Indian Journal of Dermatology
IJDŽ QUIZ
Year
: 2014  |  Volume : 59  |  Issue : 4  |  Page : 419--420

Persistent eczematous lesions over the body


Ramesh M Bhat1, Hyacinth Peter Pinto1, Reshma G Kini2, Lovely George2,  
1 Department of Dermatology, Father Muller Medical College and Hospital, Kankanady, Mangalore, Karnataka, India
2 Department of Pathology, Father Muller Medical College and Hospital, Kankanady, Mangalore, Karnataka, India

Correspondence Address:




How to cite this article:
Bhat RM, Pinto HP, Kini RG, George L. Persistent eczematous lesions over the body.Indian J Dermatol 2014;59:419-420


How to cite this URL:
Bhat RM, Pinto HP, Kini RG, George L. Persistent eczematous lesions over the body. Indian J Dermatol [serial online] 2014 [cited 2022 Jan 19 ];59:419-420
Available from: https://www.e-ijd.org/text.asp?2014/59/4/419/135514


Full Text

A 55-year-old Indian male presented with a history of asymptomatic brown-black patches over the body of 25-year duration. The lesions appeared first over the right thigh and later, gradually over the next few years progressed to involve the whole body with an increase in the number of lesions in the last 5-years. No history of drug intake prior to onset of these lesions. No history suggestive of any systemic involvement. On examination, multiple, ill-defined, hyperpigmented macules with atrophy and telangiectasia were seen over the whole body with sparing of the face and sun exposed areas such as the dorsum of the hands and feet [Figure 1], [Figure 2] and [Figure 3]. A full blood cell count and biochemical studies revealed normal results. A skin biopsy specimen was taken from a patch over his right thigh which was repeated, a month later from a patch on the right side of the chest. Histopathological examination revealed the presence of atypical lymphocytes in the epidermis along with epidermotrophism, vacuolar interface dermatitis and a bottom-heavy lymphoid infiltrate in the dermis [Figure 4]. Immunohistochemistry markers were positive for CD3 and negative for CD20 markers [Figure 5] and [Figure 6].{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

 Question



What is your diagnosis?

 View Answer

 Answer



Persistent poikilodermatous mycosis fungoides (MF).

 Discussion



MF is the most common variant of primary cutaneous T-cell lymphoma (CTCL), considered a low-grade lymphoproliferative disorder initially confined to the skin, with an indolent course.

The cutaneous involvement includes progression from an early, erythematous, scaly patch stage with eczematous features to an infiltrated plaque stage and finally a tumor stage. [1]

Morphologically clinical variants of MF such as follicular, icthyosiform, bullous, mucinous and pigmented purpura-like and poikilodermatous type have been described. However, the poikilodermatous variant is considered one of the rarer clinicopathologic variants of the same. [1]

It was initially termed poikiloderma atrophicans vasculare and some authors also referred to it as the premycotic form of MF. [2,3] However, poikilodermatous MF is now considered a distinct clinicopathological entity from classic patch/plaque MF. It presents at a younger age and is more frequently associated with lymphomatoid papulosis. [4]

It is characterized clinically by the presence of "cigarette-paper skin" with atrophy, telangiectasia and reticulate or mottled hyper and hypopigmentation. [5] Poikiloderma may be localized or diffuse and often is noted on the breasts, hips, buttocks and flexural areas. It may coexist with patches of classic MF in some patients. Patches may be asymptomatic or mildly pruritic. Persistent poikilodermatous patches on non-sun exposed areas should be considered as MF until proven otherwise by biopsy. [5]

Histopathologic features of poikilodermatous MF include epidermal atrophy, dilated blood vessels in the dermis, melanophages and melanin incontinence. Atypical lymphocytes with hyperchromatic, irregularly contoured nuclei and epidermotropism is a classical feature. These cells are referred to as mycosis cells, sezary cells or lutzner cells. [5] Immunophenotypic studies typically demonstrate a CD2 + , CD3 + , CD4 + , CD7− , CD45RO + , CD8− pattern, which is consistent with a mature helper T-cell phenotype and is similar to that observed in classic MF. Rarely, cases of MF are characterized by a CD8 + cytotoxic T-cell phenotype. [1]

To differentiate early MF from benign dermatoses is an extremely difficult task, yet very critical. To enhance the chances of establishing a conclusive diagnosis, multiple serial biopsies from a variety of lesions are required, including the fully evolved plaque, as well as fresh lesions. The differential diagnosis of poikilodermatous MF includes conditions like parapsoriasis; connective-tissue diseases, such as lupus erythematosus and dermatomyositis; poikiloderma of civatte; overuse of topical glucocorticoids; radiation dermatitis; graft-versus-host disease; and genodermatoses, like Rothmund-Thompson syndrome. [2]

