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P231 Membranous Aplasia Cutis with hair collars. A case report
S206
Posters -
compound of chilli peper, is known to have a rather selective action on type C sensory neurons and is a potent antagonist of substance P (SP). Capsaicin has been proven to first induce the neuronal release of SP and then to block further synthesis and transport of the transmitter within the neuron. Capsaicin has been clinically used in the treatment of chronic inflammatory skin disorders such as post-herpetic neuralgia, prurigo nodularis, localized pruritus, contact dermatitis, cold m&aria, notalgia paresthetica, diabetic neuropathy, Raynaud’s phenomenon. SP also acts as a growth modulator. It may be involved in hyperproliferative diseases like psoriasis and atopic dermatitis and may play a role in growth control of the hair follicle during the anagen phase. Capsaicin may induce the transition between anagen and catagen stage. Civamide, a novel isomer of capsaicin with greater depleting properties and greater analgesic potency than capsaicin, may be a promising new treatment for dermatological disorders mediated by endogenous neuropeptides. Other neuropeptide antagonists are currently tested in vitro such as capsazepin and spantide. Botulinum A exotoxin, blocks the presynaptic release of various neurotransmitters and prevents the release of acetylcholine. Very low amounts of this toxin, given subcutaneously, appeared to be a promising new drug for the treatment of axillary and palmar hyperhidrosis and in the therapy of gustatory sweating. In the near future, neuropeptides will undoubtly represent a new approach to skin therapy.
Paediatric P229 Flothmund-Thomson n study
Aljredo
da Matta,
Manaus,
Amazonas,
Brazil
ElP230 Neurofibromatosis
with neuritis: A differential diagnosis with leprosy
P.F.B. Rebello, S.N. Pennini,T.C. Tropical Brazil
ElP231
Membranous Aplasia Cutis with hair collars. A case report
A. Valcayo, R. Vives, J. Elso ’ , T. TuMn 2. Departments Dermatology; Pamplona.
’ Paediatrics; Spain
2Pathology
Hospital
of de Navarra,
Aplasia Cutis Congenita (ACC) is a rare disorder characterized by localized absence of skin at birth. There is not a single underlying cause. Drolet et al described in 1995 the “membranous” variant of ACC. This form appears in the perinatal period as a translucent, soft, atrophic scar that characteristically is surrounded by anomalous hair pattern in form of “hair collar”. We describe a newborn who presented with three congenital scalp defects showing thin, glistening, bullous-like patches encircled by a ring of dark long hair. Biopsy findings resembled the stroma of meningoceles, but special stains failed to identify meningeal or cerebral tissue. A clinical and histologic diagnosis of aplasia cutis is important in order to distinguish their variants and to improve our understanding of human embryogenesis.
IP232
The authors present a case study of Rothmund Thomson Syndrome in an 11 year old female patient from Manaus, Amazonas. When she was 6 months old pruritic, macular, hypo and hyperchromic reticular disseminated lesions appeared. She presented with discrete alopecia of her scalp, acral verrucous keratoses on her bilateral plantar aspects and hypoplasia of her thrumb. There were no alterations of her oral mucosa, nails, teeth or menstrual alterations. Although the parents were consanguineous they denied any knowledge of similar cases within the family.
Institute de Dermatologia Matta, Manaus, Amazonas,
his whole body. During 1996 he suffered from episodes of pain and bilateral thickening of the following nerve trunks: auricular, ulnar, radial, common peroneal and posterior tibial. There was no evidence of hypoanesthesic areas nor amiotrophy. Biopsies of the perineurium and of some skin lesions were taken. Histopathological examination only showed alterations indicating neurofibromatosis. The authors discuss the importance of the differential diagnosis between neurofibromatosis and leprosy especially if it occurs within an endemic area.
Syndrome - Case
I.S. Souza, R.F. Nazareth,R.C. Silva, C.B.R. Ribas, T.C. Guimaraes. Institute de Dermatologia Tropical e Venereologia
Paediatric
Guimaraes, L.S. Souza. e Venereologia
A&redo
da
The authors describe a rare case of a 30-year-old man who presents neurofibromatosis type I and peripheral neuritis as part of his pathology. From 16 years of age he presented numerous painless pseudo-herniated nodules and “cafe-au-lait” macules affecting
Erythema elevatum diutinum associated with liver transplant
N. Hemandez-Cano, R. De Lucas, M. Sigtienza, R. Diaz-Diaz, J.A. Garcia-Jimenez, M. Casado. LQ Paz Hospital, Madrid, Spain
Introduction: Erythema elevatum diutinum (EED), is a rare chronic cutaneous disease characterized by persistent red-purple and yellowish papules, plaques and nodules, that are symmetrically distributed primarily on the extensor surfaces, with a preference for joint regions. This entity is seen most frequently in middle-aged adults and ocassionally also in children. Case Report: We report a lo-year-old boy with a medical history of liver transplant four years ago, because of Alagille disease. Since then, he received treatment with CyclosporineA (CyA) and Prednisone. Approximately 5 months before our examination he noted the development of red papules over the dorsum of hands and fingers. Histologic examination showed leukocytoclastic, angioplastic vasculitus. A diagnosis of EED was made. The lesions remained unchanged during the next six months when they began the involution. This fact was simultaneous with the reduction of CyA doses. Discussion: The cause of EED, is most likely an immune complex-induced vasculitis. Arthralgias, recurrent streptococcal infections, hematologic diseases, Crohn’s disease, pyoderma gangrenosum and IIIV infection are associated with EED. To our knowledge, the association with liver transplant has not
Posters -
compound of chilli peper, is known to have a rather selective action on type C sensory neurons and is a potent antagonist of substance P (SP). Capsaicin has been proven to first induce the neuronal release of SP and then to block further synthesis and transport of the transmitter within the neuron. Capsaicin has been clinically used in the treatment of chronic inflammatory skin disorders such as post-herpetic neuralgia, prurigo nodularis, localized pruritus, contact dermatitis, cold m&aria, notalgia paresthetica, diabetic neuropathy, Raynaud’s phenomenon. SP also acts as a growth modulator. It may be involved in hyperproliferative diseases like psoriasis and atopic dermatitis and may play a role in growth control of the hair follicle during the anagen phase. Capsaicin may induce the transition between anagen and catagen stage. Civamide, a novel isomer of capsaicin with greater depleting properties and greater analgesic potency than capsaicin, may be a promising new treatment for dermatological disorders mediated by endogenous neuropeptides. Other neuropeptide antagonists are currently tested in vitro such as capsazepin and spantide. Botulinum A exotoxin, blocks the presynaptic release of various neurotransmitters and prevents the release of acetylcholine. Very low amounts of this toxin, given subcutaneously, appeared to be a promising new drug for the treatment of axillary and palmar hyperhidrosis and in the therapy of gustatory sweating. In the near future, neuropeptides will undoubtly represent a new approach to skin therapy.
