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Year : 2021  |  Volume : 66  |  Issue : 2  |  Page : 227
Mycosis fungoides associated with pseudoepitheliomatous hyperplasia

Department of Medicine of Sensory and Motor Organs, Tottori University Faculty of Medicine, Yonago, Japan

Date of Web Publication16-Apr-2021

Correspondence Address:
Kazunari Sugita
Department of Medicine of Sensory and Motor Organs, Tottori University Faculty of Medicine, Yonago
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_731_18

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How to cite this article:
Sugita K, Ito A, Yamamoto O. Mycosis fungoides associated with pseudoepitheliomatous hyperplasia. Indian J Dermatol 2021;66:227

How to cite this URL:
Sugita K, Ito A, Yamamoto O. Mycosis fungoides associated with pseudoepitheliomatous hyperplasia. Indian J Dermatol [serial online] 2021 [cited 2021 Oct 16];66:227. Available from:


A 76-year-old Japanese woman was initially referred to us in 2003 for evaluation of a 4-year history of skin eruption. The eruption first appeared as generalized or widespread patches and plaques. She showed neither lymphadenopathy nor hepatosplenomegaly. A skin biopsy specimen from erythematous plaque disclosed numerous atypical lymphocytes in the dermis with their epidermotropism [Figure 1]a. The atypical cells were positive for CD3 and CD4 but negative for CD8, CD30, and CD56. Based on the immunohistochemical findings mentioned above, we diagnosed the skin lesions as mycosis fungoides. Initially, the patient was treated with a combination of intravenous IFN-γ and PUVA irradiation for the plaques that were present on her trunk and extremities. At the first-year follow-up, this therapy was effective with subsidence of lesions and she did not receive any therapy thereafter for 3 years. However, her skin lesions gradually reappeared as erythematous plaques, and because these lesions had been chronic and persistent, she was started on PUVA irradiation or narrow band UVB therapy.
Figure 1: (a) The biopsy specimen shows infiltration of atypical lymphocytes into the epidermis and upper dermis (hematoxylin and eosin stain). (b) Multiple nodules on the left side of her buttock. Black dots indicate the lesion of excisional biopsy. (c) The biopsy shows pseudoepitheliomatous hyperplasia (hematoxylin and eosin stain). (d) Atypical lymphoid cells had infiltrated into the upper dermis (hematoxylin and eosin stain). (e) A giant cell in the epidermis (hematoxylin and eosin stain). Immunohistochemical staining revealed that lymphocytes were positive for (f) CD4 and (g) CD8. (h) Tumor cells which are stained a blue color, expressed CD4 intermingled with CD8-positive cells shown by a brown color.

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In 2017, she noticed multiple nodules on the left side of her buttock [Figure 1]b. Histopathological examination of a nodule revealed marked epidermal hyperplasia with hyperkeratosis [Figure 1]c. The upper dermis showed dense cellular infiltration consisting of both small lymphocytes and large atypical lymphocytes [Figure 1]d. A multinucleated giant cell was noted in the epidermis [Figure 1]e. The dermis was characterized by the presence of epithelioid cells associated with mixed inflammatory infiltrate such as neutrophils and eosinophils. Most of the dermal and epidermal lymphocytes were positive for CD4 [Figure 1]f and CD8 [Figure 1]g, and scattered lymphocytes were positive for granzyme B. As shown in [Figure 1]h, large atypical tumor cells, which were stained a blue color, expressed CD4 intermingled with CD8-positive cells shown by a brown color. Based on these findings, we clarified that tumor cells were of CD4 + phenotype instead of CD8+ or CD4/CD8 double-positive cells. PAS-stained and Grocott-stained sections did not reveal any organisms. Thus, we diagnosed the patient as having mycosis fungoides associated with pseudoepitheliomatous hyperplasia.

Pseudoepitheliomatous hyperplasia is most commonly associated with mycobacterial and deep fungal infections, and the spectrum of associated disorders includes other infectious conditions such as viral, bacterial, protozoal, and spirochetal processes, as well as neoplastic and chronic inflammatory disorders.[1],[2] Previously, a limited number of studies investigating the association between pseudoepitheliomatous hyperplasia and cutaneous lymphoma have been published.[1],[3] These cases almost exclusively fall in the category of CD30-positive lymphoproliferative disorders, such as anaplastic large cell lymphoma and lymphomatoid papulosis. Other cutaneous lymphomas such as CD56-positive cytotoxic T-cell lymphoma and natural-killer (NK)/T cell lymphoma, nasal-type, and mycosis fungoides have rarely been reported.[4] Another intriguing finding in our case was a giant cell in the epidermis. This evidence together with the infiltration of CD8+ lymphocytes suggest that anti-tumor immunity may have promoted transepidermal elimination and thus may have resulted in the formation of pseudoepitheliomatous hyperplasia in our patient.

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.

   References Top

Zayour M, Lazova R. Pseudoepitheliomatous hyperplasia: A review. Am J Dermatopathol 2011;33:112-22.  Back to cited text no. 1
Freeman RG. On the pathogenesis of pseudoepitheliomatous hyperplasia. J Cutan Pathol 1974;1:231-7.  Back to cited text no. 2
Price A, Miller JH, Junkins-Hopkins JM. Pseudocarcinomatous hyperplasia in anaplastic large cell lymphoma, a mimicker of poorly differentiated squamous cell carcinoma: Report of a case and review of the literature. J Cutan Pathol 2015;42:863-9.  Back to cited text no. 3
Ginsberg D, Hill H, Wilson B, Plaza JA, Schieke SM. Pseudocarcinomatous hyperplasia mimicking squamous cell carcinoma in a case of CD56-positive cytotoxic T-cell lymphoma. J Cutan Pathol 2015;42:194-8.  Back to cited text no. 4


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