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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 439-440
Blastic plasmacytoid dendritic cell neoplasm: A rare hematological entity with common skin presentation


1 From the Department of Dermatology and Venereology, Public Hospital of Şile, İstanbul, Turkey
2 Department of Pathology, University of Health Sciences, Haydarpaşa Numune Training and Research Hospital, Istanbul, Turkey

Date of Web Publication2-Nov-2022

Correspondence Address:
Gonca Sarac
From the Department of Dermatology and Venereology, Public Hospital of Şile, İstanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_310_22

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How to cite this article:
Sarac G, Güneş P. Blastic plasmacytoid dendritic cell neoplasm: A rare hematological entity with common skin presentation. Indian J Dermatol 2022;67:439-40

How to cite this URL:
Sarac G, Güneş P. Blastic plasmacytoid dendritic cell neoplasm: A rare hematological entity with common skin presentation. Indian J Dermatol [serial online] 2022 [cited 2022 Dec 4];67:439-40. Available from: https://www.e-ijd.org/text.asp?2022/67/4/439/360325




Sir,

A 60-year-old man referred to the dermatology outpatient clinic with an asymptomatic rash started 2 months ago. He did not have any other systemic disease or a routine medication. Physical examination revealed erythematous plaques on the face, trunk, arms, and upper parts of the legs, and violaceous tumoral lesions one on the chest, one on the back and one on the left arm [Figure 1]a, [Figure 1]b, [Figure 1]c.
Figure 1: (a and b) Generalized erythematous plaques and violaceous tumoral lesions. (c) Tumoral lesion on the left arm

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Punch biopsies from plaque and tumoral lesions were taken, considering tumoral stage mycosis fungoides, aggressive T-cell lymphomas and the cutaneous involvement of other hematological malignancies in clinical differential diagnoses.

Skin biopsy specimens showed a non-epidermotropic infiltration of immature blastic cells filling the entire dermis in a diffuse pattern [Figure 2]a, [Figure 2]b. In one of the biopsies, tumor cells extended deep into the reticular dermis, while infiltrating the subcutis in the other. The cells were 2-3 times the size of a mature lymphocyte, had scant cytoplasm, and nuclei with irregular fine granular chromatin and 1-2 nucleoli [Figure 2]c. High mitotic activity was accompanied.
Figure 2: (a and b) Nonepidermotropic diffuse infiltration in the dermis (H&E, x40). (c) Medium-sized cells with scant cytoplasm, irregular fine chromatin, and 1-2 nucleoli (H&E, x400)

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Immunohistochemical examination indicated diffuse and strong expression of CD4, CD56, CD123 and Bcl-2 within tumor cells [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. Small, cytoplasmic dot-like positivity detected with CD68. Ki67 proliferation index was 70-80%. The diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was made by histopathological and immunohistochemical findings.
Figure 3: (a) Positive for CD4 staining (x40). (b) Positive for CD56 staining (x100). (c) Positive for CD123 staining (x40). (d) Positive for Bcl-2 staining (x40)

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Patient's blood tests were within normal ranges. Bone marrow biopsy demonstrated BPDCN involvement. In positron emission tomography-computed tomography, an 11 × 35 mm soft tissue lesion showing hypermetabolism (SUV-max 3.2) in the left axilla was detected, no other focus suggesting metastasis was observed in another organ. Magnetic resonance imaging of the brain was negative. After the diagnosis was made, he was transferred to a hematology clinic in another hospital.

BPDCN is a rare hematological malignancy with high frequency of skin presentation. It is more common in men and the elderly. It shows an aggressive course, systemic involvement is generally found at the time of diagnosis. Bone marrow, peripheral blood and lymph nodes are the most frequently affected areas after the skin.[1] Diverse skin manifestations can be seen including bruise-like patches, single to few nodules/tumors or generalized plaques in erythematous to violaceous color. Lesions are mostly encountered on the head and neck, upper body and upper extremities, but they have also been reported in the oral and nasal mucosa.[2],[3] Cutaneous lesions of our patient were erythematous plaques and tumors, resembling tumoral stage mycosis fungoides, instead of bruise-like morphology that was frequently reported before. The proliferation of the lesions in a relatively short period of time for mycosis fungoides indicates that the patient had a more aggressive malignancy.

Histopathologically, cutaneous lesions of BPDCN have atypical, medium-sized mononuclear cells with blastic chromatin, indistinct nucleoli and scant agranular eosinophilic cytoplasm. They constitute monomorphous, non-epidermotropic infiltrates that is mostly in a nodular/diffuse pattern, filling the entire dermis or extending into subcutaneous tissues.[4],[5] Histopathology may also morphologically resemble acute myelogenous leukemia cutis or show features suggestive of natural killer/T-cell cytotoxic lymphoma, thus immunohistochemical staining is essential for diagnosis.

Positivity of CD4, CD56, CD123, CD303 (BDCA2), TCL-1 and Bcl-2 with negativity of lineage-specific markers of T-cells, B-cells and myelomonocytic cells favor BPDCN. Plasmacytoid dendritic cells, the precursor of neoplastic cells, are CD4, CD56 and CD123 positive.[4],[5]

BPDCN responds to chemotherapies, but recurrence is seen in a short time after chemotherapy. Prolonged remission has been reported in patients undergoing hematopoietic stem cell transplantation in the first complete remission following chemotherapy.[1],[3] Familiarity with the cutaneous manifestations of BPDCN can prevent diagnostic delay in this potentially fatal disease and enable prompt initiation of treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Sapienza MR, Pileri A, Derenzini E, Melle F, Motta G, Fiori S, et al. Blastic plasmacytoid dendritic cell neoplasm: State of the art and prospects. Cancers (Basel) 2019;11:595.  Back to cited text no. 1
    
2.
Julia F, Petrella T, Beylot-Barry M, Bagot M, Lipsker D, Machet L, et al. Blastic plasmacytoid dendritic cell neoplasm: Clinical features in 90 patients. Br J Dermatol 2013;169:579-86.  Back to cited text no. 2
    
3.
Brüggen MC, Valencak J, Stranzenbach R, Li N, Stadler R, Jonak C, et al. Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: A retrospective multicentre study. J Eur Acad Dermatology Venereol 2020;34:1489-95.  Back to cited text no. 3
    
4.
Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L, et al. Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol 2010;34:75-87.  Back to cited text no. 4
    
5.
Cerroni L. Skin Lymphoma: The Illustrated Guide. 5th ed. Wiley Blackwell; 2020.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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