Letters to the Editor
special C because our patient suffered from the adult multisystem LCH with involvement of intracranial lesions.
CONFLICT OF INTEREST:
None.
Takafumi NUMATA,1 Yukari OKUBO,1 Masaki UCHIYAMA,1 Yumi TAKEUCHI,1 Mayuko MURO,1 Ryoji TSUBOI,1 Akihiko GOTOH,2 Mizuho NOMURA,3 Yoshihiko MITSUHASHI1 1
Department of Dermatology, Tokyo Medical University, 2Department of Hematology, Juntendo University School of Medicine, and 3Mayumi Clinic, Tokyo, Japan doi: 10.1111/1346-8138.12732
REFERENCES 1 Morimoto A, Shimazaki C, Takahashi S et al. Therapeutic outcome of multifocal Langerhans cell histiocytosis in adults treated with the Special C regimen formulated by the Japan LCH Study Group. Int J Hematol 2013; 97: 103–108. 2 Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet 1987; 1: 208–209. 3 Lichtenstein L. Histiocytosis X: integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953; 56: 84–102. 4 Stockschlaeder M, Sucker C. Adult Langerhans cell histiocytosis. Eur J Haematol 2006; 76: 363–368. 5 Arico M, Girschikofsky M, Genereau T et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 2003; 39: 2341–2348.
Case of folliculotropic mycosis fungoides with prominent loss of T-cell antigens CD7 and CD26 in blood T cells Dear Editor, Folliculotropic mycosis fungoides (FMF) is characterized by pronounced infiltration of malignant T cells in the follicular epithelium, and it occasionally shows epidermotropism.1–3 A range of clinical features can be observed, including follicular or indurated papules, acneiform lesions, alopecia, plaques, ulcerations and tumors.1–3 The prognosis of FMF appears to be poorer than that of conventional MF.4 It has been shown that increasing numbers of abnormal T cells with loss of the T-cell antigens CD7 and CD26 in the blood of patients with MF zary syndrome (SS) are associated with worsening disor Se ease.5 This report describes a case of FMF with rapid progression and prominent loss of the T-cell antigens CD7 and CD26 from the blood. A 90-year-old Japanese man visited our hospital complaining of pruritic red papules all over his body (Fig. 1a). Tumors and alopecia were also present on his scalp (Fig. 1b). Laboratory examinations revealed no atypical zary cells in his blood. The serum mononuclear cells or Se level of soluble interleukin-2 receptor was elevated (2100 U/ mL). He was seronegative for human T-lymphotropic virus type 1. Computed tomography revealed neither lymphadenopathy nor visceral lesions. A biopsy specimen from a tumor on his forehead showed pronounced infiltration of atypical lymphocytes with hyperchromatic and convoluted nuclei into the perifollicular areas and the follicular epithelium but not in the interfollicular epithelium (Fig. 1c). Immunohistochemistry showed that the lymphocytic infiltrate was strongly positive for T-cell markers CD3 and CD4 but not for CD7,
CD20 or CD30. Southern blot analysis using DNA extracted from a skin lesion exhibited monoclonal rearrangement of the T-cell receptor (TCR)-Cb1. On the basis of these findings, we made a diagnosis of FMF. Although he was treated with systemic psoralen plus ultraviolet A (PUVA) and oral corticosteroids, the lesions progressed to erythroderma within 5 months. After disease progression, we analyzed his blood using flow cytometry. Interestingly, although we observed the loss of the T-cell antigens CD7 and CD26 in the CD3+/CD4+ T-cell populations (Fig. 1d), no monoclonal rearrangement of the TCR-Cb1 or atypical lymphocytes were detected in the blood sample. He died of pneumocystis pneumonia 8 months after the first visit. Patients with FMF are often unresponsive to standard treatments such as local steroids and PUVA. This may be due to the deep localization of the T-cell infiltrate in their hair follicles. The poorer prognosis in our case is consistent with the previous report that showed worsening prognosis of MF and SS with increasing numbers of CD3+ CD4+ CD7– CD26– T cells in the blood.5 In the previous reports, monoclonal rearrangements of TCR were sometimes detected in such cases. However, in our case, rearrangement was not detected, suggesting that these T cells were not neoplastic. In conclusion, our case suggests that increasing numbers of CD3+ CD4+ CD7– CD26– T cells in the blood could imply poor prognosis in FMF as well as MF and SS.
CONFLICT OF INTEREST:
None.
