Indian Journal of Dermatology
: 2021  |  Volume : 66  |  Issue : 3  |  Page : 330-

Clear Cell Porocarcinoma Arising in an Epidermodysplasia Verruciformis Patient

Bruno de Castro e Souza1, Neusa Yuriko Sakai Valente1, Stephen Tyring2, Walmar Roncalli Pereira de Olveira1,  
1 Department of Dermatology, University of São Paulo, São Paulo, Brazil
2 Department of Dermatology, University of Texas Health Science Center, Houston, Texas

Correspondence Address:
Bruno de Castro e Souza
Department of Dermatology, University of São Paulo, São Paulo

How to cite this article:
Souza BC, Sakai Valente NY, Tyring S, Pereira de Olveira WR. Clear Cell Porocarcinoma Arising in an Epidermodysplasia Verruciformis Patient.Indian J Dermatol 2021;66:330-330

How to cite this URL:
Souza BC, Sakai Valente NY, Tyring S, Pereira de Olveira WR. Clear Cell Porocarcinoma Arising in an Epidermodysplasia Verruciformis Patient. Indian J Dermatol [serial online] 2021 [cited 2023 Jun 6 ];66:330-330
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Epidermodysplasia verruciformis (EV) is a rare skin disease characterized by abnormalities of cell-mediated immunity, disseminated infection with a specific group of related HPV genotypes (EV-HPVs), and development of skin cancer. The EV cancers are predominantly squamous cell carcinoma (SCC).[1] Porocarcinoma is the most common subtype of malignant sweat gland neoplasia. The clear cell variant of porocarcinoma is rare.[2] We describe the presence of clear cell porocarcinoma in one patient with EV.

A 33-year-old Caucasian man with classic EV [Figure 1] had an 8-month history of an infiltrated and keratotic plaque measuring approximately 1 cm along its longest axis on the upper lip [Figure 2]. He was the only son of consanguineous marriage. Histopathological analysis showed a malignant epithelial neoplasm involving the epidermis and dermis consisting of numerous clear cells with nuclear atypia. Areas with ductal differentiation were seen, mainly in the dermal component. Among the atypical epithelial cells, cytopathic changes of EV were also found [Figure 3]. The clear tumor cells demonstrated glycogen as well as PAS-positive and diastase-labile material in the cytoplasm. The immunohistochemical exam was focally positive for EMA and CEA [Figure 4]. The diagnosis of clear cell porocarcinoma was made, and the patient underwent tumor excision. HPV subtype detected on the lip lesion was 14d through nested PCR.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

EV is an autosomal recessive genodermatosis. In most cases, there is a homozygous inactivation mutation in the TMC6 (EVER 1) and TMC8 (EVER 2), leading to a deficiency in cellular immunity against HPV. Other mutations have been determined, such as ROH, MST-1, CORO1A, and IL-17. As these mutations lead to susceptibility to other infections susceptibility, some authors have suggested naming this subgroup as nonclassical EV.[1]

Regardless of the form of EV (classical or nonclassical), the clinical presentation is quite similar. The skin lesions are highly polymorphic including flat-wart, pityriasis versicolor-like lesions, seborrheic keratosis like-plaques, and reddish macules that start in early childhood and can undergo malignant transformation in about 50% to 70% of patients at early stages of life.[1],[3],[4],[5]

The EV cancers, mostly SCCs, tend to develop on sun-exposed areas and are predominantly associated with EV-HPV types 5 and 8. In Brazil, the most prevalent oncogenic type is HPV 14d.[3] The association of EV and other types of tumors is scarce, with few cases of sebaceous carcinoma, Merkel cell carcinoma, and eccrine syringoid carcinoma.[3],[4]

To our knowledge, this is the first report of an association of EV with clear cell porocarcinoma. Considering that Urso et al. demonstrated the presence of β-HPV in porocarcinomas, and HPV14d was found in our patient's tumor, we hypothesized this may not be a coincidental finding.[2] We propose that EV-associated defects in cell-mediated immunity, together with immunosuppressive cytokines and p53 abnormalities, predisposed the patient to developing clear cell porocarcinoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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3de Oliveira WRP, He Q, Rady PL, Hughes TK, Neto CF, Rivitti EA, et al. HPV typing in Brazilian patients with epidermodysplasia verruciformis: High prevalence of EV-HPV 25. J Cutan Med Surg 2004;8:110-5.
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