baseline. Moreover, relative variation of PASI score versus relative variation of MPV showed a negative relationship for both groups, meaning that a severe reduction of PASI score did not correspond to the highest reduction of MPV.This suggests that MPV trend can not be used as a marker of disease severity or as an appropriate indicator of thrombotic risk in psoriasis. This is in accordance with the most recent hypothesis by Saleh et al. which stated that MPV is not a reliable indicator of atherosclerosis risk in Pso patients [9]. Genetic predisposition [10] or subtle systemic inflammation in these patients may be responsible for higher MPV. This relationship needs to be more deeply investigated, assessing other platelet activation markers that could be increased in psoriasis and influence the risk of atherothrombosis.
Disclosure. Financial support: none. Conflict of interest: none.

1 Department of medicine and aging science, Dermatologic Clinic, 2 Department of experimental and clinical sciences, Biostatistics Laboratory, 3 Department of experimental and clinical science, Dermatologic clinic, 4 Department of clinical medicine and surgery, Dermatologic clinic, University of Padova, Padova, Italy 5 Department of medicine and health sciences, University of Molise, Cambobasso, Italy 6 Department of medicine and aging science, Center for excellence on aging (CeSI), University G.d’Annunzio, Chieti, Italy

Alessandra CAPO1 Marta DI NICOLA2 Matteo AURIEMMA3 Stefano PIASERICO4 Chiara CUCCURULLO5 Francesca SANTILLI6 Giovanni DAVI6 Paolo AMERIO1

1. Saber TP, Veale DJ Psoriatic arthritis management updatebiotherapeutic options. J Rheumatol Suppl 2009 Aug; 83: 65-8. 2. Gelfand JM, Neimann AL, Shin DB, Wang X, Margolis DJ, Troxel AB. Risk of myocardial infarction in patients with psoriasis. JAMA 2006; 296: 1735-41. 3. Berrettini M, Parise P, Costantini V, Grasselli S, Nenci GG. Platelet activation in psoriasis. Thromb Haemost 1985; 53: 195-7. 4. Park Y, Schone N, Harris W. Mean platelet volume as an indicator of platelet activation: methodological issues. Platelets 2002; 13: 301-6. 5. Chu SG, Becker RC, Berger PB, et al. Mean platelet volume as a predictor of cardiovascular risk: a systematic review and meta-analysis. J Thromb Haemost 2010; 8: 148-56. 6. Gasparyan AY, Ayvazyan L, Mikhailidis DP, Kitas GD. Mean platelet volume: a link between thrombosis and inflammation? Curr Pharm Des 2011; 17: 47-58. EJD, vol. 24, n◦ 1, January-February 2014

7. Karabudak O, Ulusoy RE, Erikci AA, et al. Inflammation and hypercoagulable state in adult psoriatic men. Acta Derm Venereol 2008; 88: 337-40. 8. Filiz Canpolat, Hatice Akpinar, Fatma Eskioglu. Mean platelet volume in psoriasis and psoriatic arthritis. Clin Rheumatol 2010; 29: 325-8. 9. Saleh HM, Attia EA, Onsy AM, Saad AA, Abd Ellah MM. Platelet activation: A link between psoriasis per se and subclinical atherosclerosis; Case control study. Br J Dermatol 2013 Feb 28 (Epub ahead of print). 10. Meisinger C, Prokisch H, Gieger C, et al. A genome-wide association study identifies three loci associated with mean platelet volume. Am J Hum Genet 2009; 84: 66-71. doi:10.1684/ejd.2014.2269

Folliculotropic mycosis fungoides with extreme pilosebaceous involvement and hematological abnormalities Folliculotropic mycosis fungoides (fMF) has been defined as a variant of MF with follicular infiltration of atypical lymphoid cells, sparing the interfollicular epidermis [1-4]. We report two patients with fMF who presented with a variety of pilosebaceous manifestations and elevated CD4/CD8 ratios and showed distinct clinical courses. A 65-year-old woman presented with a 6-month history of multiple, discrete, reddish papules and comedones on the face. On examination, numerous reddish, follicular papules were present on the trunk and extremities (figures 1A-B), and closed comedones or milia were observed on the glabella. Hairs were loose in the scalp and eyebrows. Small lymph nodes were palpable on the neck, axillae and inguinal areas. Routine laboratory tests showed a white blood cell count of 7,690/␮L with 1,614/␮L atypical lymphocytes (21% of all lymphocytes). Biopsy specimens from the reddish papules revealed dense infiltration of lymphocytes in and around the hair follicles (figure 1C) and eccrine sweat ducts (figure 1D), without epidermotropic infiltration in the interfollicular epidermis. The swollen lymph nodes showed histopathologic features of dermatopathic lymphadenitis (N1b) with clonal rearrangement of T-cell receptor (TCR) C␤ genes by Southern blotting. The CD4/CD8 ratio in the peripheral blood lymphocytes was 5.5, with increased percentages of central memory cells expressing CD4+CD7- (76.0%), CD4+CD26(76.9%), CD45RA-CD27+ (92.0%), and CD45RACD62L+ (55.9%). The patient was diagnosed as having leukemic CTCL (T2N1M0B2, stage IVA1) with fMF and was treated with psoralen plus ultraviolet A (PUVA), narrowband ultraviolet B and etretinate, with little improvement. Three years after the onset, the patient’s eruptions coalesced to cover more than 80% of the body surfaces (figure 1E) associated with lymph node involvement, the features of which were consistent with those of Sézary syndrome. The patient responded to monotherapy with etoposide or vorinostat initially but the illness exacerbated to require intensive polychemotherapy. A 76-year-old woman had been treated by a nearby doctor for generalized eruptions for more than 10 years. On

