Secondary extramammary Paget's disease with underlying recurrent bladder carcinoma

ARTICLE

ejd.2011.1556

Auteur(s) : Yumi Nonomura¹, Miki Tanioka¹, Yoko Mitomi², Akihiro Fujisawa¹ fujiaki@kuhp.kyoto-u.ac.jp, Yoshiki Miyachi¹

¹ Department of Dermatology

² Department of Nursing, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo Kyoto 606-8507, Japan

Secondary extramammary Paget's disease is a pagetoid spread from gastrointestinal or urological carcinoma to the skin, which is a rare phenomenon that mimics dermatitis or primary Paget's disease [1]. Here, we report a case of secondary extramammary Paget's disease from bladder carcinoma in peri-urostomal skin, 9 years after total cystectomy and cutaneous ureterostomy.

An 83-year-old Japanese man presented with intractable erythema and erosion around a urostoma. Nine years before his visit to our department, he underwent on a total cystectomy and bilateral cutaneous ureterostomy for bladder cancer. Pathological examination revealed minimal infiltration of the stump of right ureter. One year after the surgery, follow-up urine cytology demonstrated malignant cells and BCG injection was undertaken. After that, recurrence was not found by urine cytology or regular CT scans for more than 8 years. One year before his first consultation with dermatologists, erythema developed around the right but not around the left urostroma. It was refractory to topical steroids, anti-fungal or antibiotic drugs. Changing the types of stoma did not work, either. It was a round erythema precisely corresponding to a covering material over the right stoma (figure 1A, B). A skin biopsy from the peripheral border of the erythema revealed Paget's cells in the epidermis (figure 1C). Immunostaining showed that the tumor cells were consistent with pagetoid spread from bladder carcinoma, and were positive for CEA, CAM5.2, CK7 and CK20, while negative for S100 and GCDFP15 (data not shown) [1, 2]. We diagnosed it as secondary extramammary Paget's disease spread from urethral invasion of bladder carcinoma by way of the urostoma. After the diagnosis, repeated examinations of urine cytology detected malignant cells in the right ureter, although no tumor mass was detected in the abdomen by a CT scan.

According to Lyon, 73% patients report peristomal skin problems [3]. Common ones are as follows; irritant reactions (42%) derived from chemical irritants from urine or stools, mechanical trauma, pre-existing diseases such as atopic dermatitis, psoriasis, seborrheic dermatitis or eczema (20%), infection (6%), allergic contact dermatitis (0-7%) and pyoderma gangrenosum (0-6%). Intractable skin troubles should be differentiated from rare coexisting illness, such as Degos’ disease, hyperpigmentation, lichen sclerosus, bullous pemphigoid, and benign or malignant tumors [4]. In the present case, we suspected allergic contact dermatitis because of the round erythema corresponding to a stoma cover. However, a malignant tumor was finally suspected because it was refractory to conventional therapies. We found some reports in the literature of carcinoma or lymphoma arising in the peristomal skin of ileostomy or colostomy stoma, but to the best of our knowledge, this is the first report of secondary Paget's disease arising in peri-urostomal skin. The wet environment of peri-urostomal skin may contribute to the progression of malignant cells and formation of the round-shaped erythema. This case was difficult to diagnose because its shape was round and it mimicked allergic contact dermatitis and also because pagetoid spread in peri-urostomal skin from urological carcinoma is very rare. We suggest that intractable skin problems in peri-urostomal skin should be closely examined by a skin biopsy in cases refractory to conventional therapies.

Disclosure

Financial support: none. Conflicts of interest: none.

References

1. Nowak MA, Guerriere-Kovach P, Pathan A, Campbell TE, Deppisch L.M. Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. Arch Pathol Lab Med 1998 ; 122 : 1077-1081.

2. Ohnishi T, Watanabe S. The use of cytokeratins 7 and 20 in the diagnosis of primary and secondary extramammary Paget's disease. Br J Dermatol 2000 ; 142 : 243-247.

3. Lyon CC, Smith AJ, Griffiths CE, Beck M.H. The spectrum of skin disorders in abdominal stoma patients. Br J Dermatol 2000 ; 143 : 1248-1260.

4. Nybaek H, Jemec G.B. Skin problems in stoma patients. J Eur Acad Dermatol Venereol 2010 ; 24 : 249-257.