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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 441-444
CD4/CD8 dual positive pityriasis lichenoides like mycosis fungoides presenting with both pityriasis lichenoid lesions and hypopigmented patches: A rare presentation


From the Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India

Date of Web Publication2-Nov-2022

Correspondence Address:
Angoori Gnaneshwar Rao
From the Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_279_22

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How to cite this article:
Rao AG, Naresh M, Shreeja V, VSVN S, Sindhuja B, Pranaya B. CD4/CD8 dual positive pityriasis lichenoides like mycosis fungoides presenting with both pityriasis lichenoid lesions and hypopigmented patches: A rare presentation. Indian J Dermatol 2022;67:441-4

How to cite this URL:
Rao AG, Naresh M, Shreeja V, VSVN S, Sindhuja B, Pranaya B. CD4/CD8 dual positive pityriasis lichenoides like mycosis fungoides presenting with both pityriasis lichenoid lesions and hypopigmented patches: A rare presentation. Indian J Dermatol [serial online] 2022 [cited 2022 Dec 4];67:441-4. Available from: https://www.e-ijd.org/text.asp?2022/67/4/441/360323




Sir,

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma with an incidence of 5.6 persons per million.[1] Certain chromosomal abnormalities have been described in MF, including the chromosomal loss at 10Q and abnormalities in P15, P16, and TP53 tumor suppressor genes. The natural history of MF is characterized by an indolent progression through four stages: patch, plaque, tumor, and erythroderma.

A 31-year-old male was presented with recurrent crops of erythematous papules and hypopigmented patches on the trunk and upper extremities for one year. He gives the history of erythematous papules, which later scale off, leaving behind hypopigmentation. Examination revealed multiple erythematous papules and atrophic spots distributed on the trunk and upper limbs. Also, there were well to ill-defined hypopigmented macules and patches distributed prominently on the front and back of the trunk and upper extremities. [Figure 1], [Figure 2], [Figure 3] Auspitz's sign was negative. There was no telangiectasia. Lymph nodes were not enlarged. Systemic examination revealed no organomegaly. A provisional diagnosis of pityriasis lichenoides chronica was offered. However, guttate psoriasis and extensive tinea versicolor were considered in the differential diagnosis. Haematological and biochemical investigations revealed normal study. Chest skiagram and ultrasonography of abdomen revealed the normal study. Serology for human immunodeficiency virus (HIV) 1 and 2 was non-reactive. Scraping on potassium hydroxide mount was negative for fungal elements. Bone marrow biopsy was normocellular. Blood smear for atypical lymphocytes was negative. Biopsy taken from papule with surrounding hypopigmented patch revealed focal parakeratosis with almost regular acanthosis, moderate lymphocytic exocytosis with focal mild spongiosis, and moderate perivascular and interstitial lymphocytic infiltrate [Figure 4]. Atypical lymphoid cells with nuclear hyperchromasia and irregularity were seen [Figure 5]. Immunohistochemical study revealed CD3 [Figure 6], CD4 [Figure 7], and CD8 [Figure 8] positivity in epidermal lymphocytes and perivascular lymphocytic infiltration. CD 20 and CD30 [Figure 9] were negative. The diagnosis of CD4/CD8 Dual positive MF was confirmed and was placed in stage IB in accordance with the staging system developed by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization for Research and Treatment of Cancer (EORTC). He was placed on narrow band ultraviolet B (NB-UVB) therapy and is under follow-up.
Figure 1: Multiple erythematous papule, atrophic spots, and hypopopigmented patches on the trunk

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Figure 2: Hypopigmented patches and few erythematous papules on right upper limb and right side of trunk

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Figure 3: Hypopigmented patches and few erythematous papules on left upper limb and left side of trunk

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Figure 4: Histopathological examination of papule; focal parakeratosis with regular acanthosis, moderate lymphocytic exocytosis with focal mild spongiosis, and moderate perivascular and interstitial lymphocytic infiltrate in the dermis. (Hematoxylin and eosin, ×10)

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Figure 5: Histopathological examination of papule; atypical lymphoid cells with nuclear hyperchromasia and irregularity. (Hematoxylin and eosin, ×40)

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Figure 6: CD4 positive cells (Immunohistochemistry × 40)

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Figure 7: CD8 positive cells (Immunohistochemistry × 40)

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Figure 8: CD3 positive cells (Immunohistochemistry × 40)

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Figure 9: CD30 negative (Immunohistochemistry × 40)

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MF is usually characterized by a T-helper memory phenotype (CD3+, CD4 + CD8-). CD4/CD8 double-positive phenotype is extremely rare in MF, and only a couple of cases have been reported in the literature.[2] These patients show a slightly lower rate of progressive disease compared to patients with conventional CD4+/CD8 − phenotype.

Pityriasis lichenoides (PL) has been conventionally classified as a benign papulosquamous disorder. Notably, only a small number of patients (5.3%) with PL have been reported to develop MF after 3–11 years of prolonged clinical course. Repeated biopsies are required in non-remitting and prolonged courses and when patches and large plaques develop in PL cases. Histopathologically, increased lymphocytic nuclear atypia decrease in apoptotic keratinocytes, and CD7+ and CD8+ lymphocytes and monoclonal T-cell receptor gene rearrangements (TCR-GR) may serve as a clue to the development of MF.

