Olmsted syndrome is a rare congenital disorder with mutilating palmoplantar keratoderma, periorificial keratotic plaques, and other variable features.
Objective:
We describe a 65-year-old woman with Olmsted syndrome complicated by the occurrence of a malignant melanoma inside the plantar keratoderma. To our knowledge, this is the first reported case of such an occurrence in Olmsted syndrome. The published cases of this rare disorder are reviewed.
Conclusion:
An association between malignant epithelial tumors and Olmsted syndrome has already been reported. The association of malignant melanoma with other types of palmoplantar keratodermas has been reported. This may suggest a predisposition to melanocytic as well as squamous cell malignancies in congenital keratodermas. Oral retinoids appear to be the most promising treatment for Olmsted syndrome and for other symptomatic keratodermas.
Get full access to this article
View all access options for this article.
References
1.
OlmstedHC. Keratodermia palmaris et plantaris congenitalis. Report of a case showing associated lesions of unusual location. Am J Dis Child1927; 33:758–764.
KeirM. Keratodermia palmaris et plantaris. J R Soc Med1967; 79:419–421.
4.
Ruiz-MaldonadoRLozano-FerralN. Hiperqueratosis palmoplantar mutilante con hipotriquia y conlesions eritematoescamosas inguinales y erianales. Int J Dermatol1972; 11:31–35.
5.
MichalowskiR. Erythrokeratodermia periorificialis mit Akrenbeteiligung. Hautarzt1983; 34:465–467.
6.
PoulinYPerryHOMullerSA. Olmsted syndrome—congenital palmoplantar and periorificial keratoderma. J Am Acad Dermatol1984; 10:600–610.
7.
HarmsMSauratJ-HBerguesJP. Syndrome de Olmsted (keratodermie palmoplantaire et periorificielle congenitale). Ann Dermatol Venereol1985; 112:479.
8.
GeorgiiAPrzybillaBSchmoeckelC. Olmsted syndrome Assoziation mit primar Sklerosierender Cholangitis und Immuschwach unklarer Genese. Hautarzt1989; 40:708–712.
9.
BattiniMLMorettiSUzielliML Giovannuci. Olmsted syndrome: A case associated with severe corneal opacities. In CaputoRGelmettiC, eds. Proceedings of the 5th International of Pediatrics Dermatology, Milan Italy, July 11–15 1989.
YamamotoTAzukizawaHTadokoroT. A case of Olmsted syndrome associated with a plantar verrucous carcinoma. Jpn J Dermatol1999; 109:933.
23.
OgawaFUdonoMKatayamaI. A case of Olmsted syndrome associated with squamous cell carcinoma. Jpn J Dermatol2001; 110:659.
24.
RequenaLManzarbeitiaFMorenoC. Olmsted syndrome: Report of a case with study of the cellular proliferation in keratoderma. Am J Dermatopathol2001; 23:514–520.
25.
BarnettJHEstesSA. Multiple epithelioma ta cuniculata occurring in a multilating keratoderma. Cutis1985; 35:345–347.
26.
YoshizakiYKankiHUedaT. A further case of plantar squamous cell carcinoma arising in Olmsted syndrome. Br J Dermatol2001; 145:685–686.
27.
PerryOHSuWPD. Olmsted syndrome. Sem Dermatol1995; 14:145–151.
28.
GoldsmithLAEpsteinEHJr. Genetics in relation to the skin. In: FreedbergIMEisenAZWolffKAustenKFGoldsmithSIKatzSIFitzpatrickTB, eds. Fitzpatrick's dermatology in general medicine, 5th ed.New York: McGraw–Hill, 1999, pp 59–69.
29.
StevensHPLeighIM. The inherited keratodermas of palms and soles. In: FreedbergIMEisenAZWolffKAustenKFGoldsmithLAKatzSIFitzpatrickTB, eds. Fitzpatrick's dermatology in general medicine, 5th ed.New York: McGraw–Hill, 1999, pp 603–613.
30.
Caceres-RiosHTamayo-SanchezLDuran-McKinsterC. KID syndrome: Review of the literature and proposal of a new terminology. Pediatric Dermatol1996; 13:105–113.
31.
NguyenEQWolvertonSE. Systemic retinoids. In: WolvertonSE, ed. Comprehensive Dermatologic Drug Therapy. Philadelphia: W.B. Saunders, 2001, p 276.
32.
SeikeTNakanishiHUranoY. Malignant melanoma developing in an area of palmoplantar keratoderma (Greither's disease). J Dermatol1995; 22:55–61.
33.
Hacham-ZadehSGoldbergL. Malignant melanoma and Papillon–Lefevre syndrome. Arch Dermatol1982; 118:2.
