TY - JOUR T1 - The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. AU - Michaëlsson,G, AU - Olsson,E, AU - Westermark,P, PY - 1981/1/1/pubmed PY - 1981/1/1/medline PY - 1981/1/1/entrez SP - 497 EP - 503 JF - Acta dermato-venereologica JO - Acta Derm Venereol VL - 61 IS - 6 N2 - This hitherto unknown and dominantly inherited disorder is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. It has been present in the family reported for at least four generations. The lesions become visible in late childhood and are most pronounced on the face. Basal cell carcinomas may develop around the age of 35. Histological observations during the early stage include irregularly distributed and atrophic hair follicles, milia, dilated dermal vessels, lack of elastin or elastin in clumps. After light irradiation a tendency to increased repair activity was observed both in epidermis and in the dermal fibroblasts. On exposure to cold the change in the skin temperature of the hands compared with that in the controls was insignificant. The response to adrenalin iontophoresis was weak. SN - 0001-5555 UR - https://www.unboundmedicine.com/medline/citation/6177160/The_Rombo_syndrome:_a_familial_disorder_with_vermiculate_atrophoderma_milia_hypotrichosis_trichoepitheliomas_basal_cell_carcinomas_and_peripheral_vasodilation_with_cyanosis_ L2 - http://www.diseaseinfosearch.org/result/6358 DB - PRIME DP - Unbound Medicine ER -