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Multiple cylindromas: Case report
Posters - Genodermatoses
Genodermatoses Bilateral extensive nevus comedonicus ElP149 associated with multiple basal cell epitheliomas, trichofolliculoma accessory digit
and
E. Alpsoy’, C. Cayirli’, G. 6zbilim*, G. Karpuzoglu*, E. Yilmaz’. ‘Departments of Dermatology; *Pathology, Akderriz University School of Medicine, Antalya, Titrkey Nevus comedonicus is an uncommon developmental anomaly of the pilosebaceous unit, characterized by grouped, slightly elevated papules that have in their center a dark, firm hyperkeratotic comedo-like plugging. Although it has generally a lineer or zosteriform configuration as an isolated phenomenon, cases with more extensive bilateral involvement and in association with follicular tumors have been infrequently published. We describe a 28-year-old man as an additional case of bilateral extensive nevus comedonicus in association with multiple basal cell epitheliomas. trichofolliculoma and accessory digit. His condition was also complicated by intermittant abscesses and cyst formation. These associations has not been reported previously.
IPi50
A case of peeling skin syndrome with pitting of fingernails
C. Catal, C. Cayirli, E. Alpsoy, E. Yilmaz, .$ Yazar, E. Bagaran. Department of Dermatology, Akdeniz University School of Medicine, Antalya, Turkey Peeling Skin Syndrome is a rare. disease with a wide clinical picture, characterized by mainly continual and spontaneous superficial peeling involving the whole body or large part of it, and histologically by separation of the stratum comeum above the stratum granulosum. We describe an otherwise asymtomatic and in good general health l6-year-old boy with spontaneous, noninflammatory extensive skin shedding, palmar and plantar hyperkeratosis, and pitting of the fingernails. Histologically, there was a mild orthohyperkeratosis with a normal epidermis, splitting and separation in the lower part of stratum corneum. Based on the clinical appearance and dermatopathological examination, he was diagnosed as type A peeling skin syndrome.
which appeared on the lower extremities at the age of eighth months and later spread to other regions of the body. The child we describe is an additional sporadic case of EKV who was diagnosed based on the clinical appearance and dermatopathological examination, and was treated with topical tretionin cream (0.05%) and emmoliation with substantial reduction of the lesions. I Pi 52 Steatocystoma multiplex: Oral isotretinoin
treatment combined with cryotherapy
R. Apaydm’, N. Bilen’, D. Bayramgiirler’, F. BqdaS’, G. Harova’, $. Dakmeci ’ . ‘Faculty of Medicine, University of Kocaeli, izmit, Turkey Steatocystoma multiplex (SM) is an inherited disorder characterized by the appearance of cyts during the first or second decade of life. It is usually a minor cosmetic problem but rarely, cysts become severely inflamed and suppurated. This rare variant is known as steatocystoma multiplex suppurativum (SMS). We describe a 30 year-old man presenting numerous papules, nodules and inflamed cysts. The lesions were located all over the body including scalp except the palms and soles. His mother and one sister have had also similar lesions but less extensively. Histopathology of the biopsy specimens obtained from the anterior chest wall, axillary and the back region was consistent with SM. Oral isotretinoin treatment was started. At the same time, cryotherapy was used for non-suppurating lesions smaller than 2 cm. When the patient was evaluated 6 months later, cosmetic results were fairly good. Therefore, isotretinoin treatment was stopped, and cryotherapy was continued. I Pi 53 Multiple cylindromas: Case report C. de Cuyper, E. Wittouck’, Ch. Geeraects*. ‘Department of Dermatology, AZ Shit-Jan, Ruddershove IO, Brugge; 2Moerstraat 2.5, Brugge, Belgium We present a 73-year-old lady with multiple cylindromas on the scalp, face and neck. Several members of her family are affected (autosomal dominant inheritance). The clinical and histological features of the tumors are illustrated. ElPI54
1Pi51 1 A case of erythrokeratodermia
variabilis
C. Cayirli’, 0. Kal’, T. Gelen*, G. Karpuzoglu*, E. Yilmaz’, E. Alpsoy’, 0. Yegin 3. ‘Departments of Dermatology; ‘Pathology; :‘Pediatric.s, Akdeniz University School of Medicine, Antalya, Turkey Erythrokeratodermia variabilis (EKV) is a very rare genodermatosis, characterized by circumscribed erythematous patches and hyperkeratotic plaques of sparse but generalized distribution. Although, it is generally believed to be autosomal dominant with incomplete penetrance and variable expressivity, sporadic cases have been published as well. An otherwise healthy, 3-year-old girl was referred to our department with the complaint of widespread skin eruptions
s221
A typical familial Papillon-Lef&vre syndrome
H.S. InalBz’, M. Hat-man”, S. Akdeniz”, S.S. Inaliiz*, A.G. Isik3. ‘Univ of Wales CON of Medicine, Cardij UK; 2Univ of Dicle Faculty of Medicine, Diyarbakir; 3Univ of Istanbul Faculty of Dentistry, Istanbul, Turkey In 1924, Papillon and Lef&vre described a new fbmi of’keratoderma as a variant of Mal de Meleda dir&e with dental abnormalities. Since then, there has been an increase in the number of cases and today it is recognised as an independent disease entity. The Papillon-LeRvre syndrome (PLS) is a rare autosomal recessive disorder which first manifestations usuaUy starting in the first 6 months of life. The main dharacteristic features of the syndrome are palmo-plantar hyperkeratosis and generalised rapidly progressive periodontitis accompanied by
Genodermatoses Bilateral extensive nevus comedonicus ElP149 associated with multiple basal cell epitheliomas, trichofolliculoma accessory digit
and
E. Alpsoy’, C. Cayirli’, G. 6zbilim*, G. Karpuzoglu*, E. Yilmaz’. ‘Departments of Dermatology; *Pathology, Akderriz University School of Medicine, Antalya, Titrkey Nevus comedonicus is an uncommon developmental anomaly of the pilosebaceous unit, characterized by grouped, slightly elevated papules that have in their center a dark, firm hyperkeratotic comedo-like plugging. Although it has generally a lineer or zosteriform configuration as an isolated phenomenon, cases with more extensive bilateral involvement and in association with follicular tumors have been infrequently published. We describe a 28-year-old man as an additional case of bilateral extensive nevus comedonicus in association with multiple basal cell epitheliomas. trichofolliculoma and accessory digit. His condition was also complicated by intermittant abscesses and cyst formation. These associations has not been reported previously.
