Abstract

---

Mycosis fungoides is the most common subtype of cutaneous lymphomas. Many clinical variants exist including follicular, icthyosiform, poikilodemic, bullous, mucinous, granulomatous, pustular and pigmented purpura like lesions. We herein report a case of Poikilodermatous mycosis fungoides in a male patient. Clinical rarity of this entity prompted this communication.

Introduction

---

Poikiloderma denotes pigmentation, telangiectasia and atrophy of skin. These changes occur in many dermatological disorders like Rothmund Thompson syndrome, Dermatomyositis, Lupus erythematosus and Mycosis fungoides.
Poikilodermatous MF is a rare form of cutaneous T-cell lymphoma that is characterized clinically by localized or diffuse patches, consisting of telangiectasia, mottled hyper- and hypopigmentation, and atrophy. The immunophenotype of neoplastic cells is similar to that observed in classical mycosis fungoides.

Case Report

---

A 45 yr old male businessman, normotensive, non-diabetic reported to the out-patient department of dermatology, SMHS hospital (associated teaching hospital of Government Medical College Srinagar) with one month history of generalized pruritus and burning sensation associated with dryness of skin and progressive wrinkled pigmentation of trunk and limbs. He also complained of multiple swellings over left side of neck from the same duration, which were insidious in onset, painless and non-tender. He also gave a history of decreased appetite and generalized weakness. The patient?s medical and surgical history was otherwise unremarkable. There was no history suggestive of photosensitivity. The patient also denied any systemic or topical medication prior to the onset of these lesions.
General physical examination of the patient was normal. Cutaneous examination revealed generalized erythema with scaling over trunk, face and extremities. Poikilodermatous changes were observed, with reticulate pigmentation, telangiectasia, intermingled with atrophic wrinkled scaly hypopigmented macules. These changes were more marked on the anterior chest ( Illustration 1), neck, upper back, upper and lower limbs (Illustration 2). Scarring alopecia was present along the scalp margin (Illustration 3) with diffuse scaling of scalp.Nails and mucosae were normal. There was also left cervical lymphadenopathy. The lymph nodes were multiple, firm, discrete, non-tender, mobile, largest being 4 x 4 cm. There? was no axillary or inguinal lymphadenopathy.Systemic examination of the patient revealed no abnormality.
Complete blood count, liver, renal and thyroid function tests were normal.Blood smear for Sezary cells was negative. ANA and anti ds DNA were also negative. Chest X-ray was normal and ultrasonography of the abdomen revealed mild spleenomegaly.Skin biopsy was taken from the back which revealed epidermal atrophy with a band like dermal infiltrate of medium sized atypical lymphoid cells, which show focal epidermotropism (Illustration 4). Immunohistochemistry was also done revealing CD 3+, CD4+, CD45RO+ and CD7-? pattern in the dermal infiltrate typical of mycosis fungoides.
In view of the clinical, histopathological and immunohistochemical findings in this patient, a diagnosis of Poikilodermatous MF was entertained and the patient was referred to medical oncology for further evaluation and management.

Discussion

---

Mycosis fungoides represents the most common type of cutaneous T-cell lymphoma(1). Traditionally, it is divided into three clinical stages: patch, plaque and tumour stage. The clinical course can be protracted over a period of years or decades.
The aetiology of mycosis fungoides is yet unknown.Genetic predisposition may play a role in some cases. Familial occurrences have been reported in a few instances.Associations with long term exposure to various allergens has also been advocated, as well as exposure to environmental agents and association with chronic skin disorders and viral infections. In some countries, mycosis fungoides like disorders are clearly associated with viral infections(HTLV-1)(2).

References

---

1. Willemze R, Kerl H, Sterry W et al. EORTC classification for primary cutaneous lymphomas: a proposal from the?????? Cutaneous??? Lymphoma Study Group of the European Organization for Research and Treatment of cancer. Blood 1997; 90: 354-371.
2. Pancake B, Zucker-Franklin D, Coutavas E. The cutaneous T-cell lymphoma, mycosis fungoides, is a human T cell lymphotropic virus-associated disease. J Clin Invest 1995; 95:547.
3. Le Boit PE. Variants of mycosis fungoides and related cutaneous T- cell lymphomas. Sem Diag Pathol 1991; 8: 73-81.
4. Matraix J et al. Poikilodermatous mycosis fungoides. Int J Dermatol 2007; 46:950.
5. Dougherty J. Poikiloderma atrophicans vasculare. Arch Dermatol 1971; 103: 550.
6. Mackie RM. Lymphomas and leukemias. In Textbook of Dermatology Champion RH, Burtan JL, Ebling FJEds. 5th edition. Oxford: Blackwell Scientific Publications, 1992; 2107-2134.
7. Howar MS, Smoller BR. Mycosis fungoides: classic disease and variant presentations. Semin Cutan Med Surg 2000; 19: 19.
8. Brecher A. Mycosis fungoides. Dermatol Online J 2003; 9: 23.
9. Samman PD. Natural history of parapsoriasis en plaque and prereticulotic poikiloderma. Br J Dermatol 1972; 84: 405-11.
10. Whittaker SJ, Foss FM. Efficacy and tolerability of currently available therapies for the mycosis fungoides and sezary syndrome variants of cutaneous T-cell lymphomas. Cancer Treat Rev 2007; 33: 146.
11. Vonderheid EC. Treatment planning in cutaneous T-cell lymphoma. Dermatol Ther 2003; 16: 276.
12. Duvic M, Lemak NA, Redman JR, et al. Combined modality therapy for cutaneous T-cell lymphoma. J Am Acad Dermatol 1996; 34: 1022-9.

Source(s) of Funding

---

source of funding: nill

Competing Interests

---

competing interests: nill

Disclaimer

---

This article has been downloaded from WebmedCentral. With our unique author driven post publication peer review, contents posted on this web portal do not undergo any prepublication peer or editorial review. It is completely the responsibility of the authors to ensure not only scientific and ethical standards of the manuscript but also its grammatical accuracy. Authors must ensure that they obtain all the necessary permissions before submitting any information that requires obtaining a consent or approval from a third party. Authors should also ensure not to submit any information which they do not have the copyright of or of which they have transferred the copyrights to a third party.
Contents on WebmedCentral are purely for biomedical researchers and scientists. They are not meant to cater to the needs of an individual patient. The web portal or any content(s) therein is neither designed to support, nor replace, the relationship that exists between a patient/site visitor and his/her physician. Your use of the WebmedCentral site and its contents is entirely at your own risk. We do not take any responsibility for any harm that you may suffer or inflict on a third person by following the contents of this website.