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  Table of Contents  
LETTER TO THE EDITOR
Year : 2015  |  Volume : 52  |  Issue : 1  |  Page : 79-80
 

Hyperpigmented mycosis fungoides with ecthyma gangrenosum


1 Department of Dermatology, SDM College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India
2 Department of Pathology, SDM College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India

Date of Web Publication3-Feb-2016

Correspondence Address:
V V Pai
Department of Dermatology, SDM College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.175608

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How to cite this article:
Pai V V, Kikkeri N N, Athanikar S C, Rai V, Dinesh U S. Hyperpigmented mycosis fungoides with ecthyma gangrenosum. Indian J Cancer 2015;52:79-80

How to cite this URL:
Pai V V, Kikkeri N N, Athanikar S C, Rai V, Dinesh U S. Hyperpigmented mycosis fungoides with ecthyma gangrenosum. Indian J Cancer [serial online] 2015 [cited 2019 Aug 22];52:79-80. Available from: http://www.indianjcancer.com/text.asp?2015/52/1/79/175608


Sir,

A 45-year-old female presented with a history of pigmentation all over the body since 6 years and ulcerative lesions over the abdomen since 1 month. Pigmentation started over the leg and gradually progressed to involve the abdomen, upper limb and face. Ulcers were initially coin shaped have gradually increased in size.

Cutaneous examination revealed hyperpigmented patches to plaques over the chest, abdomen, back and extremities. Two adjoining ulcers, measuring 6 cm in diameter were present over the abdomen. Margins of the ulcer were well-defined with sloping edges [Figure 1]. Other than a hemoglobin % - 9 g/dL, rest of the hematological and biochemical parameters were within normal limits. Culture from the ulcer showed growth of Pseudomonasaureginosa sensitive to clindamycin, teicoplanin, netilmicin.
Figure 1: Hyperpigmented plaques over the abdomen with two adjoining well-defined punched out ulcer

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Histopathological examination revealed infiltration in the basal layer by large lymphoid cells arranged in nests forming Pautrier's microabscess having hyper chromatic convoluted nucleus [Figure 2]. Melanin incontinence was seen in the dermis [Figure 3]. These features were suggestive of hyperpigmented variant of mycosis fungoides (MF). Patient was given antibiotics, topical steroids, psoralen and ultraviolet A therapy (PUVA) therapy for 3 weeks with debridement of the ulcer. Significant improvement in the ulcer was noted.
Figure 2: Hyperkeratosis with infiltration in the basal layer by large lymphoid cells having hyper chromatic convoluted nucleus was present. The cells were arranged in nests forming Pautrier's microabscess in the basal layer (H and E, ×40)

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Figure 3: Melanin incontinence seen in the dermis (H and E, ×100)

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MF is the most common cutaneous T-cell lymphoma (CTCL) that in addition to its classic presentation of patches, plaques and tumors, may show atypical features defining many clinical variants of the disease. It is seen in population working in petrochemical, glass and metal industries. It is also seen in patients with severe atopic dermatitis.[1] MF develops due to antigen persistence associated with chronic lymphocyte stimulation and eventual transformation of benign lymphocytes to a low-grade malignant T-cell lymphoma.[2]

Histopathologically, in the patch, plaque and also in the erythrodermic stage, there is an epidermotropic band-like infiltrate of T lymphocytes with hyperconvoluted cerebriform nuclei involving the upper dermis and formation of intraepidermal Pautrier's microabscesses.[1] In hyperpigmented variants in addition to the above features melanophages and melanin incontinence is seen.[3]

Hyperpigmented MF is an atypical clinical variant of CTCL, with a predilection for patients with a dark complexion. It is a relatively rare variety characterized by a predominantly CD8+ phenotype.[3] Hyperpigmentation has been seen in pigmented purpura-like eruptions, poikiloderma atrophicans vasculare and during PUVA or cytostatic agents.[4] Hyperpigmented lesions may be the sole manifestation or it may coexist with other unusual MF variants typified mostly by pigmentary changes.[3]

Infections in MF are mainly cutaneous bacterial infections followed by herpetic infection, bacteremia, bacterial pneumonia and urinary tract infections in a descending order. Though cutaneous bacterial infections are common bacterial infection in MF, ecthyma gangrenosum has never been reported in MF.[4] Ecthyma gangrenosum is Pseudomonas infection, which usually occurs in debilitated, leukemic or severely burned patients. They consist of multiple punched-out ulcers that have hemorrhagic borders.[1]

Ulcerations in MF is often associated with secondary bacterial infection and such patients may have a very poor quality-of-life and high morbidity despite having an early stage of disease.[1] Cutaneous ulceration in MF can also be due to methotrexate therapy. The mechanism of cutaneous necrosis and ulceration due to methotrexate therapy may be due to the antiproliferative effects of the drug.[5]

This case has been reported for its clinical rarity.

 
  References Top

1.
Whittaker SJ. Cutaneous lymphomas and lymphocytic infiltrates. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th ed. Oxford: Blackwell Science Ltd.; 2010. p. 51.1-51.13.  Back to cited text no. 1
    
2.
Tan RS, Butterworth CM, McLaughlin H, Malka S, Samman PD. Mycosis fungoides: A disease of antigen persistence. Br J Dermatol 1974;91:607-16.  Back to cited text no. 2
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3.
Pavlovsky L, Mimouni D, Amitay-Laish I, Feinmesser M, David M, Hodak E. Hyperpigmented mycosis fungoides: An unusual variant of cutaneous T-cell lymphoma with a frequent CD8+phenotype. J Am Acad Dermatol 2012;67:69-75.  Back to cited text no. 3
    
4.
Axelrod PI, Lorber B, Vonderheid EC. Infections complicating mycosis fungoides and Sézary syndrome. JAMA 1992;267:1354-8.  Back to cited text no. 4
    
5.
Sheehan-Dare RA, Goodfield MJ, Williamson DM, Cotterill JA. Ulceration of the palms and soles. An unusual feature of cutaneous T-cell lymphoma. Acta Derm Venereol 1990;70:523-5.  Back to cited text no. 5
    


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