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Table of Contents 
CASE REPORT
Year : 2022  |  Volume : 67  |  Issue : 3  |  Page : 283-286
Clinico-dermoscopic-pathological features of a rare case of locally invasive multifocal primary cutaneous diffuse large b cell lymphoma-leg type over the face and scalp


1 Department of Dermatology, and Venereology, AIIMS, Bhubaneswar, Odisha, India
2 Department of Medical Oncology and Hematology, AIIMS, Bhubaneswar, Odisha, India
3 Department of Pathology, AIIMS, Bhubaneswar, Odisha, India

Date of Web Publication22-Sep-2022

Correspondence Address:
Biswanath Behera
Department of Dermatology, and Venereology, AIIMS, Bhubaneswar - 751019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_783_20

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   Abstract 


Primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) is characterized by diffuse monotonous proliferation of centroblasts and immunoblasts. It commonly presents as erythematous to violaceous nodules on one or both the legs and has a poor prognosis. We report the clinico-dermoscopic-pathological features and therapeutic response of a rare case of PCDLBCL-LT in a 62-year-old diabetic man, who presented with multifocal plaques, one lobulated and two arcuate-shaped, on the face and scalp. During the investigation, one of the plaques had eroded the underlying bone without any evidence of malignant cells in the cerebrospinal fluid. He was successfully treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) along with intrathecal methotrexate.


Keywords: Dermoscopy, diffuse, large B-cell, lymphoma, skin neoplasms


How to cite this article:
Behera B, Palit A, Nayak AK, Panigrahi A, Mishra P, Sethy M. Clinico-dermoscopic-pathological features of a rare case of locally invasive multifocal primary cutaneous diffuse large b cell lymphoma-leg type over the face and scalp. Indian J Dermatol 2022;67:283-6

How to cite this URL:
Behera B, Palit A, Nayak AK, Panigrahi A, Mishra P, Sethy M. Clinico-dermoscopic-pathological features of a rare case of locally invasive multifocal primary cutaneous diffuse large b cell lymphoma-leg type over the face and scalp. Indian J Dermatol [serial online] 2022 [cited 2022 Sep 30];67:283-6. Available from: https://www.e-ijd.org/text.asp?2022/67/3/283/356758





   Introduction Top


Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), a rare subtype of primary cutaneous B-cell lymphoma, is defined by the diffuse proliferation of large B-cells. PCDLBCL-leg type (LT) is characterized by the presence of solitary or multifocal erythematous to violaceous papule or nodules on one or both legs that can rapidly progress to a distant site. Localization other than over the leg(s) is rare.[1] Hereby, we report the clinico-dermoscopic-pathological features of a rare case of multifocal PCDLBCL-LT over the face and scalp with the erosion of the underlying bone by one of the plaques.


   Case Report Top


A 62-year-old diabetic man with skin phototype IV presented with seven months history of gradually enlarging asymptomatic swelling over the right temple. It was not associated with any systemic features. Cutaneous examination showed three swellings; erythematous lobulated plaque (5 cm × 2 cm) with telangiectasia and a satellite papule over the right temporal area, two skin-colored to erythematous arcuate plaques over the occipital scalp [Figure 1]. Differential diagnoses of B-cell lymphoma, pseudolymphoma, and angiosarcoma were considered. Dermoscopy (Dermlite, DL4, 10× magnification) under polarized mode of the temporal area plaque revealed salmon-colored homogenous area, white shiny structureless area, rosette, brown peppering, and arborizing vessels [Figure 2]a. The plaques over the occipital area showed salmon-colored homogenous area, serpentine vessels, and pigment network [Figure 2]b. Laboratory investigation showed microcytic hypochromic anemia (hemoglobin-10.9 gm/dL) and mildly elevated renal parameters (urea-53 mg/dL, creatinine-1.4 mg/dL). Computed tomography of the scalp revealed erosion of scalp bone up to the dura, underlying the lesion over the temporal area, but cerebrospinal fluid examination did not show any malignant cells. Other investigations were within normal limits. Histopathology showed a narrow Grenz zone and dense diffuse infiltration of dermis and subcutis by predominant large lymphoid cells with scant cytoplasm and large vesicular nucleus along with few reactive lymphocytes. The cells were immunopositive for CD20, Bcl-2, and Bcl-6 and negative for CD10, CD5, CD30, CD138, CD21, CyclinD1, ALK1, and MUM1. The proliferation index was more than 75% (MIB1), and few cells stained positive for CD3 [Figure 3] and [Figure 4]. The diagnosis of PCDLBCL-LT was made. The patient received six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) along with intrathecal methotrexate, following which there was complete resolution of all the lesions [Figure 5]. During the therapy, he developed the following side effects: Nausea, vomiting, diffuse alopecia, bluish discoloration of the nail plates, and transient pancytopenia, for which he was given intravenous GM-CSF (Granulocyte-Macrophage Colony Stimulating Factor). One year post-therapy, the patient is doing well without any recurrence or complication of the disease or therapy.
Figure 1: (a) Erythematous lobulated plaque with telangiectasia and a satellite papule. (b) Two skin-coloured to erythematous arcuate plaques

