Unilesional Mycosis fungoides

Abstract

Background: Unilesional mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma (CTCL), characterized by a solitary lesion clinically and by histopathological features indistinguishable from those of MF, and typically having a benign course. Objective: To describe the clinicopathological features of a series of patients with unilesional MF. Methods: The records of cases of unilesional MF identified during a 10-year period in two medical departments were reviewed. Results: There were 7 patients: 6 males, 1 female; mean age at the time of diagnosis: 32 years; age range: 12–58 years; 3 were below the age of 18 years. The mean pretherapy follow-up period was 9 years (range: 2–20 years). In 5 patients, the eruption consisted of a characteristic patch or plaque of MF located on the trunk or upper extremity; in 2 it was atypical – in 1, a hypopigmented patch, and in 1, a plaque indistinguishable from MF-associated follicular mucinosis. Histopathologically all the lesions exhibited features characteristic of MF, with CD3+ lymphoid cells. In 6 cases (with available fresh frozen tissue) there was a predominance of CD3+ CD4+ cells; in 1 of 5 there was deletion of CD7, and in 3 of 5 there was an overexpression of IL-2 receptor. T-cell receptor γ gene rearrangement was found in 1 of 4 cutaneous lesions tested; in 2 cases it was found in the blood but not in the skin. Treatment modalities included localized electron beam, excision, topical nitrogen mustard or topical steroids and sunbathing, resulting in all cases in a sustained complete clinical response. In 1 patient, however, there were 2 local recurrences and in yet another patient there was distant cutaneous spread 3.5 years after therapy. Conclusions: Unilesional MF is a rare variant of CTCL, has heterogeneous clinical manifestations and can affect any age group, including children. The histopathological and immunophenotypical features are in general indistinguishable from those observed in multilesional MF. Although it is a unifocal event, there may occasionally be cutaneous spread with the appearance of noncontiguous lesions, even a long time after therapy. Whether all cases represent minimal-stage IA MF or whether some are actually T-cell pseudolymphoma remains to be clarified.

Ichthyosiform mycosis fungoides: An atypical variant of cutaneous T-cell lymphoma

“…In 5 of these 6 patients CD3 ϩ cells showed a deletion of CD7 (Ͻ30% of the T cells). 3 In 1 patient, however, there was a predominance of CD3 ϩ CD8 ϩ in the epidermis, the number of CD8 ϩ T cells being 3-fold that of the CD4 ϩ T cells. In this patient most of the CD3 ϩ T cells showed deletion of CD7.…”

“…Apart from the classic Alibert-Bazin type of MF (patch, plaque, tumor) many atypical variants have been reported, including hyperpigmented, 1 hypopigmented, 2 unilesional, 3 and follicular 4-6 MF. Acquired ichthyosis, resembling ichthyosis vulgaris or sometimes the less specific ichthyosiform eruption, usually begins in adult life and constitutes a cutaneous sign pointing toward a wide range of underlying causes, mostly malignancies.…”

Hyperpigmented mycosis fungoides: An unusual variant of cutaneous T-cell lymphoma with a frequent CD8+ phenotype

“…It is usually characterized immunohistochemically by a clonal proliferation of CD4 1 T cells or, less often, by a CD8 1 or CD4/CD8 double negative (CD4 À CD8 À ) phenotype. 1 Apart from the classic patch/plaque, AlibertBazin type of MF, several clinicopathological variants and subtypes have been described, 2 including follicular, [3][4][5] hypopigmented, 6,7 unilesional, 8 and ichthyosiform 9 MF. In recent years, there have been several published reports of cases in which MF was manifested solely or predominantly by hyperpigmented patches or plaques.…”

Mycosis-fungoides-Type Cutaneous T Cell Lymphoma of the Hands and Soles: A Variant Causing Delay in Diagnosis and Adequate Treatment of Patients with Palmoplantar Eczema

“…While MF classically presents with cutaneous lesions in unexposed areas with varied preference, distinct variants may show skin involvement restricted to defined areas only. The latter forms typically reveal a long benign course of disease and a lack of dissemination and extracutaneous spread with a favorable prognosis [6, 7, 8, 9, 10]. These variants have largely been referred to as localized pagetoid reticulosis (Woringer-Kolopp disease) [11, 12]or unilesional MF [9, 10, 13].…”

“…The latter forms typically reveal a long benign course of disease and a lack of dissemination and extracutaneous spread with a favorable prognosis [6, 7, 8, 9, 10]. These variants have largely been referred to as localized pagetoid reticulosis (Woringer-Kolopp disease) [11, 12]or unilesional MF [9, 10, 13]. Whereas histologically those cases are very similar or indistinguishable from epidermotropic MF-type CTCL [13, 14], immunohistochemically localized variants may differ to some extent.…”