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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 18  |  Issue : 4  |  Page : 321-323

Subcutaneous panniculitis-like lymphoma in a 3-year-old child


Department of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana, India

Date of Web Publication29-Sep-2017

Correspondence Address:
Sudharani Chintagunta
Department of DVL, Gandhi Hospital, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.193028

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  Abstract 


Panniculitis-like T-cell lymphoma is a rare cytotoxic T-cell lymphoma of the skin involving the subcutaneous tissue. We report panniculitis like lymphoma occurring in a 3 year old female child.She presented with multiple erythematous tender nodules and plaques on face,right thigh and genital region associated with systemic symptoms.The histopathology revealed lobular panniculitis composed of atypical lymphocytes . Immunophenotypic studies showed that the atypical lymphocytes are positive for CD3&CD8 and negative for CD20. The child improved with systemic antibiotics and supportive therapy. Now child is on regular follow up for the past four months without any new lesions.

Keywords: Child, panniculitis-like lymphoma, spontaneous regression


How to cite this article:
Chintagunta S, Arakkal G, Mohammed S, Gopidi P. Subcutaneous panniculitis-like lymphoma in a 3-year-old child. Indian J Paediatr Dermatol 2017;18:321-3

How to cite this URL:
Chintagunta S, Arakkal G, Mohammed S, Gopidi P. Subcutaneous panniculitis-like lymphoma in a 3-year-old child. Indian J Paediatr Dermatol [serial online] 2017 [cited 2019 Jun 17];18:321-3. Available from: http://www.ijpd.in/text.asp?2017/18/4/321/193028




  Introduction Top


Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is defined as cytotoxic T-cell lymphoma (CTCL) characterized by subcutaneous infiltrate of pleomorphic T-cells. SPTCL category includes cases with alpha/beta T-cell phenotype (75%) and cases with gamma-delta T-cell phenotype (25%) according to the previous classifications. At present, the WHO-European Organization for Research and Treatment of Cancer (EORTC) classifies only α/β phenotype as SPTCL [1] and T-cell receptor γδ is placed in a new category of primary cutaneous peripheral T-cell lymphomas – cutaneous γ T-cell lymphoma.[2] SPTCL is common in the age group of 20–70 years with female predominance and rare in children.[3] We herewith report a case of SPTCL in a 3-year-old child where the lesions resolved with supportive therapy.


  Case Report Top


A female child aged 3 years brought with complaints of painful swellings on the abdomen and right thigh of 2 months duration. History of high-grade fever and facial swelling and edema both feet was present for 15 days. Cutaneous examination revealed multiple erythematous tender indurated plaques and nodules on the right thigh, abdomen, and both cheeks. Ulceration and crusting were present on the thigh and vulval region [Figure 1] and [Figure 2]. Oral mucosa, hair, and nails were normal. There was no lymphadenopathy. Systemic and general examination were unremarkable. A differential diagnosis of Sweet's syndrome and panniculitis was considered. All the routine investigations such as complete urine analysis, complete blood picture, liver function test (LFT), renal function test, antistreptolysin O titer, C-reactive protein, antinuclear antibodies, double-stranded DNA, X-ray chest, and U/S of the abdomen were normal. Peripheral smear and bone marrow aspiration were normal. Skin biopsy showed normal epidermis. Dermis showed mild perivascular lymphocytic infiltrate and subcutaneous tissue infiltration by lymphocytes predominantly outlining the adipocytes. Occasional lymphocytes appeared atypical with hyperchromasia. There was no evidence of granuloma or vasculitis [Figure 3]. On immunohistochemical studies, the atypical lymphocytes were positive for CD3 and CD 8 (CD3+, CD8+, CD20−) [Figure 4]. Ki-67 index was high by 50%. Based on histopathological and immunohistochemical findings, a diagnosis of SPTCL was made. Skin lesions resolved in 4 weeks with intravenous amoxicillin and clavulanic acid and supportive therapy with hyperpigmentation. The child was under regular follow-up for the past 4 months without any new lesion.
Figure 1: Periorbital edema with erythema and crusting on cheeks

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Figure 2: Ulceration and crusting involving vulva

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Figure 3: Lobular inflammatory infiltrates and around adipocytes - predominantly lymphocytes and occasional hyperchromasia

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Figure 4: Immunohistochemical stains – CD3+ and CD8+

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  Discussion Top


CTCL is a group of lymphoproliferative disorders characterized by localization of neoplastic T-lymphocytes to the skin. The WHO-EORTC classification of CTCLs is divided into those with indolent clinical behavior, those with aggressive subtypes and natural killer-cell lymphoma. CTCLs with indolent clinical behavior include mycosis fungoides and its variants, primary cutaneous CD30+ lymphoproliferative disorder, primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma (provisional), and SPTCL.[4]

SPTCL classified as α/β T-cell phenotype (SPTCL-AB) and SPTCL with γδ T-cell phenotype (SPTCL-GD). Both entities are characterized by atypical lymphocytes that involve the subcutis in a lobular configuration with rimming of individual adipocytes. Alpha/beta phenotype is usually CD8+, infiltrates confined to the subcutaneous tissue only and with an indolent clinical course.[5] A similar-appearing lymphoma with γ/δ phenotype is CD4−, CD8−, and CD56+; the infiltration may involve the epidermis and have a poor prognosis.[5] In contrast to the α/β cases, γδ T-cell lymphoma is associated with a more aggressive clinical course and increased risk of developing hemophagocytic syndrome (HPS). HPS is seen in 15–20% of SPTCL and carries poor prognosis.[3]