Correspondence: Takamitsu Matsuzawa, M.D., Department of Dermatology, Faculty of Medicine, University of Yamanashi, 1110 Shimokato, Chuo, Yamanashi 409-3898, Japan. Email: [email protected]
108
© 2014 Japanese Dermatological Association
Letters to the Editor
(a)
(b)
(c)
(d)
Figure 1. (a,b) Clinical pictures. (a) Numerous pruritic, red, 2-mm papules on the back. (b) Indurated erythematous plaques, skin tumors and alopecia on the temporal region of the head. (c) Histological findings. Perifollicular and intrafollicular infiltration by small to medium sized atypical T cells (hematoxylin–eosin, original magnification 9100). (d) Quantitatively and phenotypically abnormal T cells observed in peripheral blood mononuclear cells (PBMC) by flow cytometric analysis. Increasing numbers of CD3+ CD4+ CD7– or CD3+ CD4+ CD7– CD26– T cells in PBMC of the patient, compared with samples from four different healthy donors. Numbers indicate percentages of cells within the indicated gates. CD3+ cells were gated in the upper panels, and CD3+ CD4+ cells were gated in the lower panels. HD, healthy donor.
Takamitsu MATSUZAWA,1 Takashi INOZUME,1 Machiko TAKAKI,1 Kazutoshi HARADA,2 Tatsuyoshi KAWAMURA,1 Naotaka SHIBAGAKI,1 Shinji SHIMADA1 1
Department of Dermatology, Faculty of Medicine, University of Yamanashi, Yamanashi, and 2Department of Dermatology, Tokyo Medical University, Tokyo, Japan doi: 10.1111/1346-8138.12738
REFERENCES
2 Lehman JS, Cook-Norris RH, Weed BR et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010; 146: 607–613. 3 Mantaka P, Helsing P, Gjersvik P, Bassarova A, Clausen OP, Delabie J. Clinical and histopathological features of folliculotropic mycosis fungoides: a Norwegian patient series. Acta Derm Venereol 2013; 93: 325–329. 4 Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J. Folliculotropic mycosis fungoides: an aggresive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008; 144: 738–746. 5 Hristov AC, Vonderheid EC, Borowitz MJ. Simplified flow cytometric zary syndrome. Am J Clin assessment in mycosis fungoides and Se Pathol 2011; 136: 944–953.
1 Shimauchi T, Ohshima A, Tokura Y. Folliculotropic mycosis fungoides presenting as papuloerythroderma. J Dermatol 2006; 33: 498–500.
© 2014 Japanese Dermatological Association
109
special C because our patient suffered from the adult multisystem LCH with involvement of intracranial lesions.
CONFLICT OF INTEREST:
None.
Takafumi NUMATA,1 Yukari OKUBO,1 Masaki UCHIYAMA,1 Yumi TAKEUCHI,1 Mayuko MURO,1 Ryoji TSUBOI,1 Akihiko GOTOH,2 Mizuho NOMURA,3 Yoshihiko MITSUHASHI1 1
Department of Dermatology, Tokyo Medical University, 2Department of Hematology, Juntendo University School of Medicine, and 3Mayumi Clinic, Tokyo, Japan doi: 10.1111/1346-8138.12732
REFERENCES 1 Morimoto A, Shimazaki C, Takahashi S et al. Therapeutic outcome of multifocal Langerhans cell histiocytosis in adults treated with the Special C regimen formulated by the Japan LCH Study Group. Int J Hematol 2013; 97: 103–108. 2 Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet 1987; 1: 208–209. 3 Lichtenstein L. Histiocytosis X: integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953; 56: 84–102. 4 Stockschlaeder M, Sucker C. Adult Langerhans cell histiocytosis. Eur J Haematol 2006; 76: 363–368. 5 Arico M, Girschikofsky M, Genereau T et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 2003; 39: 2341–2348.
