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Annular erythema centrifugum successfully treated with metronidazole1
P165
P167
EXTENSIVE TUBEROUS XANTHOMA Maria GF Bussade, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Moˆ nica Zechmeister, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Juliana H Roquini, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Marcio S Rutowitsch, MD, PhD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil Xanthomas are a common presentation of disorders of lipid metabolism. Usually associated with abnormalities of cholesterol metabolism, combined with hyperlipidaemias or hypertriglyceridaemia. Tuberous xanthoma is associated, for example, with familiar dysbetalipoproteinaemia type III, combined with familiar hyperlipidaemia and familiar hypercholesterolaemia as well as in secondary hyperlipidaemias. Lesions vary in size and shape from small papules (0.5cm in diameter) to lobulated tumors (2.5cm in diameter) or more across. Usually they are painless, but the large lesions may be tender on direct pressure. They develop slowly and are seen on the pressure areas, such as on the extensor aspects of the limbs, particularly over knees, elbows and buttocks. Histology of the skin biopsies may show the presence of intracellular sudanophilic material in abnormal quantities, particularly spumcells around capillaries. Our patient is a 62 years old man, born in Rio de Janeiro-Brazil, who has been complaining about tuberous lesions for the last 10 years on elbows, knees and heels. Those lesions are painless, though aggregated, with glabrous surface and central hypochromia, vary in size from 1.0 cm to 3.5cm of diameter. There was a slowly development of the lesions, both in size and number. The patient shows dyslipoproteinaemia and high blood pressure without treatment for almost 8 years. He does not use any medicine normally. He has been smoking for the last 50 years. There is no familiar history of dyslipidaemia or another chronic disease. The latest exams showed total lipids ⫽ 2042mg/dl, total cholesterol ⫽ 519mg/dl, HDL ⫽ 50mg/dl, LDL ⫽ 320mg/dl and VLDL ⫽ 149mg/dl. The histopathology of one lesion located on his right elbow showed numerous foamcells (xanthomacells), confirming the clinical diagnostic of tuberous xanthoma associated with dysbetalipoproteinaemia type III. The patient was advised to have the largest lesions removed by surgical treatment.
ANNULAR ERYTHEMA CENTRIFUGUM SUCCESSFULLY TREATED WITH METRONIDAZOLE Lucio Andreassi, MD, Department of Dermatology, Siena, Italy, Massimiliano Risulo, MD, Department of Dermatology, Siena, Italy, Michele Fimiani, MD, Department of Dermatology, Siena, Italy, Giovambattista De Aloe, MD, Department of Dermatology, Siena, Italy A 38-year-old man was admitted to our Department with a 18-month history of a nonitching dermatosis characterized by the presence of erythematous plaques on trunk, upper and lower extremities, of different sizes, of anular shape with central resolution and tendency to centrifugal extension. Routinary laboratory tests revealed only a mild hypereosinofia; Borrelia Burgdorferi antibodies and mycological examinations were negative. A biopsy specimen from lesional skin revealed a perivascular and periannessial lymphocitic infiltrate in superficial and deep dermis while direct skin immunofluorescence study was negative. Chest X-ray and abdominal ultrasound were negative. Annular erythema centrifugum (EAC) was diagnosed and the patient was treated with systemic metronidazole (400 mg/die per os). The lesions resolved in 1 month. No recurrence of EAC was noted in a follow-up to 1 year. EAC is an uncommon inflammatory skin disease characterized by the presence of annular erythematous lesions with tendency to central resolution and centrifugal extension. Etiology is still unknown and EAC could represent a delayed hypersensibility reaction against drugs, foods, infections, neoplasms, autoimmune diseases. There is no effective treatment and all therapeutic trials are not standardized. To our knowledge, our case is the first successfully treated with oral metronidazole. Disclosure not available at press time.
Disclosure not available at press time.
