Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Several distinct clinical forms of mycosis fungoides have been described. Hypopigmented mycosis fungoides should be regarded as a subtype of mycosis fungoides, insofar as it presents some peculiar characteristics that contrast with the clinical features of the classical form. Most patients with hypopigmented mycosis fungoides are younger than patients typically diagnosed with classical mycosis fungoides. In addition to typical dark-skinned individuals impairment, hypopigmented mycosis fungoides has also been described in Asian patients. The prognosis for hypopigmented mycosis fungoides is much better than for classical mycosis fungoides: hypopigmented mycosis fungoides is diagnosed when there are only patches of affected skin, and lesions usually will not progress beyond terminal stages, although they can persist for many years. Diagnosis should involve clinicopathologic correlation: skin biopsy analysis often reveals intense epidermotropism, characterized by haloed, large, and atypical CD8+ lymphocytes with convoluted nuclei, in contrast to mild to moderate dermal lymphocytic infiltrate. These CD8+ cells, which participate in T helper 1-mediated immune responses, prevent evolution to mycosis fungoides plaques and tumors and could be considered the main cause of the inhibition of melanogenesis. Therefore, hypopigmentation could be considered a marker of good prognosis for mycosis fungoides.     

Hypopigmented mycosis fungoides associated with atopy in two children

Mycosis fungoides is very rare in children. Hypopigmented lesions of this disease are usually observed in dark-skinned individuals and often show a T supressor CD8(+) phenotype. Two Caucasian children with predominantly hypopigmented lesions of mycosis fungoides are presented here. Atopy was a concomitant feature in both.     

Hypopigmented mycosis fungoides. Speculations about the mechanism of hypopigmentation

Hypopigmented, nonatrophic macules are an unusual manifestation of lesions of early mycosis fungoides. Presented herein is a patient with such lesions and an unusual mask-like hypopigmentation of the face. We review both the clinical and microscopic findings in this patient and in previously reported patients with hypopigmented lesions of mycosis fungoides. Finally, speculation is made as to the mechanisms of the hypopigmentation.     

Hypopigmented mycosis fungoides

INTRODUCTION: Hypopigmented mycosis fungoides is a rare clinical form of the disease, first described in 1978. Since then, only a hundred odd cases have been documented. CASE REPORT: A young 19 year-old African woman had presented with hypochromatic macules since the age of 9 and for which the diagnostic enquiry had finally concluded in hypopigmented mycosis fungoides. DISCUSSION: The particularities of this form of mycosis fungoides, grade I according to the TNM classification, are principally its onset in black-skinned persons or of Asian origin, and the age of early onset with a predominance of pediatric cases. Its course is indolent for several years and thus source of delayed diagnosis. The differential diagnoses to be evoked are basically vitiligo, achromate eczematides and parapsoriasisis. The post-inflammatory depigmentation, frequent in black-skinned subjects, is only an eliminating diagnosis. Its treatment is that of classical Grade I mycosis fungoides: topical mechlorethamine, phototherapy and topical corticosteroids.     

Hypopigmented mycosis fungoides treated successfully with puva

Hypopigmented lesions are rarely encountered in mycosis fungoides. We here report a 22-year old female patient who presented with a 5-year history of asymptomatic progressively increasing discrete and confluent hypopigmented macules and a 1-year history of a few itchy erythematous, scaly, indurated plaques. The histological features were consistent with a clinical diagnosis of mycosis fungoides. She was successfully treated with PUVA therapy.     

