated with topical polychromatic PDT. She exhibited an area measuring 1000 cm* not responding to either dithranol, vitamin D, or SUP. PDT was performed applying 10% methylene blue (MB) as sensitizer and irradiation with red light (PDT1200, Waldmann, Germany; 30 J/cm*; 70 mW/cm*). After 3 weeks of treatment (2x/week; cumulative dose: 180 J/cm*) the plaque had completely cleared without side effects. Noteworthy, the patient did not complain of pain, a symptom often necessitating dose reduction or even termination of treatment when using aminolevulinic acid as sensitizer. Given the possible involvement of bacteria in the psoriasis-pathogenesis the antimicrobial effect of MB may add to the rationale of this regimen. ElP257 Congenital dermatofibrosarcoma

protuberans presenting as a soft depressed plaque

A.D. Yticelten’, Z. Demircay ‘, 0. Gtlrbtlz’, N. Btiyiikbabani *, E. Tezel 3. ’ Marmara University

School Medicine, Department of Dermatology; 21stanbul University School of Medicine, Department of Pathology; 3Marmara University School of Medicine, Department of Plastic and Reconstructive Surgery, Istanbul, Turkey

of

Dermatofibrosarcoma protuberans (DFSP) typically presents during early or mid-adult life as a nodular cutaneous mass. It is a rare tumor which constitutes less than 0.1% of all malignancies. It is even rarer in children and in literature a total of 27 cases have been reported in people less than 16 years of age with only 5 congenital cases. Here, we report a 14-year-old boy with a DFSP lesion on his back which was present at birth. In addition to being a childhood and a congenital case the lesion was also notable since it presented as a soft and depressed

plaque lesion without induration tissue.

resembling an atrophic scar

ElP258 Malignant melanoma during the last decade

in Greece

K. Alvanopoulos, 0. Papadopoulos, E. Hatziolou, A. Vareltzidis, A.D. Katsambas. “A. Sygros” Hospital,

Athens,

Greece

We refer to 152 patients with 153 malignant melanomas, which were excised in our hospital during the decade 1987-1997 (63 men and 89 women). According to the histologic examination, 62 of them were nodular, 59 were superficial spreading melanomas, 28 were lentigo and 4 acral lentigo melanomas. Most of them were observed mainly in the elderly and the most frequent sites were the back for the male and the head for the female. The tumor thickness was various: 55 patients presented a tumor thickness less or up to 0.75 mm, 29 had thickness that varied from 0.76 mm to 1.49 mm, 20 from 1.5 mm to 2.49 mm, 23 from 2.5 mm to 3.9 mm and 26 had thickness over 4 mm. The 46 malignant melanomas were observed during the first five years, while the 107 during the last five years. The dramatic increase of the incidence of malignant melanomas during the last five years is discussed. The results concerning the follow up of the patients participated in our study are also reported. ElP259 Atypical cutaneous Kaposi Sarcoma in

immunocompetent tuberculoma

patient with suspect lung

D. Schena, G. Tessari, C. Chieregato, S. Righetti ’ , A. Barba. Department Pneumology

of Dermatology, University of Verona; ‘Division Azienda Ospedaliera di Verona, Verona, Italy

of

Case Report: In December 1996, a 73 years old woman was admitted in our Department because of the onset, 5 months before, of multiple bluish-red ovalar, fused or linear skin papules and nodules on the face, neck, forearm, trunk and thighs. The lesion size ranged from 10 to 20 mm in length and 3 to 15 mm in width. The whole disease had appeared in a few months’ interval. Routine laboratory tests, HIV serology and lymphocyte sub-populations count were all normal. Skin biopsy was positive for a Kaposi Sarcoma (KS). Chest x-ray and total body C.A.T. scan detected a solid mass with indented borders, 25 mm in diameter, on the right lung upper lobe. A chest x-ray, done in February 1996, had been negative. The patients underwent surgical treatment. Histology demonstrated multiple necrotizing granulomas with giant cells, consistent with tuberculoma KS lung dissemination and a primary neoplasm were excluded. Isoniazid therapy was started. After the surgical intervention we observed the partial spontaneous regression of skin lesions. The patient is being followed up in our Department. Discussion: We report the onset of multiple atypical KS skin lesions in a patient with no evidence of congenital, infectious or drug-induced immunodeficit. Also, skin lesions partially recovered after the lung lesion had been removed. Rappesberger et al. found some retroviral-like particles in KS lesions of immunocompetent people and considered them as possible etiologic agents of KS. He also hypothesised that infectious agents such