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Figure 2: (a) Dermoscopy (Dermlite, DL4, 10× magnification) of the temporal area plaque under the polarized mode shows salmon-coloured homogenous area, white shiny structureless area, rosette (blue arrow), brown peppering (red arrow), and arborizing vessels. (b) The plaques over the occipital area shows salmon-coloured homogenous area, serpentine vessels, and pigment network (arrow)

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Figure 3: (a) Nonepidermotrophic diffuse infiltration of the dermis by predominant large lymphoid cells (H & E, ×50). (b) The infiltration comprises centroblasts and immunoblasts with few reactive lymphocytes (H & E, ×400). (c) The lymphoid cells are immunopositive for CD20 (IHC, ×400). (d) The lymphoid cells are immunopositive for Bcl-2 (IHC, ×400). (e) The lymphoid cells are immunopositive for Bcl-6 (IHC, ×400). (f) MIB1 positivity is more than 75% (IHC, ×400). (g) The lymphoid cells are immunonegative for MUM1 (IHC, ×400). (h) The lymphoid cells are immunonegative for CD10 (IHC, ×400). (i) The lymphoid cells are occasional immunopositive for CD3 (IHC, ×400)

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Figure 4: (a) The lymphoid cells are immunonegative for CyclinD1 (IHC, ×100). (b) The lymphoid cells are immunonegative for CD5 (IHC, ×100). (c) The lymphoid cells are immunonegative for CD30 (IHC, ×100). (d) The lymphoid cells are immunonegative for CD138 (IHC, ×100). (e) The lymphoid cells are immunonegative for CD21 (IHC, ×100). (f) The lymphoid cells are immunonegative for ALK1 (IHC, ×100)

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Figure 5: Complete resolution of the plaque following therapy

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   Discussion Top


In the 2005 classification, the World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) divided PCDLBCL into leg-type and other-type. The latter included rare cases of DLBCL first presenting in the skin that could not be classified as either PCDLBCL-LT or PCFCL (primary cutaneous follicle center lymphoma). Since then, there has been much confusion regarding the term PCDLBCL-other type, as it included the large transformed cells that stain negative for Bcl-2 and cases of PCDLBCL that could not be classified properly using the Hans algorithm. Besides, there was no significant difference between cases of PCDLBCL-LT with or rare cases without Bcl-2 expression. The recent classification has scraped the others-subtype. The cases that cannot be classified as PCDLBCL-LT are termed as PCDLBCL-not otherwise specified (PCDLBCL-NOS).[1],[2]

PCDLBCL-leg type is characterized by the presence of solitary or multifocal asymptomatic rapidly enlarging large red or purple dermal nodules, commonly either on one or both legs. The nodule can develop ulcers over time. Location other than on the leg is a rare occurrence. The index case had lesions of atypical morphology and over uncommon locations; lobulated and arcuate plaques over the face and scalp.