Case of folliculotropic mycosis fungoides with prominent loss of T-cell antigens CD7 and CD26 in blood T cells Dear Editor, Folliculotropic mycosis fungoides (FMF) is characterized by pronounced infiltration of malignant T cells in the follicular epithelium, and it occasionally shows epidermotropism.1–3 A range of clinical features can be observed, including follicular or indurated papules, acneiform lesions, alopecia, plaques, ulcerations and tumors.1–3 The prognosis of FMF appears to be poorer than that of conventional MF.4 It has been shown that increasing numbers of abnormal T cells with loss of the T-cell antigens CD7 and CD26 in the blood of patients with MF zary syndrome (SS) are associated with worsening disor Se ease.5 This report describes a case of FMF with rapid progression and prominent loss of the T-cell antigens CD7 and CD26 from the blood. A 90-year-old Japanese man visited our hospital complaining of pruritic red papules all over his body (Fig. 1a). Tumors and alopecia were also present on his scalp (Fig. 1b). Laboratory examinations revealed no atypical zary cells in his blood. The serum mononuclear cells or Se level of soluble interleukin-2 receptor was elevated (2100 U/ mL). He was seronegative for human T-lymphotropic virus type 1. Computed tomography revealed neither lymphadenopathy nor visceral lesions. A biopsy specimen from a tumor on his forehead showed pronounced infiltration of atypical lymphocytes with hyperchromatic and convoluted nuclei into the perifollicular areas and the follicular epithelium but not in the interfollicular epithelium (Fig. 1c). Immunohistochemistry showed that the lymphocytic infiltrate was strongly positive for T-cell markers CD3 and CD4 but not for CD7,
CD20 or CD30. Southern blot analysis using DNA extracted from a skin lesion exhibited monoclonal rearrangement of the T-cell receptor (TCR)-Cb1. On the basis of these findings, we made a diagnosis of FMF. Although he was treated with systemic psoralen plus ultraviolet A (PUVA) and oral corticosteroids, the lesions progressed to erythroderma within 5 months. After disease progression, we analyzed his blood using flow cytometry. Interestingly, although we observed the loss of the T-cell antigens CD7 and CD26 in the CD3+/CD4+ T-cell populations (Fig. 1d), no monoclonal rearrangement of the TCR-Cb1 or atypical lymphocytes were detected in the blood sample. He died of pneumocystis pneumonia 8 months after the first visit. Patients with FMF are often unresponsive to standard treatments such as local steroids and PUVA. This may be due to the deep localization of the T-cell infiltrate in their hair follicles. The poorer prognosis in our case is consistent with the previous report that showed worsening prognosis of MF and SS with increasing numbers of CD3+ CD4+ CD7– CD26– T cells in the blood.5 In the previous reports, monoclonal rearrangements of TCR were sometimes detected in such cases. However, in our case, rearrangement was not detected, suggesting that these T cells were not neoplastic. In conclusion, our case suggests that increasing numbers of CD3+ CD4+ CD7– CD26– T cells in the blood could imply poor prognosis in FMF as well as MF and SS.
CONFLICT OF INTEREST:
None.
Correspondence: Takamitsu Matsuzawa, M.D., Department of Dermatology, Faculty of Medicine, University of Yamanashi, 1110 Shimokato, Chuo, Yamanashi 409-3898, Japan. Email: [email protected]
108
© 2014 Japanese Dermatological Association
Letters to the Editor
(a)
(b)
(c)
(d)
Figure 1. (a,b) Clinical pictures. (a) Numerous pruritic, red, 2-mm papules on the back. (b) Indurated erythematous plaques, skin tumors and alopecia on the temporal region of the head. (c) Histological findings. Perifollicular and intrafollicular infiltration by small to medium sized atypical T cells (hematoxylin–eosin, original magnification 9100). (d) Quantitatively and phenotypically abnormal T cells observed in peripheral blood mononuclear cells (PBMC) by flow cytometric analysis. Increasing numbers of CD3+ CD4+ CD7– or CD3+ CD4+ CD7– CD26– T cells in PBMC of the patient, compared with samples from four different healthy donors. Numbers indicate percentages of cells within the indicated gates. CD3+ cells were gated in the upper panels, and CD3+ CD4+ cells were gated in the lower panels. HD, healthy donor.
Takamitsu MATSUZAWA,1 Takashi INOZUME,1 Machiko TAKAKI,1 Kazutoshi HARADA,2 Tatsuyoshi KAWAMURA,1 Naotaka SHIBAGAKI,1 Shinji SHIMADA1 1
Department of Dermatology, Faculty of Medicine, University of Yamanashi, Yamanashi, and 2Department of Dermatology, Tokyo Medical University, Tokyo, Japan doi: 10.1111/1346-8138.12738
REFERENCES
2 Lehman JS, Cook-Norris RH, Weed BR et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010; 146: 607–613. 3 Mantaka P, Helsing P, Gjersvik P, Bassarova A, Clausen OP, Delabie J. Clinical and histopathological features of folliculotropic mycosis fungoides: a Norwegian patient series. Acta Derm Venereol 2013; 93: 325–329. 4 Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J. Folliculotropic mycosis fungoides: an aggresive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008; 144: 738–746. 5 Hristov AC, Vonderheid EC, Borowitz MJ. Simplified flow cytometric zary syndrome. Am J Clin assessment in mycosis fungoides and Se Pathol 2011; 136: 944–953.
1 Shimauchi T, Ohshima A, Tokura Y. Folliculotropic mycosis fungoides presenting as papuloerythroderma. J Dermatol 2006; 33: 498–500.
© 2014 Japanese Dermatological Association
109
![[Folliculotropic mycosis fungoides].](/assets/img/hold.png)
