Leukemia Cutis

Leukemia Cutis

Jeremy C. Wallentine, MD

Clinical image shows 2 small papules in a patient with acute myeloid leukemia. Histologic examination confirmed leukemic infiltrates consistent with myeloid sarcoma. (Courtesy V. Tonkovic-Capin, MD.)

A punch biopsy shows leukemic infiltrates in the superficial and deep dermis, extending into the subcutaneous adipose tissue. Sparing of the epidermis with a thin Grenz zone image is noted.



  • Leukemia cutis (LC)


  • Myeloid sarcoma (MS), granulocytic sarcoma, extramedullary myeloid cell tumor, chloroma

    • When composed of myeloid cells

  • Monoblastic sarcoma

    • When composed of monocytic precursors

  • Primary extramedullary leukemia


  • Clinically identifiable cutaneous lesions secondary to cutaneous infiltration by neoplastic leukocytes (myeloid or lymphoid)

  • Leukemia cutis commonly used to describe lymphocytic leukemias involving skin

    • Designated by precursor B- or T-cell lineage and chronic lymphocytic leukemia

    • “Myeloid sarcoma” is preferred term when leukemic cells are of myeloid or monocytic lineage


Mechanisms of Skin Homing

  • Underlying process has not been defined

    • May involve coexpression of cutaneous lymphocyte antigen (CLA) and its interaction with specific chemokines

    • Other factors may include CCR4, TARC, and CCL22

  • Predilection for sites with cutaneous inflammation (e.g., Sweet syndrome, psoriasis)



  • Incidence

    • Acute monocytic, myelomonocytic, and T-cell leukemias show highest incidence of leukemia cutis

    • LC can be observed in all forms of acute myeloid leukemia (AML)

    • Occurs in 10-15% of patients with AML

      • Varies widely with AML subtype

      • Up to 50% of patients with acute myelomonocytic and monocytic types

    • Less frequent in patients with myeloproliferative and myelodysplastic neoplasms

      • Rare reports of CML with LC presentation

      • Usually related to disease progression &/or transformation

    • Is seen in up to 25% of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) cases

    • Occurs in 20-70% of mature T-cell leukemias

      • 40-70% of adult T-cell leukemia/lymphoma (ATLL)

      • 25-30% of T-cell prolymphocytic leukemia (T-PLL)

    • Unusual in patients with precursor B- or T-cell lymphoblastic leukemia/lymphomas (LBL) and plasma cell leukemia

    • Occurs in 25-30% of infants with congenital leukemia

  • Age

    • Frequency higher among children than adults


  • Most commonly involves extremities (legs > arms)

  • Back, chest, scalp, and face may also be involved

  • Preferential involvement at sites of previous or concomitant inflammation

    • Sweet syndrome

    • Herpes zoster

    • Insect bites

    • Psoriasis


  • Presentation may precede (aleukemic LC), coincide with, or manifest as recurrence of acute leukemia

    • Most cases present after diagnosis of systemic leukemia

    • < 10% present prior to blood or bone marrow involvement (aleukemic LC or primary extramedullary leukemia)

  • Single or multiple skin lesions

    • Violaceous, red-brown, or hemorrhagic papules, nodules, and plaques of varying sizes

    • Erythematous papules and nodules most commonly reported

    • Eczematous lesions

    • Ulcers

    • “Blueberry muffin” appearance

      • Firm blue, red, or purple nodules in generalized distribution

      • Term historically used to describe cutaneous involvement in children with congenital leukemia

  • Oral petechiae

  • Thickening of the gums

    • Oral lesions more common in adults

      • Rare in congenital leukemias

  • Other sites of extramedullary involvement are frequent (e.g., meninges)

Laboratory Tests

  • Lactate dehydrogenase and β2-microglobulin

    • Higher levels reported in patients with leukemia cutis

  • Serology for HTLV-1 in cases of ATLL


  • Options, risks, complications

    • Managed by treating underlying leukemia

      • Systemic chemotherapy

      • Bone marrow transplantation

    • Local therapy (e.g., radiation)

    • Aleukemic LC should not be managed differently from patients with known leukemia


  • Poor prognosis

    • Generally a manifestation of disease progression

  • Leukemia cutis in context of congenital myelogenous leukemia is an exception

    • Not associated with worse prognosis

    • Spontaneous regression of LC without treatment has been observed

  • Prognosis in CLL patients is good

    • Exception of blastic transformation (Richter syndrome) is associated with poor prognosis


General Histologic Features

  • Low-power patterns of involvement

    • Perivascular &/or periadnexal

    • Dense and diffuse

    • Nodular

    • Subtle superficial interstitial infiltrate (rare)

  • Stromal fibrosis

  • Lineage assignment unreliable with histology alone

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Leukemia Cutis

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