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CASE REPORT
Year : 2012  |  Volume : 57  |  Issue : 1  |  Page : 45-47
Blastic plasmacytoid dendritic cell neoplasm presenting as fever with diffuse cutaneous nodules


1 Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore, India
2 Department of Haematology, Christian Medical College, Vellore, India
3 Department of Pathology, Christian Medical College, Vellore, India

Date of Web Publication10-Mar-2012

Correspondence Address:
C V Dincy Peter
Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore-632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.92677

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   Abstract 

A young man, presented with high-grade fever and disseminated asymptomatic skin lesions of 6-weeks duration. Cutaneous examination revealed multiple infiltrated monomorphic skin-colored papules and nodules upto 2Χ2 cm all over scalp, face, trunk and extremities. Light microscopy of nodules showed diffuse infiltration of dermis and subcutis by a tumor composed of medium to large cells with round to ovoid nuclei with fine chromatin, few with visible nucleoli and scanty to moderate amounts of eosinophilic cytoplasm. Tumor cells were positive for CD4, CD8, CD56 and negative for CD30, terminal deoxynucleotidyl transferase and Alk-1. Excised axillary lymph node showed similar morphologic and immunohistochemical findings. There was bone marrow involvement with infiltrate of large atypical/immature lymphoid cells. Diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. This is a rare neoplasm. presenting commonly in the skin, with or without concurrent extracutaneous disease.


Keywords: Blastic plasmacytoid dendritic cell neoplasm, cutaneous nodules, fever


How to cite this article:
Dincy Peter C V, Chakrapani A, Shah S, Shah A, Srivastava A. Blastic plasmacytoid dendritic cell neoplasm presenting as fever with diffuse cutaneous nodules. Indian J Dermatol 2012;57:45-7

How to cite this URL:
Dincy Peter C V, Chakrapani A, Shah S, Shah A, Srivastava A. Blastic plasmacytoid dendritic cell neoplasm presenting as fever with diffuse cutaneous nodules. Indian J Dermatol [serial online] 2012 [cited 2019 Dec 8];57:45-7. Available from: http://www.e-ijd.org/text.asp?2012/57/1/45/92677



   Introduction Top


The differential diagnosis for fever with cutaneous nodules are numerous. We describe one of the less common causes in this case report.


   Case Report Top


A 27-year-old gentleman presented to the dermatology department with high-grade fever of 3 months and generalized asymptomatic skin lesions of one and a half months duration. He also had knee joint arthritis, bleeding from the gums, hoarseness of voice and significant weight loss.

On examination, he was febrile, had enlarged cervical, axillary and inguinal nodes upto 1.5 cm in size and symmetrical arthritis of the knee joints.

Cutaneous examination revealed multiple infiltrated monomorphic skin-colored papules and nodules [Figure 1] over scalp, face, trunk and extremities. He had infiltration of the lid margins, gingival hyperplasia and a mucosal nodule at the anterior pillar of left tonsillar fossa. Biopsies from a cutaneous nodule [Figure 2] and [Figure 3] and an axillary lymph node were obtained.
Figure 1: Infiltrated papules and nodules over the trunk

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Figure 2: Pandermal infiltrates of atypical cells, (H and E, original magnification ×100)

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Figure 3: Atypical blastic appearing cells, (H and E, original magnification ×400)

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Histopathology and immunochemistry findings

Histopathology of the cutaneous nodules showed diffuse infiltration of the dermis and subcutis by a tumour composed of medium to large cells with round to ovoid nuclei with fine chromatin, few with visible nucleoli, increased mitosis and scanty to moderate amounts of eosinophilic cytoplasm. There was no epidermotropism. Tumor cells were positive for CD4, CD56 [Figure 4] and negative for CD8, CD68, CD30, terminal deoxynucleotidyl transferase (TdT), Alk-1 and myeloperoxidase (MPO). Histopathology of the excised axillary lymph node showed effacement of architecture due to diffuse infiltrate of large sized cells with scanty to moderate amounts of cytoplasm, round to oval or irregularly contoured vesicular nuclei with prominent nucleoli and increased mitosis. The neoplastic cells had same immunoprofile as described. The MIB- 1 proliferation index was 90%. There was bone marrow involvement with diffuse infiltrate of atypical cells.
Figure 4: CD56-positive cells, (original magnification ×400)

