Oral Leukemia Cutis and Facial Sweet’s Syndrome Associated with Acute Myeloid Leukemia

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Dear Editor, Leukemia is a malignant neoplasm of the hematopoietic system. Cutaneous manifestations of leukemia are called leukemia cutis (LC).[1] Sweet’s syndrome (SS), is a rare inflammatory condition, generally classified as idiopathic, malignancy-associated, or drug-induced.[2,3] We herein present a case of a 55-year-old female patient with onset of acute myeloid leukemia (AML) associated with febrile neutropenia. She presented with erythematous, edematous papules/plaques and vesicle-like lesions on her face [Figures 1 and 2], along with painful necrotic ulcers on the oral mucosa [Figure 3]. For histopathological estimation, two biopsies were performed. Facial lesions were found to be compatible with bullous SS [Figures 4 and 5], while the oral mucosa was compatible with LC [Figure 6]. Immunohistochemistry results were positive for myeloperoxidase, CD34, and CD117 [Figure 7].Figure 1: Skin lesions: Erythematous, edematous papules and plaques with vesicle-like lesions on the cheeks, forehead, and chinFigure 2: Skin lesions: Erythematous, edematous papules and plaques with vesicle-like lesions on the cheeks, forehead, and chinFigure 3: Oral lesions: Painful necrotic ulcers on the buccal mucosa, palate, and upper lipFigure 4: Subepidermal blister associated with perivascular neutrophil infiltrate in the dermis. No vasculitis. Compatible with bullous SS. (H/E X10)Figure 5: Dermis with diffuse inflammatory infiltrate, predominantly perivascular and periadnexal neutrophils, compatible with SS. (H/E X40)Figure 6: Infiltration of the lamina propria of the oral mucosa by blast cells. (H/E X40)Figure 7: Immunohistochemistry: Myeloperoxidase-positive. (X40)The patient was receiving chemotherapy along with premedication corticosteroids; thus, topical corticosteroids were indicated for the facial lesions. This led to the resolution of the skin lesions and clinical improvement of the oral ulcers, associated with the initiation of chemotherapy. LC is the cutaneous infiltration of neoplastic leukocytes or their precursors. The term “leukemid” refers to a cutaneous paraneoplastic disorder, which is more common and frequently presents clinically as cytopenias owing to bone marrow failure or drug eruptions.[4] LC may develop as a skin infiltration after the onset of leukemia, or it may develop simultaneously, as seen in our patient. Additionally, skin infiltration can occur by leukemic cells without blasts in peripheral blood samples, a condition referred to as aleukaemic LC. The incidence of AML with LC ranges from 5% to 10%.[1] LC can present with a wide range of clinical features, mainly erythematous to violaceous papules, nodules, or plaques. Any location can be involved, including the mucosa and genitalia, though the trunk and extremities are the most common sites. Oral manifestations typically present as gingival hyperplasia.[1-4] The diagnosis of LC must be confirmed by histopathology, and its management is directed to the treatment of the underlying leukemia.[4] SS is a neutrophilic dermatosis. Approximately 85% of patients with malignancy-associated SS have an underlying hematological malignancy, with AML being the most common.[2] SS is characterized by the abrupt onset of tender or painful erythematous plaques and nodules, often associated with fever and a neutrophil leukocytosis. Lesions predominantly affect the head, neck, upper trunk, and upper arms. However, there have been very few case reports of SS localized on the face. SS associated with AML may present with atypical clinical features, as observed in our patient. The diagnosis is both clinical and histopathological, and systemic corticosteroids are considered the “gold standard” for treatment.[3-5] Many conditions can co-exist with SS, such as LC, as seen in our patient. Early recognition and treatment of malignancy-associated SS are crucial to provide timely anti-cancer treatments.[2] In conclusion, LC lesions can present anywhere on the skin, including the mucosa, and can coexist with SS. The wide variability in appearance, distribution, and stages of leukemia at presentation, necessitates considering LC for any new skin lesions, with immediate skin biopsy to confirm the diagnosis. LC is associated with poorer overall survival and more extramedullary involvement.[1,2] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In these forms, the patient(s) has/have given their consent for 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. However, complete anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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