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H

Figure 1. Case 1. Numerous, reddish, follicular papules on the thigh (A, B). Dense infiltration of lymphocytes in and around the hair follicles and eccrine sweat duct (C, D) (hematoxylin-eosin, original magnification ×40). Generalized scaly, confluent erythema on the thigh 3 years after the onset (E). Case 2. Follicular-pointed papules and comedones (F), the dermoscopic findings (G), and follicular horn spikes on the earlobe (H).

examination, several erythematous patches and plaques were present on the trunk and extremities and indurated plaques with xanthomatous changes and a dark-reddish tumor, 25 mm in diameter, were noted on the left forehead. In addition to these cutaneous lesions, the patient presented with open comedones on the face, follicular papules on the trunk and extremities (figures 1F-G), and follicular horn spikes on the arms and earlobes (figure 1H). Skin biopsy specimens from the follicular lesion demonstrated folliculotropic infiltration of lymphocytes with mild follicular degeneration and mucinosis. No abnormalities were observed in routine blood tests. Flow cytometric analysis, however, revealed increased CD4/CD8 ratios, ranging from 5.7 to 8.8, and CD4+CD7- and CD4+CD26- fractions were 4.59% and 10.0%, respectively. No clonal proliferation of T cells was observed by polymerase chain reaction (PCR)-based TCR analysis or by Southern blotting. The patient was diagnosed as fMF (T3N0M0B0, stage IIB) with less pathogenic significance in hematological abnormalities. In addition to surgical resection of the tumor on the forehead, a combination of PUVA, excimer light treatment and etretinate was effective. To date, the patient has remained in complete remission for 2 years.

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The two patients described here presented with generalized, discrete follicular papules associated with various pilosebaceous manifestations, including comedo-like or mila-like lesions, loss of scalp and eyebrow hair and lichen spinulosa-like horn plugging. These clinical features, taken together with the histopathologic findings, were consistent with fMF in a strict sense. The first case met the B2 criteria for overt leukemic phase of central memory T cells, but a diagnosis of Sézary syndrome was excluded at the first visit because of the absence of erythroderma. Three years after the onset, however, the patient progressed to Sézary syndrome with erythroderma. Our case, taken together with previous reports [5, 6], suggests that the leukemic stage of B2 is often associated with fMF. In contrast, our second patient showed polyclonal proliferation of CD4+ T cells and has been in good health for 2 years without progression to leukemia. Our patients with fMF have taught us that, in addition to the CD4/CD8 ratio, the nature of proliferating CD4+ T cells should be examined and monitored carefully.
Disclosure. Acknowledgements: We would like to thank medical students (Yurie Murata, Hironobu Yamaoka, Kosuke Yunoki, Yuya Yokota, Mikiko Watanabe, Yusuke Nakata) for obtaining the clinical data from the patients. Financial support: This work was partially supported by grants from the Ministry of Health, Labour and Welfare : H21-Clinical Cancer Research-023 (Principal Investigator, Koichi Hirata), H23-Clinical Cancer Reseaarch-021 (Principal Investigator, Toshiki Watanabe), and H23-Clinical Cancer Research-020 (Principal Investigator, Kaoru Uchimaru). Conflict of interest: none. 1

Department of Dermatology, Department of Laboratory Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama 700-8558, Japan 2

Kazuko MIZUNO-IKEDA1 Toshihisa HAMADA1 Daisuke SUZUKI1 Shin MORIZANE1 Toshiyuki WATANABE2 Masahide IMADA2 Ken OKADA2 Keiji IWATSUKI1,2

1. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768-85. 2. Muniesa C, Estrach T, et al. Folliculotropic mycosis fungoides: clinicopathological features and outcome in a series of 20 cases. J Am Acad Dermatol 2010; 62: 418-26. 3. Gerami P, Rosen S, Kuzet T, et al. Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008; 144: 738-46. 4. Lehman JS, Robert H, Cook-Norris H, et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010; 146: 607-13. 5. Herrmann F, Wirthmuller R. Cell-surface-marker phenotyping in patients with leukemic non-Hodgkin lymphomas (NHL) of low and intermediate malignancy. Immunobiology 1982; 163: 77-94. 6. Mehta A, Dhungel BM, Khan MF. Mycosis fungoides/Sezary syndrome: report of an unusual case. J Cutan Pathol 2006; 33 (Suppl 2): 12-5. doi:10.1684/ejd.2014.2270

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