Paediatric P229 Flothmund-Thomson n study
Aljredo
da Matta,
Manaus,
Amazonas,
Brazil
ElP230 Neurofibromatosis
with neuritis: A differential diagnosis with leprosy
P.F.B. Rebello, S.N. Pennini,T.C. Tropical Brazil
ElP231
Membranous Aplasia Cutis with hair collars. A case report
A. Valcayo, R. Vives, J. Elso ’ , T. TuMn 2. Departments Dermatology; Pamplona.
’ Paediatrics; Spain
2Pathology
Hospital
of de Navarra,
Aplasia Cutis Congenita (ACC) is a rare disorder characterized by localized absence of skin at birth. There is not a single underlying cause. Drolet et al described in 1995 the “membranous” variant of ACC. This form appears in the perinatal period as a translucent, soft, atrophic scar that characteristically is surrounded by anomalous hair pattern in form of “hair collar”. We describe a newborn who presented with three congenital scalp defects showing thin, glistening, bullous-like patches encircled by a ring of dark long hair. Biopsy findings resembled the stroma of meningoceles, but special stains failed to identify meningeal or cerebral tissue. A clinical and histologic diagnosis of aplasia cutis is important in order to distinguish their variants and to improve our understanding of human embryogenesis.
IP232
The authors present a case study of Rothmund Thomson Syndrome in an 11 year old female patient from Manaus, Amazonas. When she was 6 months old pruritic, macular, hypo and hyperchromic reticular disseminated lesions appeared. She presented with discrete alopecia of her scalp, acral verrucous keratoses on her bilateral plantar aspects and hypoplasia of her thrumb. There were no alterations of her oral mucosa, nails, teeth or menstrual alterations. Although the parents were consanguineous they denied any knowledge of similar cases within the family.
Institute de Dermatologia Matta, Manaus, Amazonas,
his whole body. During 1996 he suffered from episodes of pain and bilateral thickening of the following nerve trunks: auricular, ulnar, radial, common peroneal and posterior tibial. There was no evidence of hypoanesthesic areas nor amiotrophy. Biopsies of the perineurium and of some skin lesions were taken. Histopathological examination only showed alterations indicating neurofibromatosis. The authors discuss the importance of the differential diagnosis between neurofibromatosis and leprosy especially if it occurs within an endemic area.
Syndrome - Case
I.S. Souza, R.F. Nazareth,R.C. Silva, C.B.R. Ribas, T.C. Guimaraes. Institute de Dermatologia Tropical e Venereologia
Paediatric
Guimaraes, L.S. Souza. e Venereologia
A&redo
da
The authors describe a rare case of a 30-year-old man who presents neurofibromatosis type I and peripheral neuritis as part of his pathology. From 16 years of age he presented numerous painless pseudo-herniated nodules and “cafe-au-lait” macules affecting
Erythema elevatum diutinum associated with liver transplant
N. Hemandez-Cano, R. De Lucas, M. Sigtienza, R. Diaz-Diaz, J.A. Garcia-Jimenez, M. Casado. LQ Paz Hospital, Madrid, Spain
Introduction: Erythema elevatum diutinum (EED), is a rare chronic cutaneous disease characterized by persistent red-purple and yellowish papules, plaques and nodules, that are symmetrically distributed primarily on the extensor surfaces, with a preference for joint regions. This entity is seen most frequently in middle-aged adults and ocassionally also in children. Case Report: We report a lo-year-old boy with a medical history of liver transplant four years ago, because of Alagille disease. Since then, he received treatment with CyclosporineA (CyA) and Prednisone. Approximately 5 months before our examination he noted the development of red papules over the dorsum of hands and fingers. Histologic examination showed leukocytoclastic, angioplastic vasculitus. A diagnosis of EED was made. The lesions remained unchanged during the next six months when they began the involution. This fact was simultaneous with the reduction of CyA doses. Discussion: The cause of EED, is most likely an immune complex-induced vasculitis. Arthralgias, recurrent streptococcal infections, hematologic diseases, Crohn’s disease, pyoderma gangrenosum and IIIV infection are associated with EED. To our knowledge, the association with liver transplant has not