Subcutaneous panniculitis-like lymphoma (SPTCL-AB) represents <1% of all non-Hodgkin lymphomas. It is common in the age group ranging 22–73 years but rare in children.[5],[6],[7] It is characterized by recurrent erythematous subcutaneous nodules and plaques involving the extremities, trunk, and rarely the face. Ulceration is uncommon.[1],[4] These lesions may resemble benign panniculitis and is often accompanied by systemic symptoms such as fever, fatigue, weight loss, hepatitis, and lymphadenopathy.[2] Autoimmune diseases are common among SPTCL patients, particularly systemic lupus erythematosus. The laboratory abnormalities include various cytopenias and elevated LFTs. Histologically, SPTCL is characterized by lobular panniculitis with a subcutaneous atypical lymphocyte proliferation rimming the adipocytes. Early lesions show focal atypical lobular lymphocytic infiltration of the subcutis without epidermal or dermal involvement that may confuse with benign panniculitis. Other inflammatory cells, particularly plasma cells are absent which differentiate it from lupus panniculitis, later rimming of individual fat cells by the neoplastic cells, mitoses, and karyorrhexis. The immune phenotype of atypical lymphocytes is CD3+, CD8+, CD4−, CD30−, and CD56−.[3] SPTCL may be accompanied by HPS characterized by fever, cytopenia, hepatosplenomegaly, and hemophagocytosis (phagocytosis of erythrocytes, leukocytes, platelets, and their precursors by macrophages) in the bone marrow. SPTCL-AB without associated HPS has good prognosis.[3] The 5-year overall survival rate in cases of SPTCL-AB is generally around 82%.[3]

Treatment includes systemic agents such as steroids, methotrexate, cyclosporine, bexarotene, and interferon for indolent disease with a generalized distribution whereas aggressive presentations may require combination chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP regimen) and stem cell transplantation.[2],[4],[8] Indolent localized lesions may respond to local radiotherapy.

SPTCL is a rare entity in children and very few cases have been reported below 3 years of age [9],[10],[11] Ulceration is uncommon in α-β type. Literature survey showed very few case reports of spontaneous resolution [6],[8] in SPTCL which was also observed in our case. Although this child presented with multiple cutaneous lesions along with systemic symptoms, the child showed remission with intravenous antibiotics and supportive therapy. In the early stages, panniculitis-like lymphoma clinically and pathologically may mimic benign panniculitis; hence, early diagnosis, management, and long-term follow-up are necessary for better outcome.

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.



 
  References Top

1.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008. p. 294-5.  Back to cited text no. 1
    
2.
Koh MJ, Sadarangani SP, Chan YC, Chan MY, Tan AM, Tan SH, et al. Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma). J Am Acad Dermatol 2009;61:875-81.  Back to cited text no. 2
[PUBMED]    
3.
Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: Definition, classification, and prognostic factors: An EORTC Cutaneous Lymphoma Group study of 83 cases. Blood 2008;111:838-45.  Back to cited text no. 3
[PUBMED]    
4.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 4
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5.
Huppmann AR, Xi L, Raffeld M, Pittaluga S, Jaffe ES. Subcutaneous panniculitis-like T-cell lymphoma in the pediatric age group: A lymphoma of low malignant potential. Pediatr Blood Cancer 2013;60:1165-70.  Back to cited text no. 5
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6.
Bittencourt AL, Vieira MD, Carvalho EG, Cunha C, Araujo I. Subcutaneous panniculitis-like t-cell lymphoma (SPTL) in a child with spontaneous resolution. Case Rep Oncol Med 2011;2011:639240.  Back to cited text no. 6
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7.
Francis A, Criton S, Acharya S, Shojan A, Philip RM. Subcutaneous panniculitis-like T-cell lymphoma. Pediatr Blood Cancer 2013;60:1165-70.  Back to cited text no. 7
    
8.
Go RS, Wester SM. Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: A systematic analysis of 156 patients reported in the literature. Cancer 2004;101:1404-13.  Back to cited text no. 8
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9.
Acree SC, Tovar JP, Pattengale PK, Wang LL, Church JA, Gaynon PS, et al. Subcutaneous panniculitis-like T-cell lymphoma in two pediatric patients: An HIV-positive adolescent and a 4-month-old infant. Fetal Pediatr Pathol 2013;32:175-83.  Back to cited text no. 9
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10.
Yim JH, Kim MY, Kim HO, Cho B, Chung NG, Park YM. Subcutaneous panniculitis-like T-cell lymphoma in a 26-month-old child with a review of the literature. Pediatr Dermatol 2006;23:537-40.  Back to cited text no. 10
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11.
Matteo C, Samuele N, Daniele R, Anna L, Alessandro V. Subcutaneous panniculitis-like T-cell lymphoma presenting with diffuse cutaneous edema in a 2-year-old. Pediatr Blood Cancer 2013;60:1165.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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