Hypopigmented mycosis fungoides in Caucasian patients: a clinicopathologic study of 7 cases

BACKGROUND: Hypopigmented mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned or Asian patients, particularly children. Only 9 cases in Caucasian patients have been reported in the literature so far. OBSERVATION: We describe 7 Caucasian patients (2 children and 5 adults) with hypopigmented MF. Histologic examination confirmed the diagnosis in all cases. The phenotype of neoplastic lymphocytes was T helper in 4 cases and T suppressor in 3 (2 of them children). Monoclonality of the T lymphocytes could be detected in hypopigmented lesions in all 7 cases with the use of a polymerase chain reaction technique. In 4 patients, polymerase chain reaction analysis of T-cell receptor-gene rearrangement after laser-based microdissection of the specimen revealed that the monoclonal population of T lymphocytes was confined mainly to the epidermis. CONCLUSION: Hypopigmented lesions of MF can be observed in Caucasian patients. Children affected by MF often present with this rare clinical variant of the disease. Persistent or unusual hypopigmented lesions should be subjected to biopsy to avoid delay in the diagnosis of MF, especially in children.     

Hypopigmented mycosis fungoides: case reports and literature review

BACKGROUND: Hypopigmented mycosis fungoides is a rare variant of mycosis fungoides (MF) that usually has a predilection for young individuals with dark complexion. OBJECTIVE: The aim is to describe new cases of hypopigmented MF with confirmed T-cell receptor gene rearrangement analysis. METHODS: This article includes case reports and a literature review. RESULTS: Three out of four hypopigmented MF patients had a positive TCR gene rearrangement. A fifth patient is reported who had hypopigmented mycosis fungoides and classical Pautrier microabscesses, for whom no TCR gene rearrangement analysis was performed. CONCLUSION: Although hypopigmented MF has a predilection for dark-complexioned populations, it can also affect Caucasian patients. In challenging cases, polymerase chain reaction can be a useful method for detecting early cases of hypopigmented MF.     

A case of hypopigmented mycosis fungoides successfully treated with 311 nm narrowband ultraviolet B phototherapy

Hypopigmented mycosis fungoides (HMF) is a rare clinical variant of mycosis fungoides (MF) characterized by hypopigmented lesions involving most commonly trunk and proximal extremities. We report here a case of HMF in 22-year-old Korean woman successfully treated with 311 nm narrow-band ultraviolet B (NB-UVB) phototherapy. She presented with progressive asymptomatic hypopigmented patches on wholebody 1-year ago and diagnosed as HMF based on clinicopathologic findings. She was treated with NB-UVB phototherapy and showed almost complete clearance after 8-month without any side effects.     

Leucoderma associated with flares of erythrodermic cutaneous T-cell lymphomas: four cases. The French Study Group of Cutaneous Lymphomas

We describe four patients with erythrodermic cutaneous T-cell lymphomas (two with erythrodermic mycosis fungoides, and two with Sézary syndrome) who presented with extensive hypopigmented lesions that occurred during flares of their cutaneous disease. These cases must be distinguished from previously described hypopigmented mycosis fungoides where hypopigmented lesions were the sole manifestation of the lymphoma. In two cases a biopsy was performed on hypopigmented skin, showing an infiltrate of atypical lymphocytes with epidermotropism and absence of melanocytes, as in vitiligo. It is suggested that the hypopigmentation could be due to the cytotoxicity of tumour or reactional lymphocytes directed against melanocytes.     

Mycosis fungoides with unusual vitiligo-like presentation

Mycosis fungoides (MF), the commonest variant of primary cutaneous T cell lymphoma (CTCL), is relatively uncommon among the Asians. Hypopigmented mycosis fungoides is a rare variant usually observed in dark-skinned individuals, especially children. Hypopigmented MF usually responds well to therapy, particularly to PUVA, and has a comparatively benign course. Mycosis fungoides in a 16-year-old boy, with extensive asymptomatic hypopigmented lesions developing gradually all over the body over eight years and vitiligo-like skin lesions developing for seven years, with no systemic features, is presented for its unusual clinical features and conspicuous histopathological findings of prominent epidermotropism. The case showed fairly good response to PUVASOL therapy.     

