Leukemia cutis C95.9

Leukemia is a diffuse autonomous proliferation of a leukocyte species. The expansion of a malignant cell clone leads to a generalized spread in the hematopoietic bone marrow and, in addition, to a possible infiltration of extramedullary organs, including the skin, as well as to an exudation of leukemic cells into the blood.

Leukaemia cutis is the term used to describe specific infiltrates of the skin in the various leukaemias, irrespective of their provenance.

3 disease groups are distinguished (frequencies according to the American Cancer Society)

• Acute leukemia (AL) (C95.00)
• Acute myeloid le ukemia (AML/32%) (C92.00)
• Acute lymphoblastic leukemia (ALL/10%) (C91.00)
• Chronic lymphocytic le ukemia (CLL/35%) (C91.10)
• Chronic myeloid le ukemia (CML/14%) (C92.10)
• Blastic plasmacytoid dendritic cell neoplasia (BPDCN)
• Other leukemias (11%)

In clinical practice, specific skin changes are observed mainly in the following forms of leukemia:

• chronic lymphocytic leukemia (CLL)
• and
• acute myeloid leukaemia (AML).

Very rare are specific cutaneous involvement in chronic myeloid le ukemia (CML). Their occurrence may indicate a blast relapse of CML. This is also true for acute lymphoblastic leukemia(ALL).

More common are non-specific skin changes. These can occur in almost all forms of leukemia. Viral, bacterial and mycotic infections are triggered by the consequences of the onset of immunosuppression. Increasingly, also ADRs caused by chemotherapeutic agents, monoclonal antibodies and other targeted tumor therapeutics are becoming the focus of dermatological attention.

Dermatological symptoms due to specific skin infiltrates are usually observed after the clinical diagnosis has been made, rarely as a primary manifestation of the disease.

• Chronic lymphocytic leukemia:
  • Specific skin manifestations (in 10% of patients): Generalized lymph node swelling and leukemic exanthema: Red to red-blue, possibly maculopapular PLaques, flat to bulbous, coarse infiltrates, especially in the area of the forehead, cheeks, nose and ears. Cutaneous tumors also occur, sometimes confluent tumors and infiltrates to the facies leontina. Furthermore: Lymphadenosis cutis circumscripta.
  • Non-specific skin symptoms (leukaemias, in 28% of patients): pruritus, prurigo, chronic urticaria, petechiae, purpura, ecchymosis, zoster generalisatus, herpes simplex.
• Myeloid leukemias:
  • Specific skin manifestations (relatively rare): Squamous to nodular infiltrations of the skin.
  • Nonspecific skin lesions: pallor in secondary anemia, hemorrhages in skin and mucosa with tendency to ulcerative-necrotic transformation, especially in acute myeloid leukemia; generalized pruritus, prurigo simplex subacuta, macular, nodular, figured erythema; erythrosquamous or vesiculobullous eruptions; very rarely universal nonspecific erythroderma.
• Immature cell leukemias (myelo- or paramyeloblastic leukemia)
  :Thrombopenic purpura, formation of necrosis with tendency to decay in the oral cavity, genital and anal areas (due to accompanying agranulocytosis), candidiasis of the mucous membranes, gingival hyperplasia, possibly generalized lymph node enlargement.

See also skin changes in myeloproliferative neoplasms.

Eruption of lymphocyte recovery: An "eruption of lymphocyte recovery" (ELR) is a pseudotumor-like reaction of the skin that can occur 1-2 weeks after aggressive myeloablative polychemotherapy (Hurabielle Cet al. 2018).

Primary cutaneous B- and T-cell lymphomas

Treatment of the underlying disease. Treatment of skin lesions depending on the clinic.

Cutaneous specific infiltration in the setting of a hematologic neoplasm is generally indicative of advanced disease and should prompt examination of other body sites for extramedullary involvement. However, in rare cases, cutaneous lesions may present as the primary manifestation of systemic hematologic disease (Robak E et al. 2021).

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