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P236 Subacute cutaneous lupus erythematosus associated with hereditary C1 inhibitor deficiency
Posters
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been previously reported. In addition we discuss the possible role of CyA in the patbogenesis of EED associated with liver transplant.
V. Bar&DruSko,
I. RuEeviC. Department
Clinical
Osijek,
Croatia,
0P235
Angiokeratoma a young boy
of Dermatology,
Europa
50% of patients with vitiligo develop illness before the age of 20. Authors examined 65 children with vitiligo age from O-15. The youngest child was 2.5 year old. Sex ratio was 36 female: 29 male patients. Authors were looking blood system, heredity and “trigger” factors (inflammatory focus, psychological trauma, etc.). In ABO blood system authors found the prevalence blood group A among children with vitiligo, what is prevalence in general populations too in our country. The family frequency of vitiligo among children amounts 17.00%. The most frequently observed “trigger” factors have been psychological trauma (56.93%) and inflammatory focus (30.73%).
of Fordyce of the scrotum in
I. Neri, A. Patrizi, F. Bardazzi, G. Tosti, C. Landi. Department of Clinical and Experimental Dermatology (Head Claudio Bologna, Italy
I P233 Vitiligo in childhood Hospital
S207
Paediatric
Medicine, Division Varotti), University
of of the Study
of
Angiokeratomas are vascular dark bright red to black, small papules that in time become keratotic. Four types of angiokeratomas have been recognized and angiokeratoma of Fordyce is the form that involves the genital area. We report here a case of angiokeratoma of Fordyce in a boy of 14 years of age. The lesions appeared at birth but the parents noticed a recent increase in their size and number. On examination we observed numerous dark red, well demarcated dome shaped papules 1-3 mm in diameter, some with keratotic overlying skin, localized on the scrotum and on the raphae of the penis. Although angiokeratoma of Fordyce is the most common type of angiokeratoma, it is not a frequent desease. It has been very rarely observed in childhood and adolescence and, to the best of our knowledge, no cases of congenital Fordyce angiokeratoma have been reported until now. Moreover in our patient the lesions increased in size and number with age and a similar evolutin can also be observed in congenital angiokeratoma circumscriptum.
ElP234 Pigmentovascular
phakomatosis. Report of three cases from the Middle East
G.G. Lestringant, I.H. Galadari, P.M. Frossard. Tawam Hospital
and FMHS,
Al Ain,
UAE
Pigmentovascular phakomatosis (PVP) is defined as the simultaneous occurrence of widespread telangiectatic and pigmentary nevi. Happle et al. (Hautarzt 1989; 40: 721-4) have proposed that a mechanism of twin spots, which occurs commonly in plants and animals, could explain PVP, whereby both nevi would be the result of chromosomal mosaicism following two different mutations. We report here three new cases of this rare association that has so far mostly been evidenced in Japan. Case 1: was a 20 year-old UAE woman who presented with a right sided nevus of Ota and a 30 x 20 cm macular slate gray plaque on her upper back. In addition, a nevus flammeus was spread on most of the skin of the left upper limb, axilla and breast, and on the back where it overlapped with the pigmented lesion. The patient had also a mild thoraco-lumbar scoliosis. She had 11 unaffected siblings. Case 2: was an 18 year-old Yemeni girl who presented with giant bilateral nevus of Ota and widespread left sided nevus of ho. There were also two large telangiectatic nevi on her back. There were no obvious associated anomalies. Case 3: was a 3 year-old Saudi boy treated for infantile spasms. Although brain CT scan was normal, EEG indicated a left temporo-occipital lesion. A universal blue spot spared only part of the face and limbs and the front mid-line of the trunk. The face, neck and four limbs (including palms and soles) were covered by a nevus flammeus. PVP is divided into four phenotypes according to the type of pigmented nevus, and each type is subdivided into localized (a) and systemic (b) subsets. Cases 1 and 3 were Ilb; case 2 was ha. Patients 1 and 2 displayed nevi of Ota in absence of associated Sturge-Weber syndrome - which has never been reported. The clinical presentation of patient 2 is extremely unusual.
ElP236 Subacute cutaneous lupus erythematosus
associated with hereditary Cl inhibitor deficiency
R. De Lucas ‘, N. Hemandez-Cano’, C. Vidaurrazaga’, L. Perez-Espaha ’ , F. Contreras2, M. Casado ’ ‘Departments of Dermatology; Madrid, Spain
2Pathology,
L.a Paz Universitary
Hospital,
Introduction: Subacute cutaneous lupus erythematosus (SCLE) is an entity characterized by papulo-squamous and/or annular, non-scarring lesions, distributed in sun-exposed areas with specific histopathology of lupus erythematosus (LE). This picture has been very rarely reported in childhood. Case Report: We present a 1 l-year-old girl with itching cutaneous lesions, which had started 1 month before and were located over the sun-exposed areas (face, forehead, shoulders, arms and upper trunk). These lesions showed clinical and histopathological features of SCLE. Complementary studies revealed Cl-inhibitor deficiency, and this problem was also confirmed in the patient’s mother and sister. Commentary: Clinical presentation of hereditary Cl-inhibitor deficiency is angioedema in 97% of cases. Other associated diseases in affected patients include neoplasia, hypothyroidism, Sjogren syndrome, haematologic diseases and rheumatoid arthritis. All these clinical problems were not present in our patient, who showed SCLE lesions as the unique clinical manifestation in association with complement deficiency. On review of the literature, we have not found any previous case of SCLE associated with Cl-inhibitor deficiency. Finally, we discuss the aetiopathogenic aspects of this association.
