Dermatology: Practical and Conceptual


Image Letter | Dermatol Pract Concept. 2023;13(4):e2023223 1

Photodynamic Therapy for Primary Cutaneous 
Extramammary Paget Disease

Alberto Meneguzzo1, Roberto Mazzetto1, Annalisa Lazzarotto1, Stefano Piaserico1

1 Department of Dermatology, University of Padua, Italy

Citation: Meneguzzo A, Mazzetto R, Lazzarotto A, Piaserico S. Photodynamic Therapy for Primary Cutaneous Extramammary Paget’s 
Disease. Dermatol Pract Concept. 2023;13(4):e2023223. DOI: https://doi.org/10.5826/dpc.1304a223

Accepted: May 3, 2023; Published: October 2023

Copyright: ©2023 Meneguzzo et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, 
distribution, and reproduction in any medium, provided the original authors and source are credited.

Funding: None.

Competing Interests: None.

Authorship: All authors have contributed significantly to this publication.

Corresponding Author: Roberto Mazzetto, Via Vincenzo Gallucci 4, 35121, Padova (PD), Italy. Tel +393479466312 Email: 
robertomazzetto93@gmail.com

Case Presentation

A 70-year-old man presented with a slowly growing erythem-

atous scaly plaque on the right inguinal fold ( Figure  1A). 

The patient complained about itching and burning. Derma-

toscopic features observed included a milky-red background 

with variable vascular patterns, in particular dotted vessels 

and glomerular vessels with some erosions (Figure  1C). 

 Biopsy showed nests of proliferating atypical cells with pag-

etoid spread to the entire epidermis (Figure 1E). Cerium am-

monium molybdate (CAM) 5.2 stain, marking cytokeratins 

8 and to a lesser extent cytokeratin 7, highlighted Paget cells 

proliferating in the epidermis and upper dermis (Figure 1F). 

A diagnosis of Extramammary Paget disease (EMPD) was 

thus made. Thoracic, abdominal, and pelvic computed to-

mography scan resulted negative. Also, carcinoembryonic 

antigen (CEA) and prostate-specific antigen (PSA) were in 

the normal range. The patient received 5 sessions of methyl 

aminolevulinate photodynamic therapy (MAL-PDT) every 

2 weeks with a complete resolution of the cutaneous mani-

festation and without any adverse effects or functional im-

pairments (Figure 1, B and D).

Teaching Point

EMPD is a rare neoplastic disease affecting predominantly 

patients between 50 to 80 years of age [1]. The current treat-

ments may include surgery, considered the first-line therapy 

and radiotherapy, imiquimod cream, photodynamic therapy 

(PDT), topical fluorouracil and carbon dioxide laser therapy. 

Data for treatment with fluorouracil, carbon dioxide laser, 

and combination topical therapies are limited. In patients 

unwilling to undergo surgical treatment or who are not sur-

gical candidates, PDT may represent an interesting approach 

to EMPD. In particular, MAL-PDT showed complete clear-

ance rate of 27.4 % [2].



2 Image Letter | Dermatol Pract Concept. 2023;13(4):e2023223

Figure 1. (A) A slowly growing erythematous scaly plaque on the right inguinal fold: a case of EMPD. (B) Resolution of the cutaneous man-

ifestation after five sessions of MAL-PDT. (C)  Dermatoscopic features: milky-red background with variable vascular patterns, in particular 

dotted vessels and glomerular vessels. (D) Dermatoscopic aspects of the skin after MAL-PDT. (E) Hematoxylin and Eosin stained tissue sec-

tion showed an intraepithelial proliferation of large, round, atypical cells with an abundant, pale mucin-rich cytoplasm (Paget cells), located 

in nests along the basement membrane and extending to the entire epidermis, with occasional mitoses. Hyperkeratosis and parakeratosis 

overlie the proliferation, and a dermal infiltrate is evident (H&E, 40×). (F) CAM 5.2 stain marks Paget cells proliferating in the epidermis 

and upper dermis.

References

1. Lam C, Funaro D. Extramammary Paget’s Disease: Summary of 

Current Knowledge. Dermatol Clin. 2010;28(4):807–26. DOI: 

10.1016/j.det.2010.08.002. PMID: 20883922.

2. Shim PJ, Zeitouni NC. Photodynamic therapy for extramam-

mary Paget’s disease: A systematic review of the literature. Pho-

todiagnosis Photodyn Ther. 2020;31:101911. DOI: 10.1016/j 

.pdpdt.2020.101911. PMID: 32645437.References