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P206 Spina bifida — Association with increased lower limb naevi
s200
IP204
Posters
- Miscellaneous
Clinical features and treatment of Hand-Schuller-Christian disease
P206 El
Spina biflda - Association with increased lower limb naevi
I.M. Raznatovsky, I.A. Moshkalova, E.V. Sokolovski.
N. Flanagan, F. Enright, L. Barnes, R. Watson. Departments
Department University,
Dermatology, Our Lady’s and St. James’s Hospital,
of Dermatology St. Petersburg,
Ll? Pavlov Russia
State Medical
Hospitalfor Sick Children, Dublin, Ireland
of
Crumlin
8 patienst with Langerhans cell histiocytosis (formely known as histiocytosis X) seen in seven years are reviewed. The male/female incidence ratio was 1: 1. The presentation of the disease varied from age 3 to 76. In all patients the delayed diagnosis took place; the disease ran a chronic progressive course without spontaneous involution. All the patients had different types of skin manifestation. There were red-brown to reddish yellow papules on the scalp, the upper trunk, and in the intertriginous areas resembling Darier’s disease. Individual lesions developed features of scaling, crusting, spontaneous resolution with residual scarring. Other lesions included vesicopustules, lipid infiltration of the eyelids, and ulcerated nodes highly suggestive of scrofuloderma. Two women had ulcerated oral and genital mucosa. 2/8 remained purely cutaneous; whereas 6/8 had multiple organ involvement. 4/6 had marked pulmonary changes. Brain lesions occurred in 4/6 cases; their diabetes insipidus was controlled with vasopressin. Osteolytic lesions were present in 3/6: displaced teeth, loss of alveolar bone, sclerosis of pyramids. No one exhibited exophthalmos. In patients with pulmonary lesions therapy with Prospidin (Bis-Betachlor-aethylamin) coupled with mean doses of glucocorticosteroids proved to be highly efficient. The others received prospidin or cyclophosphan only (one was also irradiated). One patient was lost to follow-up; the remainder were either improved with treatment or well. Conclusions: The degree and site of Langerhans cell proliferation varies greatly from patient to patient. More similar cases are to be reported for better understanding and better management of the disease.
Evidence that melanocyte growth may be under neural control has previously been reported’. Three spina bifida (SB) patients were noted to have increased numbers of naevi on the lower limbs on routine dermatological consultation. The following study was performed to test the hypothesis that SB may be associated with increased numbers of melanocytic naevi on the lower limbs. We prospectively examined 42 patients with SB and 42 age and sex matched controls (24 female: 18 male, age range 3-19 years, mean 10.6 years) seen consecutively at Our Lady’s Hospital, Crumlin from January to March 1996. Participants underwent full skin examination noting the number, size, distribution and presence of clinical atypia of melanocytic naevi. When compared to the Vancouver Mole Study’ both males and females with spina bifida demonstrated increased mean number of naevi on the lower limbs and this difference was most marked in females aged 13 and over (18.4 Vs 5). When compared with Irish age and sex matched controls, SB patients had similar mean total number of naevi. In females with SB the mean number of naevi on the lower limbs was over double that seen in controls (8.4 vs 4.1) whereas in males with SB it was less than that seen in controls (5.2 Vs 6.2). Fourteen of 42 SB patients had naevi measuring over 5 mm in diameter compared with 4 controls (p < 0.05). Eight of 42 SB patients had congenital naevi (outside the paraspinal area) compared with 2 controls (p = 0.09). A small number of patients were fo.und to have clinically atypical naevi. This study supports an association between spina bifida and increased numbers of naevi on the lower limbs. The reasons for this association are unclear. Potential mechanisms include closely related candidate genes and/or a possible influence of neuronal, hormonal or metabolic factors on melanocyte growth.
I P205 Why is medical malpractice so high in
References
Ireland?
