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 Table of Contents  
ONLINE ONLY - CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 1  |  Page : 53

Leukemia cutis: A sign of relapse


1 Department of Dermatology, Venereology and Leprosy, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India
2 Department of Pathology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India

Date of Submission10-Sep-2020
Date of Decision27-Dec-2020
Date of Acceptance02-Jan-2021
Date of Web Publication25-Feb-2022

Correspondence Address:
Asha B Panchagavi
OPD No: 14, Department of Dermatology, Venereology and Leprosy, SDM College of Medical Sciences and Hospital, Sattur, Dharwad - 580 009, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cdr.cdr_120_20

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  Abstract 


Leukemia cutis is an infiltration of the skin by myeloid or lymphoid neoplastic leukocytes resulting in clinically identifiable cutaneous lesions. It may follow, precede, or occur concomitantly with the diagnosis of systemic leukemia. A 55-year-old female patient was a known case of acute myeloid leukemia (AML) and was on palliative chemotherapy. She presented to the Department of Dermatology with sudden-onset nodules over the chest and abdomen which were multiple, nontender and firm in consistency. Histopathological examination from the nodule over the abdomen suggested leukemic infiltration of the skin and repeat hemogram showed reappearance of blast cells in the peripheral smear, consistent with a diagnosis of leukemia cutis with relapsed AML. The case has been reported for its rarity and poor prognosis associated with its presentation.

Keywords: Leukemia cutis, nodules, relapse


How to cite this article:
Chitapur UG, Athanikar SB, Panchagavi AB, Naveen K N, Athanikar VS. Leukemia cutis: A sign of relapse. Clin Dermatol Rev 2022;6:53

How to cite this URL:
Chitapur UG, Athanikar SB, Panchagavi AB, Naveen K N, Athanikar VS. Leukemia cutis: A sign of relapse. Clin Dermatol Rev [serial online] 2022 [cited 2022 May 17];6:53. Available from: https://www.cdriadvlkn.org/text.asp?2022/6/1/53/338582




  Introduction Top


Leukemia cutis is an infiltration of the skin by myeloid or lymphoid neoplastic leukocytes resulting in clinically identifiable cutaneous lesions.[1] It may follow, precede, or occur concomitantly with the diagnosis of systemic leukemia. It usually has a poor prognosis since it suggests dissemination or relapse of systemic leukemia, requiring early diagnosis, and aggressive treatment. We present a rare case of leukemia cutis with relapsed acute myeloid leukemia (AML) in a 55-year-old female patient because of its rarity.


  Case Report Top


A 55-year-old female patient had presented with complaints of easy fatigability and giddiness to the department of medicine. The routine blood investigations were done which showed: Hemoglobin-3.8 g%, platelet count-61,000 lakhs/cmm, Total leukocyte count-21,620 cells/cmm, Differential leukocyte count: Neutrophils-10%, Lymphocytes-12%, Eosinophils-2%, Basophils-00%, Monocytes-00%, Monoblasts-45%, Myeloblasts-31%. Peripheral smear study showed sparsely distributed red blood cells (RBCs) with predominant microcytic hypochromic cells and white blood cells were increased in the count with Myeloblasts-31%, Monoblasts-45%, Neutrophils-10%, Lymphocytes-12%, Eosinophils-02% and decreased platelet count suggestive of AML [Figure 1]. The patient was referred to a hematologist for further evaluation who performed bone marrow aspiration. It showed 32% blast cells with increased myelopoiesis, suppressed erythropoiesis, and megakaryocytes were decreased in number [Figure 2]. Flow cytometry was positive for CD11, CD13, CD15, CD14, CD33, CD64, CD117, and CD38. The patient was started on palliative treatment with injection decitabine 50 mg in normal saline over 2–3 h and packed RBC transfusion was done to improve hemoglobin levels. The patient withstood chemotherapy well and was symptomatically better.
Figure 1: Leishman-stained peripheral smear showing multiple blast cells (×100)

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Figure 2: Leishman-stained bone marrow slide showing multiple blast cells (×100)

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After completing three cycles of decitabine, the patient presented with nodular lesions over the chest and abdomen which were sudden in onset and progressive in nature. This was accompanied by easy fatiguability. On examination, multiple skin-colored nodules were present over the chest and abdomen [Figure 3] and [Figure 4]. These nodules were 1–2 cm, firm in consistency, nontender. Pallor was present on general physical examination. Systemic examination revealed splenomegaly and mucosal examination was normal. Repeat hemogram was done which showed reappearance of blast cells in peripheral smear. Biopsy was taken from the nodule over the abdomen which showed thinned out epidermis, atypical myeloid cells in clusters in the dermis, subcutaneous tissue and also infiltrating adnexal tissue. The cells were round with round-to-oval nuclei with multiple inconspicuous nucleoli and moderate cytoplasm suggestive of leukemic infiltration of skin [Figure 5]. Hence, the diagnosis of leukemia cutis with relapsed AML was made based on the above results. She was started on other chemotherapeutic agents such as cytarabine and mitoxantrone; however, no response was seen and the patient eventually died.
Figure 3: Multiple skin colored, nontender nodules over chest

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Figure 4: Multiple skin colored, nontender nodules over abdomen

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Figure 5: HPE of nodule from the abdomen showing leukemic infiltration of the skin (×100)

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  Discussion Top


Leukemia cutis is the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutaneous tissue, resulting in clinically identifiable cutaneous lesions. Skin lesions mostly develop after or concurrently with the diagnosis of myeloid disorder (60% and 25%, respectively) and only in approximately 5% of cases do they develop before any detectable bone marrow or blood involvement as an aleukemic leukemia cutis.[2],[3],[4]