P166 EFFICACY OF A NEXT-GENERATION, LIPID-BALANCED SKIN REPAIR CREAM FOR DRY SKIN AND INFLAMMATORY DERMATOSIS Steven D. Shapiro, MD, University of Miami School of Medicine, Miami, FL, United States Optimal skin barrier function is dependent, in part, on the absolute and relative proportions of lipids (ceramides, free fatty acids, and cholesterol) in the skin. Ceramides are an essential component of the skin lipid barrier, and comprise 45% of the stratum corneum lipid. Damage to the skin barrier can reduce ceramides and lipid content, leading to increased transepidermal water loss. Skin repair cream is a next-generation topical treatment that contains ceramide-3, cholesterol, and free fatty acids in a proprietary nanoparticle delivery technology. Skin repair cream, through the restoration of skin lipid balance, may be effective in rebuilding the damaged lipid barrier in patients with atopic dermatitis, allergic contact dermatitis, and irritant contact dermatitis. Indeed, studies have shown that balanced lipid mixtures are efficacious as monotherapy and in combination with topical steroids in improving skin barrier function in conditions such as contact dermatitis (Berardesca E, et al. Contact Dermatitis. 2001;45:280-285). These findings, along with the treatment-refractory nature of many dermatologic conditions, prompted the current clinical experience (case reports) with skin repair cream. In a pediatric patient who had received extensive prior therapy for atopic dermatitis, addition of skin repair cream to his current routine improved symptoms by 80% within 3 weeks. In a case involving a 48-year-old patient with keratoderma climactericum, treatment with skin repair cream resulted in impressive improvements in skin thickness at 2 weeks. Another patient with thickened skin on the soles of her feet (attributed to psoriasis) experienced marked improvement on treatment with skin repair cream and symptom flares on discontinuing treatment. In 3 patients with wrinkling beneath the eyes secondary to lax skin or blepharochalasis, treatment with skin repair cream reduced fine wrinkles at 4 weeks. Skin repair cream was well tolerated by all patients, with no complications reported. Additional case reports will be provided to supplement this clinical data, including before-and-after photographs. On the basis of early clinical experiences, the effectiveness and safety of this balanced lipid mixture warrant further evaluation. Received honoraria and travel expenses from Ferndale Laboratories for lectures and this poster. The product was supplied by Ferndale Laboratories at no cost to the doctor or patient. Did not receive funding for studying repair. Approximately 10% sponsored by Ferndale Laboratories Inc for poster production.
P42
J AM ACAD DERMATOL
P168 SEGMENTAL NEUROFIBROMATOSIS A.K.A. NEUROFIBROMATOSIS TYPE 5: ANTIQUATED TERMINOLOGY FOR A MOSAIC FORM OF NEUROFIBROMATOSIS? Roopal R Shanghvi, MD, SUNY Downstate Dermatology, Brooklyn, NY, United States, Karen Kim, MD, SUNY Downstate Dermatology, Brooklyn, NY, United States, Eve Lowenstein, MD, PhD, SUNY Downstate Dermatology, Brooklyn, NY, United States Segmental neurofibromatosis (NF), also known as NF type 5, has been attributed to a postzygotic somatic mutation of the NF1 gene, a process known as somatic mosaicism. We present a case of a 68 year old Jamaican male admitted to the hospital for hemoptysis. The dermatology service was called to evaluate asymptomatic left mid-facial lesions of over forty years duration, with the main concern being leprosy or cutaneous tuberculosis. The skin exam was otherwise unremarkable. Skin biopsy of one of the lesions was performed with histology consistent with neurofibroma. The patient denied a family history of neurofibromatosis. The diagnosis of segmental NF in the left facial V2 sensory distribution was made. In this poster, the definition, diagnosis, and prognosis of segmental NF are discussed. With studies demonstrating a common genetic background in both generalized and localized NF, literature in the genetics and neurosurgical fields have proposed the use of the terms “mosaic-generalized” and “mosaic-localized” to describe NF1 and segmental NF, respectively. These two entities arise at different stages of embryonic development from mutations in the same gene. This case is presented here to promote adopting this terminology in the dermatologic community, to support the concept that generalized NF1 and the previously termed NF5, or segmental NF, are likely variant expressions of the same disease. Disclosure not available at press time.
