Perforating granuloma annulare [proceedings].
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Biomedical subjects
Publications and source records attributed to S C van der Putte.
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A histopathological investigation was performed in newborn pigs with congenital hereditary lymphedema. The edema varied greatly in degree and extension. Although mostly confined to the hind legs it may involve other parts of the body, and even attain a most severe generalized distribution. It was found due to malformations of the lymphatic system which were general in character in all animals irrespective the grade in their edema. Parallel to the grade of edema however, these lymphvascular abnormalities varied from minor hypoplasia and localized aplasia to a complete agenesis of the whole lymphatic system.
The morphogenesis of congenital hereditary lymphedema was studied in pigs. The disorder, which is essentially a general underdevelopment or even total non-development of the lymphatic system proved to be present during the whole period of lymphatic development. It is suggested that a retardation in the differentiation of the lymphatic primordia from the primitive veins is the early event regulated by a chromosomal aberration. The longer this delay the more serious the lymphatic malformations.
In 7 spontaneously aborted fetuses characterized by a large cystic hygroma in the nuchal region and a prominent and generalized edema, the structure and extension of the lymphatic system was studied. In all fetuses marked malformations of this system were found. Although the morphologic appearance seemed to vary greatly it is suggested that the disorder is essentially a generalized hypoplasia and partial agenesis of the lymphatic system, which ceases to extend peripherally at an early embryonic stage. A suggestion as to the mechanism of this growth inhibition is made.