Ichthyosis Hystrix (Curth-Macklin): Title and subTitle BreakLight and Electron Microscopic Studies Performed Before and After Etretinate Treatment

Lasse Kanerva, MD; Jaakko Karvonen, MD; Aarne Oikarinen, MD; Jorma Lauharanta, MD; Aimo Ruokonen, MD; Kirsti-Maria Niemi, MD

[+] Author Affiliations

Accepted for publication Oct 28, 1983.

Reprint requests to the Institute of Occupational Health, Haartmaninkatu 1, 00290 29 Helsinki, Finland (Dr Kanerva).

From the Institute of Occupational Health (Dr Kanerva) and the Department of Dermatology, University Central Hospial (Drs Kanerva, Lauharanta, and Niemi), Helsinki; the Department of Dermatology, University Central Hospital, Oulu, Finland (Drs Karvonen and Oikarinen); and the Department of Clinical Chemistry, University of Oulu (Dr Ruokonen).

Arch Dermatol. 1984;120(9):1218-1223. doi:10.1001/archderm.1984.01650450100030

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ABSTRACT

ABSTRACT | REFERENCES

• Clinical and light and electron microscopic observations of a 16-year-old male patient suffering from ichthyosis hystrix (Curth-Macklin) are presented. The patient had no family history for this disease. The diagnosis was based on the distinct electron microscopic finding of continuous perinuclear tonofibril shells in the keratinocytes. About 10% of the keratinocytes were binucleate and one third contained conspicuous vacuoles. The steroid sulphatase activity in a skin biopsy was normal. Etretinate treatment proved beneficial during the first year of therapy. Later the treatment was less effective. The basic genetic defect persisted in the phenotype of the keratinocytes during etretinate therapy, but the exceedingly thick horny layer was considerably thinned.

(Arch Dermatol 1984;120:1218-1223)

REFERENCES

ABSTRACT | REFERENCES

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Kanerva L, Lauharanta J, Niemi K-M, et al: Ultrastructure of pityriasis rubra pilaris with observations during retinoid (etretinate) treatment . Br J Dermatol 1983;;108:653-663.

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Kanerva L, Lauharanta J, Niemi K-M, et al: Fine structure of the intercellular space of psoriatic epidermis during retinoid (Ro 10-9359) and retinoid-PUVA treatment . Acta Derm Venereol 1981;;61:319-328.

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