case4

Mycosis Fungoides mimic chronic eczema? Observation

Jacek Andruszkiewicz¹, Piotr Brzezinski²

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma [1]. Because of its great variety of clinical features and nonspecific histological findings (especially in early stages) has been named the "great imitator "and can induce many wrong diagnosis [2,3]. Mycosis fungoides (MF), is an epidermotropic lymphoma included as an indolent form in the recent WHO/EORTC classification. From a clinical point of view, the classic disease progression usually is slow and takes over years or even decades, and characterized by the evolution from patches to more infiltrated plaques and eventually to tumours or erythroderma. However, the analysis of the MF disease course has been greatly impaired by the rarity of the disease, thus data about the time course of disease progression and pattern of relapse during time are not well known [4,5]. Therefore very often Mycosis fungoides is misdiagnosed as chronic eczema [6]. MF can also mimic: vitiligo [6], alopecia-Areata [7], ecchymosis [8].

 

Case Report

A 60-years-old male pensioner presented with multiple erythrematoses plaque with fine scales all over his body of 2 years; with periodic pruritus (Fig. 1-6). On examination, there were multiple erythrematoses plaque over trunk and limbs. There was no lymphadenopathy. Lesions had not responded to long-term use of various medications: potent topical steroids, emollients and immunosuppressive therapy Tacrolimus. The patient had no personal history of atopic dermatitis, psoriasis or causative drug intake. No personal or heredo-collateral history of allergic events, no new drugs intake, no contact with substances that can induce allergic or irritant contact dermatitis.

Since 21. April 2013 colorectal cancer, radiation therapy.

 

Figure 1. Clinical image	Figure 2. Clinical image
Figure 3. Clinical image	Figure 4. Clinical image
Figure 5. Clinical image	Figure 6. Clinical image

 

Routine investigations: CRP – 24,4 (norm 5); monocytes – 12,4% (norm 12,3); RBC – 4,28 (norm 4,5-5,7); C protein – 6,20 (norm 6,40-8,30); albumin-52,57 (norm 53-60); globulin alpha 1 – 4,67 (norm 2-5,5); globulin alpha 2 – 13,83 (norm 6-12); globulin beta 1 – 10,69 (norm 9,2-12,5); globulin beta 2 – 5,28 (norm 2,9-4,5); globulin gamma – 12,96 (norm 11-21). Chest X-ray was normal. Biopsy from the skin showed in dermal small infiltrate of lymphocytes and eosinophils. Histology is not fully diagnostic (Fig. 7-10 ).

 

 

Figure 7. Histopathological image	Figure 8. Histopathological image
Figure 9. Histopathological image	Figure 10. Histopathological image

 

Discussion

The predominant CTCL subtype is mycosis fungoides. It corresponds to the cutaneous lymphoma originated in the peripheral epidermotropic T-lymphocyte, which expresses the T-cell receptor (TCR) with αβ+subunits and CD4+ immunophenotype, known as memory T-lymphocyte (CD45RO+), and constitutes the skin immunosurveillance. The designation MF can only be used for classic cases characterized by the development of patches, plaques and tumors and for variants with a similar clinical course. MF represents less than 1% of the total number of non-Hodgkin lymphomas; however, it is the most common cutaneous lymphoma. It usually has an indolent course and good prognosis when identified in its early stages [1]. The classic form of MF, which is also referred to as Alibert-Bazin, and three variants, namely, the "folliculotropic", "pagetoid reticuloid" and "granulomatous cutis laxa" variants, are acknowledged in the WHO-EORTC classification and in the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. Other subtypes have been reported in the literature due to their peculiar clinical, demographic or histological characteristics: "hypopigmented/hyperpigmented", "erythrodermic", "poikilodermic", "pigmented purpura-like", "bullous/dyshidrotic", "papular" and even the "invisible" subtype. The leukemic MF subtype is referred to as SS, with distinct clinical and evolutive expressions [9-11].

From a biological point of view, there is the need to understand the molecular basis of the different clinical pathways of disease progression, to be able to potentially identify at an earlier phase of disease evolution, the patients who are more likely to develop erythroderma or tumour-stage progression. In conclusion, if MF is indeed a true "lion queen", as dermatologists we need to be expert and wise tamers to keep it under control. 

 

 

References

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5. Quaglino P, Pimpinelli N, Berti E, Calzavara-Pinton P, Lombardo GA, Rupoli S, et al: Mycosis fungoides: disease evolution of the "lion queen" revisited. G Ital Dermatol Venereol. 2012;147:523-31.

6. Kumar DJ, Kumar GA: Mycosis fungoides with unusual vitiligo-like presentation. Indian J Dermatol Venereol Leprol. 2004;5:304-6.

7. Farnaghi F, Safaee Naraghi Z, Mohit S: Alopecia-Areata Like Alopecia in Mycosis Fungoides, a Case Report. Tehran Univ Med J. 1998;1;83-85.

8. Mohammad N: Ecchymosis-like hyperpigmented mycosis fungoides. Ann Saudi Med. 2008;3:228-9.

9. Nakamura-Wakatsuki T, Yamamoto T: Periorbital necrobiotic xanthogranuloma without paraproteinemia. Our Dermatol Online. 2013;4:341-3.

10. Kannangara AP, Fleischer AB, Yosipovitch G: The Sparing Phenomenon. A case series of the inverse Koebner and related phenomena. Our Dermatol Online 2013;4:35-9.

11. Abreu Velez AM, Klein AD, Howard MS: Immunologic findings in central centrifugal cicatricial alopecia. Our Dermatol Online 2012;3:202-5.