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March 1994

Inherited Extensive Speckled Lentiginous Nevus With Ichthyosis: Report of a Previously Undescribed Association

Author Affiliations

Clinica Dermatologica IV Ospedale San Paolo Via di Rudini 8, 20142, Milano Italy

Arch Dermatol. 1994;130(3):393-395. doi:10.1001/archderm.1994.01690030129028

Linear or zosteriform patterns of hyperpigmentation often involving large areas of the body have been described infrequently. There is confusion about the nomenclature of speckled pigmented lesions. One of these, speckled lentiginous nevus (SLN), is a specific1 acquired lesion, although congenital cases have been reported. We have observed the case of a previously unreported inherited giant SLN in which an extensive pattern of pigmentation is combined with ichthyosis. Both skin conditions were first present in the patient's grandfather.

Report of a Case.  A 16-year-old white boy came to us for evaluation of widespread pigmented cutaneous lesions that dated back to birth and extended progressively. Physical examination revealed numerous darkly pigmented macules and papules not invariably located on a background of tan to light-brown macular hyperpigmentation. The lesions were extensively distributed on the face and trunk (Figure 1), with the exception of some areas that were spared. The tan patches were