Unilateral mycosis fungoides
Published Web Locationhttps://doi.org/10.5070/D30963069h
Unilateral mycosis fungoides1. Department of Dermatology, Isfahan University of Medical Sciences, Isfahan, Iran
Fariba Iraji MD1, Ali Asilian MD1, Amir Hossein Siadat MD2, Parvin Rajabi MD3, Afshin Arbabi MD4
Dermatology Online Journal 14 (6): 25
2. Skin Diseases and Lesihmaniasis Research Center, Isfahan University of Medical Sciences, Isfahan, Iran. email@example.com
3. Pathology Department, Isfahan University of the Medical Sciences, Isfahan, Iran
4. Afshin Arbabi, Dermatology Resident, Alzahra Hospital
We present an unusual case of unilateral mycosis fungoides (MF) in a 38-year-old woman. This woman presented to us because of multiple, localized, pruritic confluent plaques that appeared on the right-side of her chest and back 8 years ago. Skin biopsies showed characteristic findings of mycosis fungoides (e.g., Pautrier microabscesses, follicular epitheliotropism, wiry bundles of collagen, etc.). T-cell receptor gene rearrangement analysis in the lesional skin demonstrated rearrangement of the gamma chain. PUVA therapy improved her skin lesions and pruritus, but these plaques progressed again after discontinuation of treatment. We conclude that MF can be localized and unilateral. Thus, a high index of suspicion is necessary for diagnosis of this potentially lethal disease.
Mycosis fungoides (MF) shows a large variety of clinical appearances, including bullous , hypo-  or hyper-pigmented , hyperkeratotic , granulomatous , pustular , pigmented purpuric, verrucous  and pityriasis lichenoides-like lesions. Mycosis fungoides mimicking inflammatory linear verrucous epidermal nevus  has also been reported recently.
However, unilateral MF has not yet been reported. We describe a case in which the lesions were localized to only one side of the body. Histology and T-cell receptor gene rearrangement findings were used for establishing the diagnosis of MF in this patient.
A 38-year-old woman presented with an 8-year history of localized pruritic plaques with mild scaling on the right side of her chest and back (Fig. 1). Her lesions had not responded to long-term use of various medications and ointments. The patient had no personal history of atopic dermatitis, guttate psoriasis, or causative drug intake.
On physical examination, the lesions displayed a unilateral arrangement but did not follow dermatomes or lines of Blaschko. The general physical examination was otherwise unremarkable; there was no lymphadenopathy or organomegaly.
|Figure 1||Figure 2|
|Figure 1. Patches of mycosis fungoides that are restricted to the right side of body|
Figure 2. Closer view of Fig. 1
Human T-cell lymphotropic virus type 1 serology was negative. Laboratory investigations showed a normal complete blood count. Other laboratory examinations including chest X-ray, liver function tests, and electrolytes were normal.
Histopathology examination of a skin biopsy showed psoriasiform hyperplasia, a sharply demarcated alteration of orthokeratosis and parakeratosis in the cornified layer, epidermotrophism, Pautrier microabscess, and haloed lymphocytes in the epidermis. In the upper dermis, there were coarse collagen bundles and a patchy infiltrate, mainly consisting of lymphocytes. Analysis of T-cell receptor gene rearrangement demonstrated rearrangement of the gamma chain.
The histological picture led to the diagnosis of mycosis fungoides. The treatment of the disease was begun with potent topical corticosteroid. After a week, systemic 8-methoxypsoralen plus UVA (PUVA) therapy 3 times weekly was added to this treatment.
The pruritus and skin lesions were partially improved after 8 weeks of therapy, although they were aggravated after discontinuation of the treatment. However, the patient unfortunately failed to appear for further follow-up.
Mycosis fungoides is one of several presentations of forms of cutaneous T-cell lymphoma; it progresses through clinical stages including initial premycotic stage (macules or patches), plaque stage, and finally tumor stage [10-11].