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been previously reported. In addition we discuss the possible role of CyA in the patbogenesis of EED associated with liver transplant.
V. Bar&DruSko,
I. RuEeviC. Department
Clinical
Osijek,
Croatia,
0P235
Angiokeratoma a young boy
of Dermatology,
Europa
50% of patients with vitiligo develop illness before the age of 20. Authors examined 65 children with vitiligo age from O-15. The youngest child was 2.5 year old. Sex ratio was 36 female: 29 male patients. Authors were looking blood system, heredity and “trigger” factors (inflammatory focus, psychological trauma, etc.). In ABO blood system authors found the prevalence blood group A among children with vitiligo, what is prevalence in general populations too in our country. The family frequency of vitiligo among children amounts 17.00%. The most frequently observed “trigger” factors have been psychological trauma (56.93%) and inflammatory focus (30.73%).
of Fordyce of the scrotum in
I. Neri, A. Patrizi, F. Bardazzi, G. Tosti, C. Landi. Department of Clinical and Experimental Dermatology (Head Claudio Bologna, Italy
I P233 Vitiligo in childhood Hospital
S207
Paediatric
Medicine, Division Varotti), University
of of the Study
of
Angiokeratomas are vascular dark bright red to black, small papules that in time become keratotic. Four types of angiokeratomas have been recognized and angiokeratoma of Fordyce is the form that involves the genital area. We report here a case of angiokeratoma of Fordyce in a boy of 14 years of age. The lesions appeared at birth but the parents noticed a recent increase in their size and number. On examination we observed numerous dark red, well demarcated dome shaped papules 1-3 mm in diameter, some with keratotic overlying skin, localized on the scrotum and on the raphae of the penis. Although angiokeratoma of Fordyce is the most common type of angiokeratoma, it is not a frequent desease. It has been very rarely observed in childhood and adolescence and, to the best of our knowledge, no cases of congenital Fordyce angiokeratoma have been reported until now. Moreover in our patient the lesions increased in size and number with age and a similar evolutin can also be observed in congenital angiokeratoma circumscriptum.
ElP234 Pigmentovascular
phakomatosis. Report of three cases from the Middle East
G.G. Lestringant, I.H. Galadari, P.M. Frossard. Tawam Hospital
and FMHS,
Al Ain,
UAE
Pigmentovascular phakomatosis (PVP) is defined as the simultaneous occurrence of widespread telangiectatic and pigmentary nevi. Happle et al. (Hautarzt 1989; 40: 721-4) have proposed that a mechanism of twin spots, which occurs commonly in plants and animals, could explain PVP, whereby both nevi would be the result of chromosomal mosaicism following two different mutations. We report here three new cases of this rare association that has so far mostly been evidenced in Japan. Case 1: was a 20 year-old UAE woman who presented with a right sided nevus of Ota and a 30 x 20 cm macular slate gray plaque on her upper back. In addition, a nevus flammeus was spread on most of the skin of the left upper limb, axilla and breast, and on the back where it overlapped with the pigmented lesion. The patient had also a mild thoraco-lumbar scoliosis. She had 11 unaffected siblings. Case 2: was an 18 year-old Yemeni girl who presented with giant bilateral nevus of Ota and widespread left sided nevus of ho. There were also two large telangiectatic nevi on her back. There were no obvious associated anomalies. Case 3: was a 3 year-old Saudi boy treated for infantile spasms. Although brain CT scan was normal, EEG indicated a left temporo-occipital lesion. A universal blue spot spared only part of the face and limbs and the front mid-line of the trunk. The face, neck and four limbs (including palms and soles) were covered by a nevus flammeus. PVP is divided into four phenotypes according to the type of pigmented nevus, and each type is subdivided into localized (a) and systemic (b) subsets. Cases 1 and 3 were Ilb; case 2 was ha. Patients 1 and 2 displayed nevi of Ota in absence of associated Sturge-Weber syndrome - which has never been reported. The clinical presentation of patient 2 is extremely unusual.
ElP236 Subacute cutaneous lupus erythematosus
associated with hereditary Cl inhibitor deficiency
R. De Lucas ‘, N. Hemandez-Cano’, C. Vidaurrazaga’, L. Perez-Espaha ’ , F. Contreras2, M. Casado ’ ‘Departments of Dermatology; Madrid, Spain
2Pathology,
L.a Paz Universitary
Hospital,
Introduction: Subacute cutaneous lupus erythematosus (SCLE) is an entity characterized by papulo-squamous and/or annular, non-scarring lesions, distributed in sun-exposed areas with specific histopathology of lupus erythematosus (LE). This picture has been very rarely reported in childhood. Case Report: We present a 1 l-year-old girl with itching cutaneous lesions, which had started 1 month before and were located over the sun-exposed areas (face, forehead, shoulders, arms and upper trunk). These lesions showed clinical and histopathological features of SCLE. Complementary studies revealed Cl-inhibitor deficiency, and this problem was also confirmed in the patient’s mother and sister. Commentary: Clinical presentation of hereditary Cl-inhibitor deficiency is angioedema in 97% of cases. Other associated diseases in affected patients include neoplasia, hypothyroidism, Sjogren syndrome, haematologic diseases and rheumatoid arthritis. All these clinical problems were not present in our patient, who showed SCLE lesions as the unique clinical manifestation in association with complement deficiency. On review of the literature, we have not found any previous case of SCLE associated with Cl-inhibitor deficiency. Finally, we discuss the aetiopathogenic aspects of this association.