|Year : 2012 | Volume
| Issue : 1 | Page : 55-57
Mycosis fungoides: A great mimicker
A Prasad Chowdary1, C Lakshmi Sowjanya1, S Satyanarayana2, G Raghu Rama Rao1, K Sandeep1, A Krishna Phanindra Prasad1
1 Department of DVL, GSL Medical College and General Hospital, Lakshmipuram, Rajanagaram, Rajahmundry, India
2 Department of Pathology, GSL Medical College and General Hospital, Lakshmipuram, Rajanagaram, Rajahmundry, India
|Date of Web Publication||21-Mar-2012|
G Raghu Rama Rao
Department of DVL, GSL Medical College and General Hospital, Rajahmundry - 533 294
Source of Support: None, Conflict of Interest: None
Mycosis fungoides (MF) mimics a variety of dermatoses. A case of hypopigmented MF, a rare variant mimicking leprosy and vitiligo, is reported.
Keywords: Hypopigmented mycosis fungoides, leprosy, vitiligo
|How to cite this article:|
Chowdary A P, Sowjanya C L, Satyanarayana S, Rama Rao G R, Sandeep K, Phanindra Prasad A K. Mycosis fungoides: A great mimicker. J NTR Univ Health Sci 2012;1:55-7
|How to cite this URL:|
Chowdary A P, Sowjanya C L, Satyanarayana S, Rama Rao G R, Sandeep K, Phanindra Prasad A K. Mycosis fungoides: A great mimicker. J NTR Univ Health Sci [serial online] 2012 [cited 2020 Jul 15];1:55-7. Available from: http://www.jdrntruhs.org/text.asp?2012/1/1/55/94178
| Introduction|| |
Mycosis fungoides (MF), a rare cutaneous T cell lymphoma, mimics several dermatoses. In addition to the classical presentation of eczematous patches, infiltrated plaques, and tumors, several atypical forms like hypopigmented MF, follicular, purpuric, palmoplantar form, granulomatous slack skin, and poikilodermatous forms have been reported.  Hypopigmented MF is an under-recognized variant of MF, and may resemble other common hypopigmented dermatoses like vitiligo, leprosy and postinflammatory hypopigmentation.  Very few cases of hypopigmented MF have been reported from India. We are reporting a rare case of hypopigmented MF in a 34-year-old man.
| Case Report|| |
A 34-year-old healthy man presented with multiple asymptomatic hypopigmented patches over the body for 9 years. These lesions started appearing over the back and gradually spread to other areas. The lesions were initially hypopigmented, and for the last 2 years, the patient noticed erythema and pigmentation over some patches. There were no constitutional symptoms and no other associated diseases. No other family member had similar lesions. On examination, there were multiple ill-defined hypopigmented patches over the back, abdomen, arms, and forearms [Figure 1]. Poikilodermatous changes like erythema, telangiectasia, and pigmentation were seen in some patches. All sensations were intact over the patches, and peripheral nerves were normal and not thickened. There was no lymphadenopathy and organomegaly. Other systems were normal. All hematological and biochemical investigations were within normal limits. Serological tests like VDRL and HIV serology were negative. Skiagram of chest and ultrasound of abdomen were normal. The differential diagnoses considered were vitiligo, borderline leprosy, and postinflammatory hypopigmentation. Slit skin smears revealed no acid-fast bacilli. Lesional biopsy revealed a dense monomorphic lymphoid infiltrate at the dermoepidermal junction with extension to dermis as single files. Epidermotropism was seen [Figure 2] and [Figure 3]. Immunohistochemistry (IHC) showed positivity for pan T cell marker (CD 20 + ) [Figure 4]. Based upon duration, clinical features, and histopathology, a diagnosis of hypopigmented variant of MF was made. The patient was treated with narrowband ultraviolet B therapy (NB UVB), with starting dose of 150 mJ/cm 2 and increments of 20 mJ/cm 2 on alternate exposures. After 30 exposures, majority of the lesions regressed and the patient is under follow-up.