It is interesting to note that the index case presented with PL like lesions and hypopigmented patches. Ko et al.[3] first described PL like MF, which is a rare type of early MF that develops in young adults and has a favourable prognosis. Its clinical presentation is similar to PL. Notably, the index case of PL like MF is dual CD4+/CD8+. Jang et al.[4] in their study of 316 patients of MF documented 15 cases (4.74%) of MF presenting with PL-like lesions. The presentation of the index case is unique as the dual positive CD4/CD8 MF exhibited both PL-like lesions and hypopigmented patches. PL like MF and hypopigmented MF have been reported to occur independently. Contrarily, various presentations, including sclerotic plaque, erythematosquamous patches, extensive ichthyosis, bluish-red eczematized tumor, and erythroderma, have been reported in the literature [Table 1]. Notably, PL like MF was found to have a better prognosis than other histopathological variants, including bullous, granulomatous, and follicular types. However, no clear conclusion can be drawn regarding the morphological presentation and behaviour of this rare phenotype because only a few cases have been reported in the literature.
Table 1: (Original) Published case reports of Dual positive CD4/CD8 Mycosis fungoides

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Most patients of hypopigmented MF are dark-skinned individuals from Asia. Similarly, the index case of hypopigmented dual positive CD4/CD8 MF is dark-skinned Asian. Most patients of hypopigmented MF are younger compared to classical MF. The prognosis in hypopigmented MF patients is better compared to classical MF. Similarly, our patient with co-expression of CD4/CD8 in stage IB seems to have a lower tendency of disease progression, which may be attributable to increased activity of anti-tumor proliferating CD8 + lymphocytes infiltrating the lesions.[5]

The cause for hypopigmentation in hypopigmented MF was elucidated by Nasr et al.,[6] who demonstrated an increase in tumor necrosis factor-alpha (TNF alpha) levels and a decrease in basic fibroblast growth factor in MF lesions that cause altered melanogenesis and melanocyte apoptosis resulting in hypopigmentation.

Treatment of MF depends on the stage of the disease, systemic conditions, age, and disease course. Various treatment modalities used in the treatment of MF include potent topical corticosteroids, bexarotene, topical nitrogen mustard, psoralen + ultraviolet A, NB-UVB, acitretin, electron beam therapy, extracorporeal photochemotherapy, denileukindiftitox, doxorubicin, gemcitabine, allogenic bone marrow transplantation, zanolimumab, and alemtuzumab. Because the patient is in stage IB, he was managed with NB-UVB and is under follow-up. Notably, NB-UVB demonstrated complete improvement in a study of 10 patients of PL like MF and suggested that phototherapy is adequate in PL like MF. It acts by inhibiting proliferating neoplastic T cells and penetrates into the upper dermis.[7]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.[11]



 
   References Top

1.
Korgavkar K, Xiong M, Weinstock M. Changing incidence trends of cutaneous T-cell lymphoma. JAMA Dermatol 2013;149:1295-9.  Back to cited text no. 1
    
2.
Knapp CF, Mathew R, Messina JL, Lien MH. CD4/CD8 dual-positive mycosis fungoides: A previously unrecognized variant. Am J Dermatopathol 2012;34:e37.  Back to cited text no. 2
    
3.
Ko JW, Seong JY, Suh KS, Kim ST. Pityriasis lichenoides-like mycosis fungoides in children. Br J Dermatol 2000;142:347-52.  Back to cited text no. 3
    
4.
Jang MS, Kang DY, Park JB, Kim JH, Park KA, Rim H, et al. Pityriasis lichenoides-like mycosis fungoides: Clinical and histologic features and response to phototherapy. Ann Dermatol 2016;28:540-7.  Back to cited text no. 4
    
5.
Hoppe RT, Medeiros LJ, Warnke RA, Wood GS. CD8-positive tumor-infiltrating lymphocytes influence the long-term survival of patients with mycosis fungoides. J Am Acad Dermatol 1995;32:448-53.  Back to cited text no. 5
    
6.
Nasr SEH, Shaker OG, Fawzi MM, El-Hanafi G. Basic fibroblast growth factor and tumour necrosis factor alpha in vitiligo and other hypopigmented disorders: Suggestive possible therapeutic targets. J Eur Acad Dermatol Venereol 2013;27:103-8.  Back to cited text no. 6
    
7.
Krutmann J, Morita A. Mechanisms of ultraviolet (UV) B and UVA phototherapy. J Investig Dermatol Symp Proc 1999;4:70-72.  Back to cited text no. 7
    
8.
Tournier E, Laurent C, Thomas M, Meyer N, Viraben R, Brousset P, et al. Double –positive CD4/CD8 Mycosis fungoides; a rarely reported immunohistochemical profile. J Cutan Pathol 2014;41:58-62.  Back to cited text no. 8
    
9.
Kaku Y, Ostuka A, Tanizaki H, Shibuya R, Endo Y, Nonomura Y, et al. Dual CD4/CD8 positive Icthyosiform Mycosis Fungoides with lymphnode, peripheral blood and cardiac involvement: a case report. Acta Derm Venereol 2016;96:564-6.  Back to cited text no. 9
    
10.
Raychaudhuri S, Rohvar M, Jedrych J, Karunamurthy A, Kruglov O, Rakfal S, et al. CD4/CD8 Primary cutaneous Peripheral T-cell lymphoma, previously classified as Mycosis Fungoides a tumor D'emblee. Am J Dermatopathol 2018;40:836-40.  Back to cited text no. 10
    
11.
Ding X, Chen J, Kuai L, Xing M, Ru Y, Luo Y, et al. CD4/CD8 Dual positive Mycosis Fungoides: a case report and literature review. Medicine 2020;99:e22786.  Back to cited text no. 11
    


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