Juvenile mycosis fungoides diagnosed before 18 years of age

The literature regarding mycosis fungoides in children is sparse. To shed further light on the characteristics of mycosis fungoides in the paediatric population we analysed the clinicopathological features of 10 patients in whom this malignancy was diagnosed before the age of 18 years. All were Jews and Arabs with histologically proven patch/early plaque stage disease: 4 in stage IA, 4 in IB and 2 with unilesional disease. Seven patients had hypopigmented lesions either constituting the sole manifestation (2 patients) or in combination with classic lesions (5 patients); of these, 3 had light skin and 4 pigmented skin. Most patients had immunohistochemical features characteristic of mycosis fungoides, with a predominance of CD4+ T cells. Some had deletion of CD7+ cells. In 3 patients, however, the epidermotropic cells were exclusively or predominantly CD8+ cells. All patients responded to conventional therapy and during an average follow-up of 3.4 years only one patient showed stage progression, but without extracutaneous involvement. It is concluded that juvenile mycosis fungoides is characterized by early stage disease, occasionally with unilesional disease, usually with hypopigmented lesions irrespective of skin colour, and a good response to therapy. On the basis of our experience and review of the literature, it appears that the CD8+ phenotype is over-represented in juvenile disease.     

Hypopigmented mycosis fungoides in a 10-year-old boy

Cutaneous T-cell lymphoma (CTCL) presenting with hypopigmented lesions is an uncommon clinical variant of the disease, usually described in dark-skinned patients. We report a case of hypopigmented CTCL in a 10-year-old boy. The disease has responded favorably to narrowband UVB therapy. This case illustrates the importance of clinical suspicion for mycosis fungoides in patients with widespread hypopigmentation.     

Annular hypopigmented mycosis fungoides: a novel ringed variant

Hypopigmented mycosis fungoides (MF) is a relatively uncommon variant of cutaneous lymphoma that is mostly seen in darker skin types. We present a novel and unique clinical presentation in an African-American female patient, consisting of regular hypopigmented annular rings in areas of normal skin and in more typical hypopigmented patches of MF. The lesions appeared diffusely on all extremities, anterior chest and back. Histopathologic examination showed an atypical lymphocytic infiltrate at the dermal-epidermal junction with epidermotropism and few Pautrier's collections. The patient was otherwise healthy and improved with narrowband ultraviolet (UV)-B. This case represents a presentation of a most unusual variant of hypopigmented MF, for which we propose the name 'annular hypopigmented MF'.     

Efficacy of narrowband ultraviolet B twice weekly for hypopigmented mycosis fungoides in Asians

Hypopigmented mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma. To date, there have been no data published about the efficacy of a twice-weekly regimen of narrowband ultraviolet B (NB-UVB) for the treatment of hypopigmented MF. We retrospectively reviewed 11 patients with hypopigmented MF who were treated with NB-UVB twice weekly between 2001 and 2010. Of the 11 patients, 7 achieved a complete response with a mean of 40 treatments; the remaining 4 patients had a partial response. Upon discontinuation of treatment, three patients had clinical relapse after complete remission. Median time to relapse was 10 months. A twice-weekly regimen of NB-UVB is an effective treatment for hypopigmented MF with minimal side-effects. However, the relapse rate is high, and unfortunately, no clinical or histological features can predict the relapse of the disease.     

Hypopigmented interface T‐cell dyscrasia: A form of cutaneous T‐cell dyscrasia distinct from hypopigmented mycosis fungoides

Hypopigmentation in cutaneous T‐cell lymphoproliferative disease should not always be equated with hypopigmented mycosis fungoides (MF). A form of hypopigmented pre‐lymphomatous T‐cell dyscrasia falling under the designation of the so‐called hypopigmented interface variant of T‐cell dyscrasia has recently been proposed. The aim of the present study was to establish hypopigmented interface T‐cell dyscrasia as its own entity apart from other T‐cell dyscrasias and MF using a patient case series. Twenty four cases of hypopigmented interface T‐cell dyscrasia were identified in the dermatopathology database of Weill Medical College of Cornell University. There were 17 females and seven males (mean age, 36 years). In children and adolescents, the patients were most commonly of African American extraction. Truncal photo‐protected areas manifesting as large solitary patches or multiple smaller macules were characteristic; disease progression to MF occurred in only one patient. The lesions responded to topical steroids and light therapy. The pathology was defined by a cell poor interface associated with degeneration of keratinocytes and melanocytes, and by lymphocytes whose nuclei showed low‐grade cerebriform atypia, and which expressed a significant reduction in CD7 and CD62L expression. In 50% of the cases, the implicated cell type was of the CD8 subset. Clonality was not identified. Hypopigmented interface T‐cell dyscrasia is a distinct entity separate from and rarely progressive to MF.     

