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Pagetoid Dyskeratosis of the Scrotum: Histologic Findings Resembling Extramammary Paget's Disease

Lee, Hyung Rae MD; Han, Tae Young MD; Lee, June Hyunkyung MD; Son, Sook-ja MD

The American Journal of Dermatopathology: October 2011 - Volume 33 - Issue 7 - p 755-757
doi: 10.1097/DAD.0b013e31820a264f
Letters to the Editor

Department of Dermatology, College of Medicine, Eulji University, Seoul, Korea

The authors declare no conflicts of interest.

To the Editor:

A 54-year-old man with pruritic symptoms involving the scrotum of 10-year duration presented for evaluation. The patient intermittently applied topical steroids prescribed at local clinics. The personal and family histories were noncontributory. The physical examination demonstrated ill-defined mild erythematous and skin-colored patches with focal hyperpigmentation on the scrotum (Fig. 1). The laboratory findings were unremarkable. A potassium hydroxide preparation was negative. The histologic examination shows that multiple intraepidermal pale cells resembling Paget disease were present singly or in groups. The lesion consisted of clusters of large and round pagetoid cells characterized by centrally condensed pyknotic nuclei with perinuclear clear halos (Fig. 2). These pale cells showed premature keratinization. Thus, the cells were characterized by a lack of acantholysis and keratohyalin granules. These cells did not show mucin deposits by periodic acid-Schiff staining. Immunohistochemical stains for cytokeratin 7 and cytokeratin 20 in pagetoid cells were negative. Based on these characteristic histologic features, the patient was diagnosed with pagetoid dyskeratosis. Pagetoid dyskeratosis is defined as the presence of pale cells resembling those of Paget disease in the epidermis. Pagetoid cells have a similar pagetoid appearance and intraepidermal spread of epithelial, melanocytic, neuroendocrine, lymphoid, and histiocytic lineage.1,2 Pagetoid dyskeratosis is an incidental finding in a variety of skin lesions, such as nevi, achrocordons, lentigos, fibrous papules, seborrheic keratosis, lichen simplex chronicus, hypertrophic scars, and milia.1,3 Pagetoid dyskeratosis has been observed in intertriginous areas, trunk, buttocks, face, and extremities. Other anatomic sites, such as the lips, hemorrhoids, cervix in uterine prolapse, and hands have been reported.3-6 A common feature of the anatomic prevalence sites is exposure to trauma and friction. The presence of dyskeratotic cells in these areas has led some authors to estimate that the cause of this process may be induced by friction.7 Pagetoid dyskeratosis cells must be differentiated from artifactual clear cells of the squamous epithelium, glycogen-rich cells, koilocytes, extramammary Paget cells, and pagetoid spread of carcinoma cells.3 However, it is important to distinguish pagetoid dyskeratosis from extramammary Paget disease in the scrotal area. Pagetoid dyskeratosis cells show large cells with central pyknotic nuclei and a mucin-negative cytoplasm, instead of the large atypical nuclei and cytoplasmic mucin characteristic of the cells existing in patients with extramammary Paget disease.1,3 Special stains are helpful in establishing the diagnosis of pagetoid dyskeratosis, but routine histologic examination is usually sufficient. Our case also had negative findings on periodic acid-Schiff staining. In conclusion, pale cells resembling extramammary Paget disease can be seen in otherwise normal skin of the scrotum. The anatomic site and characteristic pale cells together lead to the possibility of overdiagnosing the disease. Therefore, a careful histologic examination is important when pale cells are noted in the scrotal area.





Hyung Rae Lee, MD

Tae Young Han, MD

June Hyunkyung Lee, MD

Sook-ja Son, MD

Department of Dermatology, College of Medicine, Eulji University, Seoul, Korea

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1. Val-Bernal JF, Garijo MF. Pagetoid dyskeratosis of the prepuce. An incidental histologic finding resembling extramammary Paget's disease. J Cutan Pathol. 2000;27:387-391.
2. Garjio MF, Val D, Val-Bernal JF. Pagetoid dyskeratosis of the nipple epidermis: an incidental finding mimicking Paget's disease of the nipple. APMIS. 2008;116:139-146.
3. Val-Bernal JF, Pinto J, Garijo MF, et al. Pagetoid dyskeratosis of the cervix. Am J Surg Pathol. 2000;24:1518-1523.
4. Val-Bernal JF, Pinto J. Pagetoid dyskeratosis is a frequent incidental findings in hemorrhoidal disease. Arch Pathol Lab Med. 2001;125:1058-1062.
5. Garijo MF, Val D, Val-Bernal JF. Pagetoid dyskeratosis of the lips. Am J Dermatopathol. 2001;23:329-333.
6. Wang LC, Medenica MM, Shea CR, et al. Pagetoid dyskeratosis of the hand. J Am Acad Dermatol. 2004;50:483-484.
7. Tschen JA, McGavran MH, Kettler AH. Pagetoid dyskeratosis: a selective keratinocytic response. J Am Acad Dermatol. 1988;19:891-894.
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