MARCH 2004
P167
EXTENSIVE TUBEROUS XANTHOMA Maria GF Bussade, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Moˆ nica Zechmeister, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Juliana H Roquini, MD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil, Marcio S Rutowitsch, MD, PhD, Hospital dos Servidores do Estado do Rio de Janeiro/MS, Rio de Janeiro, Brazil Xanthomas are a common presentation of disorders of lipid metabolism. Usually associated with abnormalities of cholesterol metabolism, combined with hyperlipidaemias or hypertriglyceridaemia. Tuberous xanthoma is associated, for example, with familiar dysbetalipoproteinaemia type III, combined with familiar hyperlipidaemia and familiar hypercholesterolaemia as well as in secondary hyperlipidaemias. Lesions vary in size and shape from small papules (0.5cm in diameter) to lobulated tumors (2.5cm in diameter) or more across. Usually they are painless, but the large lesions may be tender on direct pressure. They develop slowly and are seen on the pressure areas, such as on the extensor aspects of the limbs, particularly over knees, elbows and buttocks. Histology of the skin biopsies may show the presence of intracellular sudanophilic material in abnormal quantities, particularly spumcells around capillaries. Our patient is a 62 years old man, born in Rio de Janeiro-Brazil, who has been complaining about tuberous lesions for the last 10 years on elbows, knees and heels. Those lesions are painless, though aggregated, with glabrous surface and central hypochromia, vary in size from 1.0 cm to 3.5cm of diameter. There was a slowly development of the lesions, both in size and number. The patient shows dyslipoproteinaemia and high blood pressure without treatment for almost 8 years. He does not use any medicine normally. He has been smoking for the last 50 years. There is no familiar history of dyslipidaemia or another chronic disease. The latest exams showed total lipids ⫽ 2042mg/dl, total cholesterol ⫽ 519mg/dl, HDL ⫽ 50mg/dl, LDL ⫽ 320mg/dl and VLDL ⫽ 149mg/dl. The histopathology of one lesion located on his right elbow showed numerous foamcells (xanthomacells), confirming the clinical diagnostic of tuberous xanthoma associated with dysbetalipoproteinaemia type III. The patient was advised to have the largest lesions removed by surgical treatment.
ANNULAR ERYTHEMA CENTRIFUGUM SUCCESSFULLY TREATED WITH METRONIDAZOLE Lucio Andreassi, MD, Department of Dermatology, Siena, Italy, Massimiliano Risulo, MD, Department of Dermatology, Siena, Italy, Michele Fimiani, MD, Department of Dermatology, Siena, Italy, Giovambattista De Aloe, MD, Department of Dermatology, Siena, Italy A 38-year-old man was admitted to our Department with a 18-month history of a nonitching dermatosis characterized by the presence of erythematous plaques on trunk, upper and lower extremities, of different sizes, of anular shape with central resolution and tendency to centrifugal extension. Routinary laboratory tests revealed only a mild hypereosinofia; Borrelia Burgdorferi antibodies and mycological examinations were negative. A biopsy specimen from lesional skin revealed a perivascular and periannessial lymphocitic infiltrate in superficial and deep dermis while direct skin immunofluorescence study was negative. Chest X-ray and abdominal ultrasound were negative. Annular erythema centrifugum (EAC) was diagnosed and the patient was treated with systemic metronidazole (400 mg/die per os). The lesions resolved in 1 month. No recurrence of EAC was noted in a follow-up to 1 year. EAC is an uncommon inflammatory skin disease characterized by the presence of annular erythematous lesions with tendency to central resolution and centrifugal extension. Etiology is still unknown and EAC could represent a delayed hypersensibility reaction against drugs, foods, infections, neoplasms, autoimmune diseases. There is no effective treatment and all therapeutic trials are not standardized. To our knowledge, our case is the first successfully treated with oral metronidazole. Disclosure not available at press time.
Disclosure not available at press time.