Mycosis fungoides is usually thought of as a disease of the elderly, but it can develop at any age, including childhood . In fact, many cases reported in the literature had historical onset of cutaneous lesions in childhood that have later been histologically diagnosed as MF in adulthood. Mycosis fungoides in children shows a variety of clinical features such as hypopigmented or pytiriasis lichenoides-like MF .These clinical types, however, had histological features consistent with MF (Pautrier microabscesses, epidermotrophic mononuclear cell, accompanied scant spongiosis, numerous cerebriform lymphocytes, lymphocytes aligned as solitary units along the basal layer, epidermal lymphocytes larger than dermal lymphocytes, coarse bundles of collagen in the papillary dermis, and epitheliotropism in the hair follicle .
The diagnosis of clinical variants of MF is often difficult to establish both clinically and histologically . Although immunohistochemical and molecular biological studies have become helpful for establishing this diagnosis, interpretation of routine histological sections remains the most important tool . Our patient showed histopathological features of MF. In addition, the molecular genetic analysis demonstrated the presence of a clonal population of T-cell receptor gamma gene rearrangement. Thus, this case points out a unique manifestation of MF unilateral distribution. In addition, our patient was young when the diagnosis of MF was made.
The mechanism that is responsible for unilateral localization of the MF infiltration is unknown. In addition, although there has been increasing recognition that MF may occur in young children and adolescents, reports of childhood and adolescent MF are relatively rare . Moreover, atypical presentations, such as hypopigmented or pityriasis lichenoides-like MF appear to arise more commonly in young people . Therefore, MF should be considered in the differential diagnosis of chronic dermatoses in young people.
In summary, we conclude that mycosis fungoides can be unilateral. In any patient with chronic atypical eczematous lesions, we recommend long-term follow-up with repeated skin biopsies to rule out mycosis fungoides.
References1. Kartsonis J, Brettschneider F, Weissmann A, Rosen L. Mycosis fungoides bullosa. Am J Dermatopathol 1990; 12: 76-80. PubMed
2. Stone ML, Styles AR, Cockerell CJ, Pandya AG. Hypopigmented mycosis fungoides: a report of 7 cases and review of the literature. Cutis 2001; 67: 133-138. PubMed
3. Kikuchi A, Shimizu H, Nishigawa T. Mycosis fungoides with marked hyperpigmentation. Dermatology 1996; 192: 360-363. PubMed
4. Hannah M, Jacyk WK. Palmoplantar hyperkeratotic plaques. Hyperkeratotic MF restricted to the palms and soles. Arch Dermatol 1998; 134: 1021-1024. PubMed
5. Fischer M, Wohlrab Kartsonis J, Brettschneider F, Weissmann A, Rosen L. Mycosis fungoides J, Audring TH et al. Granulomatous mycosis fungoides. Report of two cases and review of the literature. J Eur Acad Dermatol Venereol 2000; 14: 196-202. PubMed
6. Camisa C, Aulisio A. Pustular mycosis fungoides. Cutis 1994; 54: 202. PubMed
7. Georgala S, Katoulis AC, Symeonidou S et al. Persistent pigmented purpuric eruption associated with mycosis fungoides: a case report and review of the literature. J Eur Acad Dermatol Venereol 2001; 15: 62-64. PubMed
8. Wakelin SH, Stewart EJ, Emmerson RW. Poikilodermatous and verrucous mycosis fungoides. Clin Exp Dermatol 1996; 21: 205-208. PubMed
9. Jang JG, Sim HJ, Kim SH, Doh KS, Jang MS, Suh KS. Mycosis fungoides mimicking inflammatory linear verrucous epidermal nevus. J Eur Acad Dermatol Venereol 2004; 18:218. PubMed
10. Micaily B. Radiotherapy for unilesional mycosis fungoides Int J Rad Oncol Biology and Physics, 1998 ; 42 :61. PubMed
11. Peters MS, Thibodeau SN, White JW Jr, Winkelmann RK. Mycosis fungoides in children and adolescents. J Am Acad Dermatol 1990; 22: 1011 1018. PubMed
12. Ackerman AB, Chongchitnant N, Sanchez J, Guo Y. Simulators of inflammatory disease. In: Ackerman AB, Chongchitnant N, Sanchez J, Guo Y, editors. Histopathologic Diagnosis of Inflammatory Skin Diseases, 2nd ed. William & Wilkins, Baltimore, 1997: 838- 856.
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