|Figure 2: Atypical lymphocytic infiltrate of the upper dermis and dermoepidermal junction (H and E, ×10)|
Click here to view
|Figure 3: Large atypical lymphocytes with perinuclear halo in the epidermis (H and E, ×40)|
Click here to view
| Discussion|| |
The term "hypopigmented MF" was mentioned for the first time by Ryan et al., in 1973.  This variant of MF is more commonly seen in children and dark-skinned individuals, especially in Asians, and occurs at a much earlier age, usually in the third decade, when compared to the classical MF.  Male preponderance is observed. Clinically, the hypopigmented patches are asymptomatic, not sharply circumscribed, and have a slightly dry surface. Mild atrophy may be appreciated. Some of these patches may develop focal hyperpigmentation, resulting in early poikilodermatous changes in the centre. One recent study of MF revealed that the most common clinical presentation in patients with MF was patch disease (68%) with 59% presenting as hypopigmented patches. 
Hypopigmented MF lesions were considered to be the earliest and benign lesions, and they were quite often clinically mistaken for other hypopigmented lesions like leprosy, vitiligo, and postinflammatory hypopigmentation. , Therefore, a high degree of clinical suspicion is required to identify this rare variant of MF. Histopathology, IHC, and T cell receptor gene re-arrangement studies are helpful in arriving at an accurate diagnosis. , The essential histopathological features of hypopigmented MF are scattered lymphocytes in the basal epidermis (epidermotropism), with or without Pautrier's microabscesses. The occurrence of melanin incontinence is also seen. , Ultrastructural studies show focal invasion of the epidermis by mycosis cells with degenerative changes in adjacent melanocytes and keratinocytes, which explains the occurrence of melanin incontinence and hypopigmentation in hypopigmented MF.  IHC showed predominantly cytotoxic CD8 + lymphocytes in the epidermis, unlike classical MF, which usually shows CD4 + T cells. ,
Hypopigmented MF shows response to treatment with topical corticosteroids, UVB, PUVA, topical nitrogen mustard, or topical carmustine. Our case has also responded very well to NB-UVB therapy.
Majority of workers believe that hypopigmented variant of MF is early and benign form of MF. It confers a better prognosis than classical MF. ,,, It seems to have a favorable, indolent course, and even without treatment the lesions remain for a long period without transforming into other aggressive forms like infiltrated plaques and tumors.
In our clinical scenario of common hypopigmented conditions like leprosy, vitiligo, and others, a high index of suspicion is needed to diagnose this uncommon variant of MF.
| References|| |
|1.||Assaf C, Sterry W. Cutaneous Lymphoma. In, Klaus Wolff editor. Fitzpatrick's Dermatology In General Medicine, seventh ed. Mc Graw Hill Medical; 2008. p. 1386-402. |
|2.||Khopkar U, Doshi BR, Dongre AM, Gujral S. A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides. Indian J Dermatol Venereol Leprol 2011;77:167-73. |
|3.||Ryan EA, Sanderson KV, Bartak P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis. Br J Dermatol 1973;88:419-29. |
|4.||Inchara YK, Rajalakshmi T. Early mycosis fungoides vs inflammatory mimics: How reliable is histology? Indian J Dermatol Venereol Leprol 2008;74:462-6. |
|5.||Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams J, et al. Mycosis fungoides in the pediatric population: Report from an International Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010;14:1-6. |
|6.||Lambroza E, Cohen SR, Phelps R, Lebwohl M, Braverman IM, DiCostanzo D. Hypopigmented variant of mycosis fungoides: Demography, histopathology, and treatment of seven cases. J Am Acad Dermatol 1995;32:987-93. |
|7.||Stone ML, Styles AR, Cockerell CJ, Pandya AG. Hypopigmented mycosis fungoides: A report of 7 cases and review of the literature. Cutis 2001;67:133-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]