The characteristic features of PCDLBCL-NOS are poorly defined. Recent studies found it to be common in younger individuals and present as a slowly evolving plaque of size more than 5 cm at the time of primary diagnosis, due to the delay in consultation. Besides, NOS-subtype often has multiple lesions located in one or two contiguous body regions. There was a statistically significant difference between the LT and NOS for their location on the lower leg, but there was no difference in the prevalence of ulceration between them. However, the Authors opined that a definitive diagnosis could not be made based upon the location of the tumor.[1],[3],[4]

The histopathology of PCDLBCL is characterized by the nonepidermotropic diffuse infiltration of the dermis with large cells consisting of centroblasts and immunoblasts that may extend into the subcutis. Its monotonous nature and prominent mitosis mark the infiltration with few inflammatory or reactive T-cells. Although, by definition, NOS is Bcl-2 negative, a complete absence of Bcl-2 immunostaining was rarely seen, and it varies from 0-50%, while in LT, it is more than 70%.[1]

Both variants of DCLBCL are aggressive in nature, with a 5-year survival rate of 20-60%. The expression of Bcl-2 is considered as the most important prognostic factor, which may be responsible for the relatively less aggressive behavior of NOS.[1] Ruling out PCFCL (primary cutaneous follicle center lymphoma), especially one with the diffuse growth pattern, is essential as it has a different therapeutic approach and a very favorable prognosis (90% 5-year survival rate).[5],[6] PCFCL is typified by the presence of predominant centrocytes and negative immunostaining for Bcl-2, MUM1, and cytoplasmic IgM.[1]

Dermoscopy, as a supplementary diagnostic tool, has been used for various primary cutaneous B cell lymphomas. Two cases of PCDLBCL -LT showed salmon-colored background/area, white circles/areas, scales, arborizing vessels, and polymorphous vascular pattern. We observed a similar dermoscopic pattern. Besides, PCMZL (primary cutaneous marginal zone lymphoma) and PCFCL reported having similar features (except for polymorphic vascular pattern). So, dermoscopy can be useful in suspecting the diagnosis of lymphoma by demonstrating a salmon/orange color that correlates the dense dermal lymphocytic infiltration.[7],[8] However, it cannot differentiate a lymphoma from pseudolymphoma.

In conclusion, we report a rare morphological presentation of a case of locally invasive multifocal PCDLBCL-LT over the face and scalp that was successfully treated with R-CHOP and intrathecal methotrexate therapy. We are also describing dermoscopic features like salmon-colored homogenous area, white shiny structures, and arborizing vessels, which can be useful in pointing the diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Felcht M, Klemke CD, Nicolay JP, Weiss C, Assaf C, Wobser M, et al. Primary cutaneous diffuse large B-cell lymphoma, NOS and leg type: Clinical, morphologic and prognostic differences. J Dtsch Dermatol Ges 2019;17:275-85.  Back to cited text no. 1
    
2.
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019;133:1703-14.  Back to cited text no. 2
    
3.
Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: Clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 2005;106:2491-7.  Back to cited text no. 3
    
4.
Lucioni M, Berti E, Arcaini L, Croci GA, Maffi A, Klersy C, et al. Primary cutaneous B-cell lymphoma other than marginal zone: Clinicopathologic analysis of 161 cases: Comparison with current classification and definition of prognostic markers. Cancer Med 2016;5:2740-55.  Back to cited text no. 4
    
5.
Grange F, Petrella T, Beylot-Barry M, Joly P, D'Incan M, Delaunay M, et al. BCL-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas. Blood 2004;103:3662-8.  Back to cited text no. 5
    
6.
Kempf W, Zimmermann AK, Mitteldorf C. Cutaneous lymphomas-an update 2019. Hematol Oncol 2019;37:43-7.  Back to cited text no. 6
    
7.
Navarrete-Dechent C, Del Puerto C, Abarzúa-Araya Á, Molgó M, Geller S, Andreani S, et al. Dermoscopy of primary cutaneous B- and T-cell lymphomas and pseudolymphomas presenting as solitary nodules and tumors: A case-control study with histopathologic correlation. Int J Dermatol 2019;58:1270-6.  Back to cited text no. 7
    
8.
Bombonato C, Pampena R, Lallas A, Giovanni P, Longo C. Dermoscopy of lymphomas and pseudolymphomas. Dermatol Clin 2018;36:377-88.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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