At a meeting of the Christiania Medical Society, April 24, 1889, Dr. August Koren read a paper with the above title, which is published in the Norsk Magazin for Laegevidenskaben of September, 1889. He said ichthyosis, as is well known, is, as a rule, a diffuse disease, generally spreading from the extensor side of the extremities over the whole body, with the exception of the face, scalp, flexor surfaces of joints, palmar and plantar areas, and the genitalia, beginning usually in the first or second year of life and reaching its height about the age of puberty, when, in spite of all treatment, it remains stationary, except that toward the close of summer the skin may often become more soft and moist for a time, soon returning, however, to its previous condition. The disorder does not affect the general health of the patient. The forms described as serpentina, cornea, and hystrix are merely the various developmental stages of the disease. General diffuse ichthyosis is never, according to the authors, congenital, but appears at the earliest in the second month of life. But, in his own experience in the skin department of the Rigshospital, he had seen a case of normally developed khthyosis serpentina that was congenital, and a similar case had also previously been under treatment at the same hospital. . . .
An observation of Dr. R. Hubert (Virchow's Archiv, vol. xcix, page 569) in a 24-year-old girl in Bremerhaven is of interest here. There was hypertrichosis of the whole left arm, with a small ichthyosis serpentina in the scapular region about an inch in diameter. (Her mother had seen an ape during pregnancy.)
Dr. Koren presented drawings of a noteworthy case of his own. . . .It is an ichthyosis cornea developed in the form of brownish papillomatous stripes with normal skin lying between them. . . .
The case impressed the author, as soon as he saw it, as one of naevus, but, as naevi undergo no change or improvement such as had taken place in this disease, he was led to think of ichthyosis; and closer observation of the strongly developed epidermal layer, which felt like a grater on stroking it with the hand, assured him of the correctness of his diagnosis. At the same time there was a remarkable resemblance to a rare form of naevus, which, because of its asymmetrical appearance upon one side of the body, has been termed by von Bärensprung naevus unius lateris, and because of its following branches of nerves has been called by Gerhardt papilloma neuropathicum, but is more familiar under the name now in general use given by Simon, naevus nervosus. Such naevi are uncommon, but an ichthyosis developing so exquisitely along nerve branches, as in this case, is still more rare. . . .
These forms of ichthyosis and naevus are so similiar in pathologicoanatomical respects that it is not to be wondered at that there are transitional forms which might be considered either the one or the other, according to one's taste. . . .
J Cutan Genitourin Dis.
PETERSON F. ICHTHYOSIS LINEARIS NEUROPATHICA. Arch Dermatol. 1990;126(2):162. doi:10.1001/archderm.1990.01670260032004
Customize your JAMA Network experience by selecting one or more topics from the list below.