P166 EFFICACY OF A NEXT-GENERATION, LIPID-BALANCED SKIN REPAIR CREAM FOR DRY SKIN AND INFLAMMATORY DERMATOSIS Steven D. Shapiro, MD, University of Miami School of Medicine, Miami, FL, United States Optimal skin barrier function is dependent, in part, on the absolute and relative proportions of lipids (ceramides, free fatty acids, and cholesterol) in the skin. Ceramides are an essential component of the skin lipid barrier, and comprise 45% of the stratum corneum lipid. Damage to the skin barrier can reduce ceramides and lipid content, leading to increased transepidermal water loss. Skin repair cream is a next-generation topical treatment that contains ceramide-3, cholesterol, and free fatty acids in a proprietary nanoparticle delivery technology. Skin repair cream, through the restoration of skin lipid balance, may be effective in rebuilding the damaged lipid barrier in patients with atopic dermatitis, allergic contact dermatitis, and irritant contact dermatitis. Indeed, studies have shown that balanced lipid mixtures are efficacious as monotherapy and in combination with topical steroids in improving skin barrier function in conditions such as contact dermatitis (Berardesca E, et al. Contact Dermatitis. 2001;45:280-285). These findings, along with the treatment-refractory nature of many dermatologic conditions, prompted the current clinical experience (case reports) with skin repair cream. In a pediatric patient who had received extensive prior therapy for atopic dermatitis, addition of skin repair cream to his current routine improved symptoms by 80% within 3 weeks. In a case involving a 48-year-old patient with keratoderma climactericum, treatment with skin repair cream resulted in impressive improvements in skin thickness at 2 weeks. Another patient with thickened skin on the soles of her feet (attributed to psoriasis) experienced marked improvement on treatment with skin repair cream and symptom flares on discontinuing treatment. In 3 patients with wrinkling beneath the eyes secondary to lax skin or blepharochalasis, treatment with skin repair cream reduced fine wrinkles at 4 weeks. Skin repair cream was well tolerated by all patients, with no complications reported. Additional case reports will be provided to supplement this clinical data, including before-and-after photographs. On the basis of early clinical experiences, the effectiveness and safety of this balanced lipid mixture warrant further evaluation. Received honoraria and travel expenses from Ferndale Laboratories for lectures and this poster. The product was supplied by Ferndale Laboratories at no cost to the doctor or patient. Did not receive funding for studying repair. Approximately 10% sponsored by Ferndale Laboratories Inc for poster production.
P42
J AM ACAD DERMATOL
P168 SEGMENTAL NEUROFIBROMATOSIS A.K.A. NEUROFIBROMATOSIS TYPE 5: ANTIQUATED TERMINOLOGY FOR A MOSAIC FORM OF NEUROFIBROMATOSIS? Roopal R Shanghvi, MD, SUNY Downstate Dermatology, Brooklyn, NY, United States, Karen Kim, MD, SUNY Downstate Dermatology, Brooklyn, NY, United States, Eve Lowenstein, MD, PhD, SUNY Downstate Dermatology, Brooklyn, NY, United States Segmental neurofibromatosis (NF), also known as NF type 5, has been attributed to a postzygotic somatic mutation of the NF1 gene, a process known as somatic mosaicism. We present a case of a 68 year old Jamaican male admitted to the hospital for hemoptysis. The dermatology service was called to evaluate asymptomatic left mid-facial lesions of over forty years duration, with the main concern being leprosy or cutaneous tuberculosis. The skin exam was otherwise unremarkable. Skin biopsy of one of the lesions was performed with histology consistent with neurofibroma. The patient denied a family history of neurofibromatosis. The diagnosis of segmental NF in the left facial V2 sensory distribution was made. In this poster, the definition, diagnosis, and prognosis of segmental NF are discussed. With studies demonstrating a common genetic background in both generalized and localized NF, literature in the genetics and neurosurgical fields have proposed the use of the terms “mosaic-generalized” and “mosaic-localized” to describe NF1 and segmental NF, respectively. These two entities arise at different stages of embryonic development from mutations in the same gene. This case is presented here to promote adopting this terminology in the dermatologic community, to support the concept that generalized NF1 and the previously termed NF5, or segmental NF, are likely variant expressions of the same disease. Disclosure not available at press time.
MARCH 2004