P237 Chronic mucocutaneous candidiasis: Immediate hypersensitivity reactions as possible complicating factors in a case of pseudotumor of candidoma in brain

S208

IP237

Posters -

Chronic mucocutaneous candidiasis: Immediate hypersensitivity reactions as possible complicating factors in a case of pseudotumor of candidoma in brain

M. Moin, Z. Pourpak, A. Farhudi. Department & Allergy,

Children’s

Hospital

Medical

Center;

of Immunology I.R, Iran

An 11 year old girl, a known case of CMC operated for a space occupying lesion in temporal lobe of brain is described which the patololgy report revealed pseudotumor or of candidoma. Her history included oral candida from birth, food allergy with IgE > 1500, positive Skin Prick test & positive open food challenge test. Immune evaluation revealed normal lymphocyte count & differentiation, allergy to candida delay skin test but sensitivity to PPD & DT, and so normal LIT with PHA but defect in LTT with candida. Skin prick test and intradermal test with cadida antigen, negative and positive control, showed a + 4 IgE mediated response to candida. All of the other immunologic tests were normal. Since disseminated candidasis as brain candidoma is unusual in CMC, thus a combination of CMC, IgE mediated hypersensitivity reaction to candida and food allergy (that probably there is a cross reaction with some food allergens and candida antigens) seems to be the complicating factors in this case which can be due to THl - TH2 immunodysregulation. I P238 Granulomatous

skin lesions in a patient with ataxia telangiectasia: A case report

N. Sentiirk, S. Sahin, A. Gdkoz, N. Atakan. Hacettepe Medical

School,

Dept.

of Dermatology,

Ankara,

Univ.

Paediatric

pulmonary stenosis and focal septal hypertrophy when he was 2 months old. He has a dysmorphic face with prominent forehead, hypertelorism, broad depressed nasal bridge and low-set ears. Pectus excavatus and cubitus valgus were also noticed. When he was 2 months old, he developed erythema and follicular hyperkeratosis affecting the outer aspects of the eyebrows and extensor surfaces of the arms. We diagnosed them as ulerythema ophrygenes and keratozis pilaris. We observed a red to blue colored soft keloidal tissue on his left foot, at the tip of the third toe. It had occurred after trauma when he was 4 and was resected by a surgeon at age 5 but a few months later, it reoccurred larger. Our case nearly fulfills all the characteristic features of Noonan syndrome, was a tendency to form keloidal tissue. I P240 Juvenile discoid lupus erythematosus E.A. McMullen, K. Armstrong, E.A. Bingham. Royal Victoria Hospital,

Belfast,

Ireland

I present the case of a 9 year old girl with a short history of crusted discrete lesions on the cheeks and ears, without history of photoaggravation. A clinical diagnosis of impetigo was made but there was no clinical response to antistaphlococcal agents. On review areas of scarring were apparent and new lesions developed. A differential diagnosis of DLE, Erythropoetic protoporphyria or Hydroa vacciniforme was proposed. Histology and full serological investigation confirmed the diagnosis of Juvenile Discoid Lupus Erythematosus.

Turkey

Ataxia-telangiectasia is a rare autosomal recessive syndrome characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, variable degrees of humoral and cellular immunodeficiency, recurrent sinopulmonary infections and a high risk of malignancy. In patients with Ataxia-telangiectasia, progeria and sclerotic changes, lipoatrophy, recurrent severe impetigo, xeroderma, keratosis pilaris, seborrheic dermatitis, atopic dermatitis, nummular eczema, mottled hyper and hypopigmentation, vitiligo, cafe au lait spots, acanthosis nigricans scalp hair and alopecia areata have been described. In addition to all these findings cutaneous granulomatous lesions have been described in Ataxia-telangiectasia patients. Here in we report a twelve-year old girl with Ataxia-telangiectasia who developed granulomatous skill lesions in which no infectious agent could be isolated.

I P241 Goltz syndrome: Case report T. Mansur, S. Gtindiiz, T. Uygur Derin. Haydarpasa Hospital,

Istanbul,

Numune

Turkey

Focal dermal hypoplasia (Goltz syndrome) is a rare multisystem condition with characteristic cutaneous and facial manifestations, dental, skeletal, opthalmologic and urinary abnormalities. In this report, we present a twenty year-old woman with a variety of cutaneous defects, right-sided phytosis bulbi and ectropion, left-sided coloboma, protruding ears and thinning of the earlobes, early loss of teeth, deviation of the nasal septum, scoliosis and syndactyly of the feet. The patient had patchy alopecia of the scalp, linear streaks of atrophy and pigmentation on extremities and trunk, soft, yellow nodules on axillary folds, cribriform hypo and hyperpigmented patches on the abdomen and forehead. The clinical manifestations, possible inheritence pattern are discussed and a review of literature is given.

rl P239 Noonan syndrome: A case with recurrent

keloid formation

T.O. Giilec, A. Karaduman. University Medicine,

Department

of Dermatology,

of Hacettepe, Faculty Ankara, Turkey

of

Noonan syndrome, a genetic condition with autosomal dominant inheritance with variable penetrance, is characterized by typical craniofacial appearance, congenital cardiac defects, orthopedic abnormalities, psychomotor and growth retardation and some skin changes including keloidal tissue formation. We want to report a 6 year old boy having a diagnosis of

I P242 Visualization of transglutaminase

1

deficiency in lamellar ichthyosis

M. Rhagunath ’ , F. Velten ‘I*, H.Ch. Hennies3, A. Reis3, PM. Steinert4, H. Traupe’ ‘Dept. of Dermatology, Munster; 31nst. of Human Genetics, Virchow Hospital, Berlin, *Lab mixte, INRA-CEA de radiobiologie applique’e, Josa, France, 4Lab. of Skin Biology, NIH, Bethesda,

In some families

autosomal

recessive lamellar

Germany, Jouy-en USA

ichthyosis