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   Discussion Top


The clinical differential diagnoses considered in this patient were cutaneous T-cell lymphoma, cutaneous infiltrates of a myelomonocytic variant of myeloid leukemia, extranodal NK/T-cell lymphoma, Sweets syndrome associated with a myeloproliferative disorder and a lepromatous Hansen's disease in type 2 reaction. Histopathology and relevant laboratory tests like skin smears ruled out the latter two conditions. The diagnosis of CD4 + CD56+ hematodermic neoplasm (Blastic plasmacytoid dendritic cell neoplasm) was made based on the histopathology and immunohistochemistry findings. The tumor cells of blastic plasmacytoid dendritic cell neoplasm have blastoid morphology resembling lymphoblasts or myeloblasts histologically and they are positive for CD4 and CD56, and in some cases for TdT, but do not express MPO or surface CD3. Since lymphoblastic and myeloblastic neoplasms can also be positive for CD56, CD4 and TdT, stains for CD3 and MPO should always be performed in order to exclude these entities. Similarities exists between CD56+ myeloid leukemia and CD4 + CD56+ hematodermic neoplasm in histology and immunophenotype; however, presence of CD33, MPO and lysozyme is used to diagnose myelomonocytic myeloid leukemia. The distinctive features of extranodal NK/T-cell lymphoma is the presence of angiotropism and necrosis, CD4 negativity and an association with Epstein-Barr Virus.

According to the WHO/EORTC classification CD4 + /CD56+ hematodermic neoplasm is placed under precursor hematological neoplasm and renamed by WHO as blastic plasmacytoid dendritic cell neoplasm. [1] This entity was also known as blastic NK cell lymphoma, agranular CD4 + natural killer cell leukemia and agranular CD4 + /CD56+ hematodermic neoplasm.

This is a rare neoplasm and accounts for 0.7% of cutaneous lymphomas. [2],[3] It most commonly presents in the skin, with or without concurrent extracutaneous disease. Cutaneous disease may be the first detected manifestation of systemic disease in blood, bone marrow, lymph node and other organs including the central nervous system. [4] Characteristic skin lesions include infiltrated purple-colored papules and nodules. While localized disease responds well to chemotherapy with radiotherapy, the prognosis of blastic plasmacytoid dendritic cell neoplasm is poor. Treatment consists of aggressive chemotherapy with or without radiotherapy. Studies revealed that the patients treated with allogenic or autologous stem cell transplantation had a significantly better prognosis than those treated with only chemotherapy and radiation therapy. Allogenic stem cell transplantation has been attempted with success in few cases. [5],[6] Single agent low-dose etoposide may be useful in some patients. The overall survival ranges from 1.5 to 2 years with current therapy.

Learning points

  • Blastic plasmacytoid dendritic cell neoplasm is a rare neoplasm constituting 0.7% of cutaneous lymphomas
  • Infiltrated purple-colored papules and nodules are the characteristic skin lesion
  • Prognosis of blastic plasmacytoid dendritic cell neoplasm is poor.


 
   References Top

1.Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization Classification of Tumors: Pathology and Genetics of Tumours of Hematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2008.  Back to cited text no. 1
    
2.Feuillard J, Jacob MJ, Valensi F, Maynadie M, Gressin R, Chaperot L, et al. Clinical and biologic features of CD4 + CD56+ malignancies. Blood 2002;99:1556-63.  Back to cited text no. 2
    
3.Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, et al. Blastic NK-cell lymphomas (agranular CD4+ CD56+ hematodermic neoplasms). Am J Clin Pathol 2005;123:662-75.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
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
[PUBMED]  [FULLTEXT]  
5.Jacob MC, Chaperot L, Mossuz P, Feuillard J, Valensi F, Leroux D, et al. CD4 + CD56+ lineage negative malignancies: A new entity developed from malignant early plasmacytoid dendritic cells. Haematologica 2003;88:941-55.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Karube K, Ohshima K, Tsuchiya T, Yamaguchi T, Suefuji H, Suzumiya J, et al. Non-B, non-T neoplasms with lymphoblast morphology: Further clarification and classification. Am J Surg Pathol 2003;27:1366-74.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  


    Figures

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



 

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    Abstract
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   Case Report
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    References
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