The clinical appearance of leukemia cutis varies, with the most common lesions being erythematous to violaceous papules or nodules (60%), followed by infiltrating plaques, generalized cutaneous eruption, and erythroderma.[5] Oftentimes, they have no symptoms.[6] Usually having a solid or rubbery consistency, the nodules might turn purpuric in patients who are thrombocytopenic.[5]
Leukemia cutis may develop concurrently with the diagnosis of systemic leukemia, come first, or come later. Most cases of leukemia cutis occur in the context of an existing leukemia (44–77%) or at the time of systemic leukemia presentation (23–44%).[5] On rare occasions, leukemia cutis may occur before leukemia in the bone marrow or peripheral blood becomes observable.[7]
Leukemia cutis is linked to a number of gene abnormalities, such as chromosome 8 numerical anomalies, translocation (8; 21) (q22; q22), and inversion (16) (p13; q22).[8][9]
Mechanism
Leukemic skin involvement's pathogenic mechanism is poorly understood.[10] Nonetheless, it's thought that chemokine receptors, adhesion molecules, and the genetic features of leukemia all have an impact.[11]Adhesion molecules, specifically chemokineintegrin, may have a role in the migration of leukemic cells into the skin through processes known as skin-selective homing.[12][13]Adhesion molecules and chemokine receptors may also have significant effects. On the dermal post-capillary venules, TARC (thymus- and activation-regulated chemokine)/CCL17 (CC chemokine ligand 17) and/or E-selectin may interact with the cutaneous leucocyte-associated antigen (CLA) receptor and CC chemokine receptor 4 (CCR4) on the leukemic cells. Leukemic cell migration and binding into the dermis may be facilitated by this process. Additionally, integrins and endothelial-bound chemokines may interact to promote leukemic cell migration into the dermis.[10]
Diagnosis
Leukemia cutis is diagnosed by looking at the morphologic pattern of skin infiltration, cytologic characteristics, and most importantly the tumor cells' immunophenotype. The diagnosis is frequently confirmed by correlation with peripheral blood and bone marrow results as well as clinical data.[11]
Treatment
Leukemia cutis is a localized symptom of a systemic underlying disease that requires systemic therapy tailored to the individual subtype of leukemia. Hematologic remission typically happens in tandem with a full or partial response to cutaneous infiltrations in the majority of individuals.[10] Local radiotherapy, however, may be employed in patients with resistant leukemia cutis or recurrent skin infiltration.[14] The treatment of refractory cutaneous leukemia has led to the proposal of simultaneous integrated boost with helical arc radiotherapy of total skin (HEARTS) for cutaneous symptoms.[15]
Outlook
The prognosis is poor, with many patients suffering from additional extramedullary diseases and low survival rates. Most patients pass away months after being diagnosed. Patients without skin lesions who have acute myelogenous leukemia had a 30% survival rate after two years, but those with skin lesions have a 6% survival rate, which indicates an unfavorable prognosis for leukemia cutis.[5]
Epidemiology
Leukemia cutis can occur anywhere between 2% and 30% of the time, depending on the underlying leukemia diagnosis.[10]
^Agis, H.; Weltermann, A.; Fonatsch, C.; Haas, O.; Mitterbauer, G.; Müllauer, L.; Schreiber, S.; Schwarzinger, I.; Juretzka, W.; Valent, P.; Jäger, U.; Lechner, K.; Geissler, K. (2002-01-23). "A comparative study on demographic, hematological, and cytogenetic findings and prognosis in acute myeloid leukemia with and without leukemia cutis". Annals of Hematology. 81 (2). Springer Science and Business Media LLC: 90–95. doi:10.1007/s00277-001-0412-9. ISSN0939-5555. PMID11907789. S2CID9364519.
^Kubonishi, Ichiro; Seto, Masao; Murata, Naoaki; Kamioka, Mikio; Taguchi, Hirokuni; Miyoshi, Iaso (1998). "Translocation (10;11)(p13;q13) and MLL Gene Rearrangement in a Case of AML (M5a) with Aggressive Leukemia Cutis". Cancer Genetics and Cytogenetics. 104 (1). Elsevier BV: 28–31. doi:10.1016/s0165-4608(97)00414-7. ISSN0165-4608. PMID9648554.
^ abCho-Vega, Jeong Hee; Medeiros, L. Jeffrey; Prieto, Victor G.; Vega, Francisco (2008). "Leukemia Cutis". American Journal of Clinical Pathology. 129 (1): 130–142. doi:10.1309/WYACYWF6NGM3WBRT. ISSN0002-9173. PMID18089498.
^Li, Alvin W.; Yin, Emily S.; Stahl, Maximilian; Kim, Tae Kon; Panse, Gauri; Zeidan, Amer M.; Leventhal, Jonathan S. (2017). "The skin as a window to the blood: Cutaneous manifestations of myeloid malignancies". Blood Reviews. 31 (6). Elsevier BV: 370–388. doi:10.1016/j.blre.2017.07.003. ISSN0268-960X. PMID28732587.
^Elsayad, Khaled; Oertel, Michael; Haverkamp, Uwe; Eich, Hans Theodor (2017-01-16). "The effectiveness of radiotherapy for leukemia cutis". Journal of Cancer Research and Clinical Oncology. 143 (5). Springer Science and Business Media LLC: 851–859. doi:10.1007/s00432-016-2338-6. ISSN0171-5216. PMID28093639. S2CID24622600.
