Pigmented malignant hidroacanthoma simplex mimicking irritated seborrheic keratosis

Pigmented variant of malignant hidroacanthoma simplex (PMHS) is very rare. We are aware of only two reported cases, all arising in pigmented hidroacanthoma simplex (HS). We report the third case of PMHS arising in a pigmented HS. A 71-year-old-woman presented with a well-demarcated pigmented hyperkeratotic tumor on the right knee resembling irritated seborrheic keratosis. Histopathologic examination of the excised tumor revealed intraepidermal proliferation of atypical polygonal poroid cells forming large, sharply demarcated nests with colonization of dendritic melanocytes. In addition, there were focal changes of a benign pigmented HS and syringofibroadenoma. The key diagnostic features of ductal structures and intracytoplasmic lumina were highlighted by carcinoembryonic antigen and epithelial membrane antigen immunostaining. PMHS should be differentiated from irritated seborrheic keratosis, melanoacanthoma, Bowen's disease and malignant melanoma both clinically and pathologically.