Using the International Society for Cutaneous Lymphomas diagnostic algorithm and staging criteria for CTCL, we diagnosed this patient to have Stage 1A CTCL. Patients with Stage 1A CTCL are treated with skin directed therapies, which include topical corticosteroids, topical chemotherapy (nitrogen mustard or carmustine), topical retinoids, local radiation (X-ray or electron beam), phototherapy (ultraviolet B [UVB] or psoralen ultraviolet A). [3] Narrowband UVB phototherapy is the first-line therapy for poikilodermatous MF. [4]

Poikilodermatous MF has been found to have an excellent prognosis. Retrospective studies revealed, 96% of the study patients with poikilodermatous MF remained at the same stage over a mean follow-up period of more than 11 years. [4] In this case, the lesions have been persistent and stable, i.e., without progression to the plaque and tumor stage for the past 25 years.

MF is considered a dermatological masquerader. It has also been termed "the great imitator." It has been suggested that in every chronic disease resistant to treatment, lymphoma has to be considered, which must be confirmed by clinical, histological and molecular examinations, conducted serially during the course of the disease. [6]

 Learning Points



Mycosis fungoides (MF) is the commonest variant of primary cutaneous T-cell lymphoma, considered a low-grade lymphoproliferative disorder initially confined to the skin, with an indolent courseIt is considered a dermatological masquerader, which mimics many other dermatological conditionsIt must be confirmed by clinical, histological and molecular examinations, conducted serially during the course of the diseasePersistent poikilodermatous patches on non-sun exposed areas should be considered as MF until proven otherwise by biopsyPoikilodermatous MF has been found to have an excellent prognosis.

The names of the winners(first three correct entries) are:

Shraddha Uprety, PGIMER, ChandigarhSoumya Jagadeesan, Amrita Institute of Medical SciencesAkhislesh Shukla, ESI PGIMSR DELHI

"Others who have sent correct answers are mentioned below:"

Vishal Gupta , Shraddha Uprety, Resham Vasani, Anupam Das, Riti Bhatia, Soumya Jagadeesan, Mrinal Gupta, Akhilesh Shukla, Dipti Das, Sumit Gupta, Priyanka C. Patil, Jimish Bagadia, Suneil Pravin Gandhi, Sourabh Jain, Anirban Das, Shouvik Ghosh, Keshavmurthy Adya, Uday Raj, Urmi Khanna, Sweta Rambhia, Himanshu Gupta, Suresh Kumar K, Vishal Chugh, Kumud Agarwal, Deblina Bhunia, Shikha Arora, Pranshu Mishra, G.V.Seethalakshmi, Tarang Goyal, Balachandra S Ankad, Indrashis Podder, Esther Nimisha, Swetha Jain, Vikas.Yatagiri, Poonam Puri, S. Murugan, Kingshuk Chatterjee, Ragunatha S, AKHILESH.A, T S Nagesh, C. Vijay Krishna, Nisha V Parmar, Rashmi Mahajan, Monica Chahar, Deepali Anand Kamble, Kritika Pandey, Geeti Khullar, Mounika ala, HARSH SHARMA, A.Gnaneshwar Rao, Sarita Sanke, Rajesh Rajagopalan, P.Arun Prasath, Shilpa Y K, Anisha Sethi, Navin Modi, Kopal Maheshwari, Brahmita Monga, M.Riswana Jasmine, Vanya Narayan, Naveen Thomas, Mrinal Besra, Praveen Jain, Surekha. A, Rinu Ruth George, Savitha l beergouder, Vinitha Iyer, Kanu verma, Grandhi Usha, Sirisha, Masuma P Bhengra, P.Anila Sunandini, Atul Dongre, Swati Gondse, Sarika Holmukhe , Venkatesh Purohit, Priya Jeevamani C, Vishalakshi P, Himabindu B, Ranju Choudhary, Sukesh M.S, Keerthi Subramaniam, Sarvesh Sunil Thatte, Vijay Zawar, C.Janaki, Sanath S Rao

References

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2Mataix J, Bañuls J, Lucas A, Belinchón I, Betlloch I. Poikilodermatous mycosis fungoides. Int J Dermatol 2007;46:950-1.
3Farley-Loftus R, Mandal R, Latkowski JA. Poikilodermatous mycosis fungoides. Dermatol Online J 2010;16:8.
4Abbott RA, Sahni D, Robson A, Agar N, Whittaker S, Scarisbrick JJ. Poikilodermatous mycosis fungoides: A study of its clinicopathological, immunophenotypic, and prognostic features. J Am Acad Dermatol 2011;65:313-9.
5Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al. Defining early mycosis fungoides. J Am Acad Dermatol 2005;53:1053-63.
6Nashan D, Faulhaber D, Ständer S, Luger TA, Stadler R. Mycosis fungoides: A dermatological masquerader. Br J Dermatol 2007;156:1-10 .