Leukemia cutis is most common in patients with myeloid neoplasms, especially in AML and in leukemias with monocytic differentiation, such as chronic myelomonocytic leukemia. The risk of leukemia cutis is higher in AML with neoplastic cells displaying the chromosomal translocation t(8,21). Within the lymphocytic neoplasms, the risk of leukemia cutis is relatively high for adult T-cell leukemia/lymphoma, T-cell prolymphocytic leukemia, and CLL.[5]

The molecular basis underlying the migration of leukemic cells to the skin in leukemia cutis is not fully understood. Homing of malignant cells to the skin may depend on specific chemokine receptors, such as chemokine receptor type 4 (CCR4) interacting with specific intercellular adhesion molecules (ICAM) such as ICAM-1.[6]

Cutaneous lesions of leukemia can be classified into two groups; true leukemic infiltrations or specific lesions (leukemia cutis) and leukemids or nonspecific lesions. Leukemia cutis is the result of the dissemination of leukemic cells to the skin. The clinical appearance is variable with erythematous to violaceous papules or nodules being the most frequent lesions (60%), followed by infiltrated plaques, generalized cutaneous eruption, and erythroderma. The nodules are frequently asymptomatic and firm or rubbery in consistency. Unusual presentations include those resembling figurate erythemas, guttate psoriasis, stasis dermatitis, and ulcers. M4 and M5 AML may present with gingival hypertrophy secondary to leukemic infiltration, while those with acute myelomonocytic leukemia may have leonine facies.[7]

Nonspecific cutaneous signs of leukemia are much more common, occurring in up to 30%–40% of patients with leukemia (can be inflammatory, paraneoplastic, or secondary to marrow failure). The most common nonspecific signs are hemorrhagic, including petechiae, purpura, and ecchymosis. Other signs related to marrow failure include those associated with infections, such as varicella-zoster, herpes simplex, or cutaneous mycoses. The reactive or paraneoplastic lesions include generalized pruritus, Sweet's syndrome, pyoderma gangrenosum, exfoliative dermatitis, urticarial erythema multiforme, erythema nodosum, ichthyosis, and vasculitis.[7]

The diagnosis of the specific type of leukemia depends on a detailed examination of the peripheral blood and bone marrow. The diagnosis of AML is established by the presence of >20% myeloblasts in blood or bone marrow. In most cases, the immunohistochemical study is necessary to characterize the immunophenotype of tumor cells. Specific cell markers include CD3 (T-cells), CD20 (B-cells), CD30 (activated T-cells), CD45RO (mature T-cells), CD45 (lymphocytes, monocytes), CD43 (T-cells, monocytes, granulocytes), and CD68 (monocytes).[8]

Histopathology of the skin lesion shows diffuse infiltration of the dermis with leukemic cells with a higher concentration around the blood vessels and appendages. AML characteristically has large blast cells with a vesicular, round, or oval nucleus and basophilic cytoplasm with abundant mitotic activity. In contrast, CML has a pleomorphic infiltrate containing eosinophils and granulocytes in different stages of differentiation. ALL has an infiltrate of large blast-like cells having scanty cytoplasm, while CLL has small mature lymphocytes. The monocytic leukemias have monocytoid cells with prominent nuclei and fine chromatin pattern.[8]

Current treatment involves chemotherapy. The various antineoplastic agents used in the treatment of AML are Daunorubicin, Idaurubicin, Cytarabin, Tretinoin, Gemtuzimab, Etoposide, and methotrexate. Radiotherapy is considered to be beneficial in widespread skin involvement. The prognosis is poor and most patients die within months of diagnosis of leukemia cutis.


  Conclusion Top


Leukemia cutis is the infiltration of the skin with leukemic cells suggesting dissemination of leukemia or relapse of leukemia carrying a poor prognosis.

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.
Wagner G, Fenchel K, Back W, Schulz A, Sachse MM. Leukemia cutis - epidemiology, clinical presentation, and differential diagnoses. J Dtsch Dermatol Ges 2012;10:27-36.  Back to cited text no. 1
    
2.
Bénet C, Gomez A, Aguilar C, Delattre C, Vergier B, Beylot-Barry M, et al. Histologic and immunohistologic characterization of skin localization of myeloid disorders: A study of 173 cases. Am J Clin Pathol 2011;135:278-90.  Back to cited text no. 2
    
3.
Su WP. Clinical, histopathologic, and immunohistochemical correlations in leukemia cutis. Semin Dermatol 1994;13:223-30.  Back to cited text no. 3
    
4.
Vishalakshi V, Torsekar RG, Shinde S. Aleukemic leukemia cutis. Indian J Dermatol Venereol Leprol 2007;73:109-11.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Gniadecki R. The skin and disorders of the haematopoietic and immune systems. In: Griffiths CE, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Text Book of Dermatology. 9th ed. West Sussex , UK: Blackwell Publication; 2016. p. 148.2-4.  Back to cited text no. 5
    
6.
Petrella T, Meijer CJ, Dalac S, Willemze R, Maynadié M, Machet L, et al. TCL1 and CLA expression in agranular CD4/CD56 hematodermic neoplasms (blastic NK-cell lymphomas) and leukemia cutis. Am J Clin Pathol 2004;122:307-13.  Back to cited text no. 6
    
7.
Rao AG, Danturty I. Leukemia cutis. Indian J Dermatol 2012;57:504.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Verma K, Sahni K. Lymphoproliferative disorders. In: Sacchidanand S, Oberai C, Inamadar AC, editors. IADVL Textbook of Dermatology. 4th ed. Mumbai, India: Bhalani Publishing House; 2015. p. 2193-4.  Back to cited text no. 8
    


    Figures

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



 

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