"Hypopigmented mycosis fungoides" is not always mycosis fungoides!

We conducted a critical review of hypopigmented mycosis fungoides in historical perspective with emphasis on criteria clinical and histopathologic for diagnosis of that lymphoma as they are set forth in every article ever written about it. Toward that end, we undertook analysis of each article in the medical literature that mentioned hypopigmentation in mycosis fungoides (34 in toto). Each was scrutinized regarding content, photographs of lesions clinical pictured, and photomicrographs. On the basis of all the information in the 34 publications available to us, we made a determination about which patients had mycosis fungoides without doubt, which surely did not, and which about whom no judgment could be made by us because too little data requisite for such a decision was provided, especially in terms of photographs of lesions clinical and of photomicrographs. To date, 106 patients with "hypopigmented mycosis fungoides" have been reported on. Features clinical and findings histopathologic in 23 of those 106 patients were sufficient to permit us to determine, with a high degree of confidence, whether or not a particular patient truly had mycosis fungoides. In our judgment, 19 patients did have mycosis fungoides, whereas at least four patients did not. In regard to the other 83 patients, the information provided by the authors simply was not sufficient to allow us to come to a decision that we could justify.     

Unusual Clinical Presentation of Epidermotropic Cutaneous Lymphoma:Small Hypopigmented Macules

A 35-year-old black woman developed numerous vitiligo-like small hypopigmented macules on the upper and lower extremities. Histologically the lesions were consistent with epidermotropic mycosis fungoides or pagetoid reticulosis. The patient is presented because of a clinical picture unusual for either of the above microscopic diagnoses.     

Unusual Clinical Presentation of Epidermotropic Cutaneous Lymphoma

Abstract: A 35-year-old black woman developed numerous vitiligo-like small hypopigmented macules on the upper and lower extremities. Histologically the lesions were consistent with epidermotropic mycosis fungoides or pagetoid reliculosis. The patient is presented because of a clinical picture unusual for either of the above microscopic diagnoses.     

儿童色素减退型蕈样肉芽肿1例

 报告色素减退型蕈样肉芽肿1例。患儿男,4岁。全身散发绿豆大小色素减退斑半年余,加重1个月。皮肤科检查：颈部、胸、背及四肢可见多发类圆形或圆形色素减退斑,境界清楚,未见融合,部分白斑中央见暗红色丘疹及斑片,表面干燥,覆盖白色鳞屑。左手背有两颗高于皮面黑色斑丘疹。皮损组织病理：表皮灶性角化不全,基底细胞液化变性,淋巴样细胞进入表皮内,少许核扭曲,见空晕,真皮浅层较多淋巴组织细胞苔藓样浸润,少许细胞轻异性。免疫组化提示CD20散在少量细胞（+）、CD79a散在少量细胞（+）、CD3（+）、CD4少量细胞（+）、CD5（+）、CD7大部分细胞（+）、CD8（+）、CD4与CD8比例为1 ∶ 4;CD30约30%细胞（+）、CD68散在少量细胞（+）、TIA 1（+）、Ki 67约30%细胞（+）;CD7表达减弱。EBER原位杂交：（-）。根据临床表现、组织病理和免疫荧光,诊断为色素减退型蕈样肉芽肿。予盐酸氮芥溶液治疗,后失访。