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LETTER TO EDITOR
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 27-28

Leukemia cutis presenting as non-healing ulcers with marginal umbilicated nodules


1 Department of Dermatology, Siksha O Anusandhan University, Bhubaneswar, Odisha, India
2 Pathology, IMS and SUM Hospital, Siksha O Anusandhan University, Bhubaneswar, Odisha, India

Date of Submission29-Sep-2020
Date of Decision01-Jun-2021
Date of Acceptance10-Jun-2021
Date of Web Publication11-Aug-2021

Correspondence Address:
Ishan Agrawal
Department of Dermatology, IMS and SUM Hospital, K-8, Kalinga Nagar, Bhubaneswar, Odisha.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_108_20

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How to cite this article:
Ray A, Agrawal I, Patnayak R, Kar BR. Leukemia cutis presenting as non-healing ulcers with marginal umbilicated nodules. Indian J Dermatopathol Diagn Dermatol 2021;8:27-8

How to cite this URL:
Ray A, Agrawal I, Patnayak R, Kar BR. Leukemia cutis presenting as non-healing ulcers with marginal umbilicated nodules. Indian J Dermatopathol Diagn Dermatol [serial online] 2021 [cited 2021 Dec 2];8:27-8. Available from: https://www.ijdpdd.com/text.asp?2021/8/1/27/323702



Sir,

Leukemia cutis (LC) is a cutaneous infiltration of leukemic cells, commonly manifesting as nodules, plaques, and patches, rarely as bullae and ulcers. It is mostly reported with acute myeloid leukemia (AML) suggesting extramedullary involvement and poor prognosis.[1] Unusually, LC may be the first evidence of an underlying hematological malignancy. We report an elderly female presenting with non-healing ulcers on bilateral lower limbs, subsequently diagnosed as acute myelomonocytic leukemia (AML-M4). Additionally, there were unusual umbilicated nodules causing clinical bafflement.

A 58-year-old female presented with painful necrotic ulcers over both legs. Before 2 months, multiple erythematous papules appeared on the legs, which evolved into intact bullae that collapsed showing a rapidly progressive ulcer with increasing lesional pain. She had low-grade fever, generalized fatigue, and arthralgia in both knees. The patient had no known comorbidities. On examination, multiple shallow, necrotic ulcers of different sizes, with differentially raised margins, thick adherent hemorrhagic crusting, and non-indurated base, were present over both the legs. Distinct bluish-gray, umbilicated nodules were clustered around the ulcer [Figure 1]A. Peripheral lymph nodes were non-palpable. The patient was on oral linezolid for a week, after culture there was Staphylococcal growth, but ulcer size and depth continued to increase.
Figure 1: (A) Multiple shallow, necrotic ulcers with distinct bluish-gray perilesional umbilicated nodules (black arrow). (B) Decrease in ulcer size and depth after treatment with Sorafinib and Azacytidine

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Blood reports showed gross derangement with hemoglobin 3.3 g/dl, RBC count 1.01×106/μL, and platelet count 7000/μL. Peripheral smear showed 53% blasts (occasional thin Auer rods with prominent nucleoli).

Bone marrow aspirate showed 60% blasts with >3% myeloperoxidase (MPO) positivity and dysplasia. Flow cytometry of bone marrow aspirate was positive for CD34, CD7, CD4, CD13, CD33, CD64, CD14, CD11c, CD117, HLA-DR, diagnostic of AML-M4 (French-American-British subtype).

Biopsy of the umbilicated nodule showed a typical Grenz zone, perivascular lymphoplasmacytic cells with multiple atypical cells and intracytoplasmic inclusion bodies (Mott cells) [Figure 2]A and B, and immunohistochemistry positive for CD68 and CD117 [Figure 3]A and B, supporting a diagnosis of AML-M4 with LC. Hematology consultation revealed poor prognostic factors like Fms-like tyrosine kinase 3-internal tandem duplication (FLT3-ITD)-positive mutation and complex karyotyping (trisomy 7, 8, and 20). After treatment with Sorafinib and Azacytidine, the ulcer size started to decrease [Figure 1B].
Figure 2: (A) Histopathology showing Grenz zone, perivascular lymphoplasmacytic cells with multiple atypical cells (H&E stain, 10×). (B) Intracytoplasmic inclusion bodies (Mott cells) (yellow arrow) (H & E stain, 40×)

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Figure 3: Immunohistochemistry positively stained for (A) CD68 and (B) CD117 (10×)

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The proposed mechanisms of leukemic cells infiltration into the skin are by selective homing of memory T-cells aided by chemokines, integrin, and adhesion molecules.[2] Exceptionally, the patient may first present to the dermatologist, as in our case. LC usually manifests as erythematous rubbery nodules, over face, trunk, and extremities.[3] Ulcers, as seen in our patient, are hardly reported.[2],[3]

Presence of umbilicated nodules added to the clinical perplexity. Bullae with rapidly progressing necrotic ulcer raised a strong suspicion of paraneoplastic bullous pyoderma gangrenosum, ruled out by histopathological absence of neutrophilic infiltrates. Large non-healing ulcers with marginal umbilicated nodules, as seen in our patient, are a rare presentation of LC. Previously, only a couple of instances of LC manifesting as umbilicated bullae[4] and papules[5] have been reported.

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.
Findakly D, Amar S. A rare case of leukemia cutis as the first presentation of a myelodysplastic syndrome to acute myeloid leukemia transformation. Cureus 2020;12:e8698.  Back to cited text no. 1
    
2.
Chang YW, Lee CH, Tseng HC. Leukemia cutis in a medical center in southern Taiwan: A retrospective study of 42 patients. J Formos Med Assoc 2021;120:226-33.  Back to cited text no. 2
    
3.
Rao AG, Danturty I. Leukemia cutis. Indian J Dermatol 2012;57:504.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Pinto B, Dhir V, Dambalkar A, De D, Kumar M, Sharma P. Bullous lesion with central umbilication in leukemia. Int J Dermatol 2014;53:145-6.  Back to cited text no. 4
    
5.
Verma P, Sharma S, Sharma R, Yadav P. Leukemia cutis manifesting umbilicated cutaneous lesions. Indian J Dermatol Venereol Leprol 2012;78:752-4.  Back to cited text no. 5
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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