Content » Vol 94, Issue 1

Short communication

CD4/CD8 Double-negative Mycosis Fungoides Mimicking Erythema Gyratum Repens in a Patient with Underlying Lung Cancer

Kotaro Nagase, Reiko Shirai, Takeshi Okawa, Takuya Inoue, Noriyuki Misago and Yutaka Narisawa

Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan. E-mail: kotaro@nagase-skin.com

Accepted Feb 21, 2013; Epub ahead of print May 17, 2013

Mycosis fungoides (MF), a common type of cutaneous T-cell lymphoma (TCL), can mimic various dermatoses. We describe here a case of MF, presenting as erythema gyratum repens (EGR)-like lesions, in a patient with lung cancer.

CASE REPORT

A 73-year-old Japanese man presented with a 10-year history of worsening, itchy, erythematous eruptions on his trunk and extremities. Dermatological examination revealed concentric, slightly infiltrated, annular, red patches and plaques, closely resembling EGR, on his chest, abdomen and extremities (Fig. 1). The lesions were not painful. Microscopy and culture excluded the presence of a mycotic infection. Type 1 human T-lymphotropic virus infection was also excluded. Cutaneous histopathology revealed a dense infiltrate of hyperchromatic, atypical lymphocytes that showed extensive epidermotropism. Immunohistochemical studies showed that most cells within the epidermis and dermis were CD3+, CD4, CD8 and CD45RO+ (Fig. 2). Molecular biology investigations indicated a monoclonal rearrangement of the T-cell receptor (TCR)-beta chain. Clinical evaluation did not show spreading of TCL, but indicated primary lung adenocarcinoma.

Fig. 1. Mycosis fungoides mimicking erythema gyratum repens. Clinical findings of concentric, annular, red patches on the trunk.

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Fig. 2. (a) Histology revealed a dense infiltrate containing small lymphocytes with epidermotropism of atypical lymphocytes. (b–d) Immunohisto­chemical features: the intraepidermal lymphocytes are CD3+ (b), CD4(c) and CD8(d). (H&E-stain: (a) × 100; (b) CD3; (c) CD4 and (d) CD8 × 200).

A diagnosis of Stage IB (T2N0M0B0) MF was confirmed, and treatment with cycles of photochemotherapy (psoralen plus ultraviolet A radiation; PUVA) combined with topical corticosteroids was initiated, achieving partial clinical remission. No major changes in the skin lesions were noted following surgical excision of the lung cancer.

DISCUSSION

MF is characterized by extremely variable presentation, and reportedly mimicks at least 25 dermatoses (1). Patients usually present with patches, plaques, tumours, and/or erythroderma. EGR-like MF, has recently been described as a possible rare form of MF. EGR is a rare paraneoplastic syndrome strongly associated with various malignancies, particularly lung, breast, and oesophageal cancers. The rash consists of serpiginous, erythematous, concentric bands that can be figurate, gyrate, or annular, and are arranged in parallel rings lined by a fine, trailing edge of scale; a pattern often described as “wood grained.”

To our knowledge, only 6 cases, including the present case, of MF resembling EGR, have been reported (2–6) (Table I). The present case is the first associated with an underlying malignancy.

Table I. Summary of reported cases of mycosis fungoides producing eruptions resembling erythema gyratum repens

Reference

Age, years

Extent

T-cell receptor rearrange­­ment studies

Mycotic superinfection

Underlying internal malignancy

Poonawalla et al. 2006 (3)

55

Trunk and extremities

Positive

Trichophyton rubrum

NM

Moore et al. 2008 (4)

73

Trunk

Positive

Negative

None

Jouary et al. 2008 (5)

77

Abdomen, back and buttock

NM

Trichophyton rubrum

NM

Cerri et al. 2010 (2)

61

Chest and upper limbs

Positive

Negative

None

Holcomb et al. 2012 (6)

75

Chest and anterior abdomen

Positive

Negative

NM

Current case 2013

73

Trunk and extremities

Positive

Negative

Lung cancer

NM: not mentioned.

The neoplastic T cells present in MF are typically CD4+ and CD8; however, there are variants in which 1 or both of these subpopulations are lost. A review indicated that cases with unusual cell populations, such as the present case, often have unique clinical presentations (7). Various clinical morphologies may be elicited by lymphocytes with the differing phenotypes. Moreover, the host responses between patients may differ, which may contribute to the various presentations (1). The patient’s underlying lung cancer, a malignancy closely related to true EGR, did not appear to contribute to the pathogenesis of the EGR-like lesions, based on the clinical course of the disease. It is notable that an association between MF and lung cancer has been reported in 2 large cohorts of patients (8, 9).

REFERENCES

1. Zackheim HS, McCalmont TH. Mycosis fungoides: the great imitator. J Am Acad Dermatol 2002; 47: 914–918.

2.
Cerri A, Vezzoli P, Serini SM, Crosti C, Berti E, Marzano AV. Mycosis fungoides mimicking erythema gyratum repens: an additional variant? Eur J Dermatol 2010; 20: 540–541.

3. Poonawalla T, Chen W, Duvic M. Mycosis fungoides with tinea pseudoimbricata owing to Trichophyton rubrum infection. J Cutan Med Surg 2006; 10: 52–56.

4.
Moore E, McFarlane R, Olerud J. Concentric wood grain erythema on the trunk. Arch Dermatol 2008; 144: 673–678.

5.
Jouary T, Lalanne N, Stanislas S, Vergier B, Delaunay M, Taieb A. Erythema gyratum repens-like eruption in mycosis fungoides: is dermatophyte superinfection underdiagnosed in cutaneous T-cell lymphomas? J Eur Acad Dermatol Venereol 2008; 22: 1276–1278.

6.
Holcomb M, Duvic M, Cutlan J. Erythema gyratum repens-like eruptions with large cell transformation in a patient with mycosis fungoides. Int J Dermatol 2012; 51: 1231–1233.

7.
Hodak E, David M, Maron L, Aviram A, Kaganovsky E, Feinmesser M. CD4/CD8 double-negative epidermotropic cutaneous T-cell lymphoma: an immunohistochemical variant of mycosis fungoides. J Am Acad Dermatol 2006; 55: 276–284.

8.
Kantor AF, Curtis RE, Vonderheid EC, van Scott EJ, Fraumeni JF, Jr. Risk of second malignancy after cutaneous T-cell lymphoma. Cancer 1989; 63: 1612–1615.

9.
Vakeva L, Pukkala E, Ranki A. Increased risk of secondary cancers in patients with primary cutaneous T cell lymphoma. J Invest Dermatol 2000; 115: 62–65.