M.M. Young. Adelaide
Hospital,
Dublin,
Ireland
The high level of medical litigation is a reflection of a litiginous society. Consequently medical insurance fees are considerably higher than Britain. A comparison of attitudes in Ireland and the rest of Europe help to explain these findings. Unlike Britain, Ireland has a written constitution which guarantees individual rights which confer greater protection to an individual than those given by the European Court for the protection of human rights. In Ireland lawyers take cases on a ‘no foal, no fee’ basis, thus giving greater access to the courts. Reports from expert witnesses, such as medical practitioners, can be obtained without expense to the plaintiff. Judicial awards are higher than in other E.U countries, being on average 40% higher than in Britain and 70% higher in comparison to some E.U. countries and they continue to remain high despite the abolition of juries. The increasing trend of patients to question and influence medical treatment makes it likely that litigation will continue to rise.
[II Linear arrangement of multiple congenital melanocytic nevi.
Effendy I, Happle R. J Am Acad Dermatol 1992; 27: 8534. 121 Gallagher KP, McLean DI, Yang CP, Coldmau AJ, Silver HK Spinelli JJ, Beagrie M. Anatomic Distribution of Acquired Melanocytic Nevi in White Children. Arch Dermatol 1990; 126: 466-47 1.
1P207 1 Calciphylaxis P. Gheeraert ’ , A. Theunis ’ , J. Andre ’ , M. Dratwa ‘, M. Song ’ . ‘Department of Dermatology: 2Department Nephrology
CHU
Brugmann,
Brussels,
of
Belgium
Two cases of calcipbylaxis are presented. In the first case, a 57-year-old woman with chronic renal failure was admitted for ecchymotic, extremely painful and rapidly necrotic lesions of both tighs. Blood examination showed: urea 134 mgldl, creatinine 6.1 mg/dl, calcium 9.8 mg/dl, phosphorus 5.6 mg/dl, PTH 42.2 pglml (N: 10-55) and normal coagulation parameters. In second case, a 46-year-old man was admitted for liver failure on alcoolic cirrhosis and acute renal failure (hepatorenal syndrom) developing identical lesions. Blood examination
IP204
Posters
- Miscellaneous
Clinical features and treatment of Hand-Schuller-Christian disease
P206 El
Spina biflda - Association with increased lower limb naevi
I.M. Raznatovsky, I.A. Moshkalova, E.V. Sokolovski.
N. Flanagan, F. Enright, L. Barnes, R. Watson. Departments
Department University,
Dermatology, Our Lady’s and St. James’s Hospital,
of Dermatology St. Petersburg,
Ll? Pavlov Russia
State Medical
Hospitalfor Sick Children, Dublin, Ireland
of
Crumlin
8 patienst with Langerhans cell histiocytosis (formely known as histiocytosis X) seen in seven years are reviewed. The male/female incidence ratio was 1: 1. The presentation of the disease varied from age 3 to 76. In all patients the delayed diagnosis took place; the disease ran a chronic progressive course without spontaneous involution. All the patients had different types of skin manifestation. There were red-brown to reddish yellow papules on the scalp, the upper trunk, and in the intertriginous areas resembling Darier’s disease. Individual lesions developed features of scaling, crusting, spontaneous resolution with residual scarring. Other lesions included vesicopustules, lipid infiltration of the eyelids, and ulcerated nodes highly suggestive of scrofuloderma. Two women had ulcerated oral and genital mucosa. 2/8 remained purely cutaneous; whereas 6/8 had multiple organ involvement. 4/6 had marked pulmonary changes. Brain lesions occurred in 4/6 cases; their diabetes insipidus was controlled with vasopressin. Osteolytic lesions were present in 3/6: displaced teeth, loss of alveolar bone, sclerosis of pyramids. No one exhibited exophthalmos. In patients with pulmonary lesions therapy with Prospidin (Bis-Betachlor-aethylamin) coupled with mean doses of glucocorticosteroids proved to be highly efficient. The others received prospidin or cyclophosphan only (one was also irradiated). One patient was lost to follow-up; the remainder were either improved with treatment or well. Conclusions: The degree and site of Langerhans cell proliferation varies greatly from patient to patient. More similar cases are to be reported for better understanding and better management of the disease.