IPi50
A case of peeling skin syndrome with pitting of fingernails
C. Catal, C. Cayirli, E. Alpsoy, E. Yilmaz, .$ Yazar, E. Bagaran. Department of Dermatology, Akdeniz University School of Medicine, Antalya, Turkey Peeling Skin Syndrome is a rare. disease with a wide clinical picture, characterized by mainly continual and spontaneous superficial peeling involving the whole body or large part of it, and histologically by separation of the stratum comeum above the stratum granulosum. We describe an otherwise asymtomatic and in good general health l6-year-old boy with spontaneous, noninflammatory extensive skin shedding, palmar and plantar hyperkeratosis, and pitting of the fingernails. Histologically, there was a mild orthohyperkeratosis with a normal epidermis, splitting and separation in the lower part of stratum corneum. Based on the clinical appearance and dermatopathological examination, he was diagnosed as type A peeling skin syndrome.
which appeared on the lower extremities at the age of eighth months and later spread to other regions of the body. The child we describe is an additional sporadic case of EKV who was diagnosed based on the clinical appearance and dermatopathological examination, and was treated with topical tretionin cream (0.05%) and emmoliation with substantial reduction of the lesions. I Pi 52 Steatocystoma multiplex: Oral isotretinoin
treatment combined with cryotherapy
R. Apaydm’, N. Bilen’, D. Bayramgiirler’, F. BqdaS’, G. Harova’, $. Dakmeci ’ . ‘Faculty of Medicine, University of Kocaeli, izmit, Turkey Steatocystoma multiplex (SM) is an inherited disorder characterized by the appearance of cyts during the first or second decade of life. It is usually a minor cosmetic problem but rarely, cysts become severely inflamed and suppurated. This rare variant is known as steatocystoma multiplex suppurativum (SMS). We describe a 30 year-old man presenting numerous papules, nodules and inflamed cysts. The lesions were located all over the body including scalp except the palms and soles. His mother and one sister have had also similar lesions but less extensively. Histopathology of the biopsy specimens obtained from the anterior chest wall, axillary and the back region was consistent with SM. Oral isotretinoin treatment was started. At the same time, cryotherapy was used for non-suppurating lesions smaller than 2 cm. When the patient was evaluated 6 months later, cosmetic results were fairly good. Therefore, isotretinoin treatment was stopped, and cryotherapy was continued. I Pi 53 Multiple cylindromas: Case report C. de Cuyper, E. Wittouck’, Ch. Geeraects*. ‘Department of Dermatology, AZ Shit-Jan, Ruddershove IO, Brugge; 2Moerstraat 2.5, Brugge, Belgium We present a 73-year-old lady with multiple cylindromas on the scalp, face and neck. Several members of her family are affected (autosomal dominant inheritance). The clinical and histological features of the tumors are illustrated. ElPI54
1Pi51 1 A case of erythrokeratodermia
variabilis
C. Cayirli’, 0. Kal’, T. Gelen*, G. Karpuzoglu*, E. Yilmaz’, E. Alpsoy’, 0. Yegin 3. ‘Departments of Dermatology; ‘Pathology; :‘Pediatric.s, Akdeniz University School of Medicine, Antalya, Turkey Erythrokeratodermia variabilis (EKV) is a very rare genodermatosis, characterized by circumscribed erythematous patches and hyperkeratotic plaques of sparse but generalized distribution. Although, it is generally believed to be autosomal dominant with incomplete penetrance and variable expressivity, sporadic cases have been published as well. An otherwise healthy, 3-year-old girl was referred to our department with the complaint of widespread skin eruptions
s221
A typical familial Papillon-Lef&vre syndrome
H.S. InalBz’, M. Hat-man”, S. Akdeniz”, S.S. Inaliiz*, A.G. Isik3. ‘Univ of Wales CON of Medicine, Cardij UK; 2Univ of Dicle Faculty of Medicine, Diyarbakir; 3Univ of Istanbul Faculty of Dentistry, Istanbul, Turkey In 1924, Papillon and Lef&vre described a new fbmi of’keratoderma as a variant of Mal de Meleda dir&e with dental abnormalities. Since then, there has been an increase in the number of cases and today it is recognised as an independent disease entity. The Papillon-LeRvre syndrome (PLS) is a rare autosomal recessive disorder which first manifestations usuaUy starting in the first 6 months of life. The main dharacteristic features of the syndrome are palmo-plantar hyperkeratosis and generalised rapidly progressive periodontitis accompanied by