Evidence that melanocyte growth may be under neural control has previously been reported’. Three spina bifida (SB) patients were noted to have increased numbers of naevi on the lower limbs on routine dermatological consultation. The following study was performed to test the hypothesis that SB may be associated with increased numbers of melanocytic naevi on the lower limbs. We prospectively examined 42 patients with SB and 42 age and sex matched controls (24 female: 18 male, age range 3-19 years, mean 10.6 years) seen consecutively at Our Lady’s Hospital, Crumlin from January to March 1996. Participants underwent full skin examination noting the number, size, distribution and presence of clinical atypia of melanocytic naevi. When compared to the Vancouver Mole Study’ both males and females with spina bifida demonstrated increased mean number of naevi on the lower limbs and this difference was most marked in females aged 13 and over (18.4 Vs 5). When compared with Irish age and sex matched controls, SB patients had similar mean total number of naevi. In females with SB the mean number of naevi on the lower limbs was over double that seen in controls (8.4 vs 4.1) whereas in males with SB it was less than that seen in controls (5.2 Vs 6.2). Fourteen of 42 SB patients had naevi measuring over 5 mm in diameter compared with 4 controls (p < 0.05). Eight of 42 SB patients had congenital naevi (outside the paraspinal area) compared with 2 controls (p = 0.09). A small number of patients were fo.und to have clinically atypical naevi. This study supports an association between spina bifida and increased numbers of naevi on the lower limbs. The reasons for this association are unclear. Potential mechanisms include closely related candidate genes and/or a possible influence of neuronal, hormonal or metabolic factors on melanocyte growth.
I P205 Why is medical malpractice so high in
References
Ireland?
M.M. Young. Adelaide
Hospital,
Dublin,
Ireland
The high level of medical litigation is a reflection of a litiginous society. Consequently medical insurance fees are considerably higher than Britain. A comparison of attitudes in Ireland and the rest of Europe help to explain these findings. Unlike Britain, Ireland has a written constitution which guarantees individual rights which confer greater protection to an individual than those given by the European Court for the protection of human rights. In Ireland lawyers take cases on a ‘no foal, no fee’ basis, thus giving greater access to the courts. Reports from expert witnesses, such as medical practitioners, can be obtained without expense to the plaintiff. Judicial awards are higher than in other E.U countries, being on average 40% higher than in Britain and 70% higher in comparison to some E.U. countries and they continue to remain high despite the abolition of juries. The increasing trend of patients to question and influence medical treatment makes it likely that litigation will continue to rise.
[II Linear arrangement of multiple congenital melanocytic nevi.
Effendy I, Happle R. J Am Acad Dermatol 1992; 27: 8534. 121 Gallagher KP, McLean DI, Yang CP, Coldmau AJ, Silver HK Spinelli JJ, Beagrie M. Anatomic Distribution of Acquired Melanocytic Nevi in White Children. Arch Dermatol 1990; 126: 466-47 1.
1P207 1 Calciphylaxis P. Gheeraert ’ , A. Theunis ’ , J. Andre ’ , M. Dratwa ‘, M. Song ’ . ‘Department of Dermatology: 2Department Nephrology
CHU
Brugmann,
Brussels,
of
Belgium
Two cases of calcipbylaxis are presented. In the first case, a 57-year-old woman with chronic renal failure was admitted for ecchymotic, extremely painful and rapidly necrotic lesions of both tighs. Blood examination showed: urea 134 mgldl, creatinine 6.1 mg/dl, calcium 9.8 mg/dl, phosphorus 5.6 mg/dl, PTH 42.2 pglml (N: 10-55) and normal coagulation parameters. In second case, a 46-year-old man was admitted for liver failure on alcoolic cirrhosis and acute renal failure (hepatorenal syndrom) developing identical lesions. Blood examination