Case Report - (2022) Volume 8, Issue 6
Received: 08-May-2022, Manuscript No. IPCPDR-22-13311; Editor assigned: 11-May-2022, Pre QC No. IPCPDR-22-13311(PQ); Reviewed: 25-May-2022, QC No. IPCPDR-22-13311; Revised: 10-Oct-2022, Manuscript No. IPCPDR-22-13311(R); Published: 17-Oct-2022, DOI: 10.36648/2472-0143.8.6.29
Leukemia Cutis (LC) is a term used to describe the infiltration of leukemic cells into the dermis or sub cutis tissue, resulting in different forms of skin manifestations. Aleukemia leukemia cutis refers to the presence of LC prior to the development of systemic leukemia. Leukemia cutis is more commonly associated with Acute Myeloid Leukemia (AML) compared to Acute Lymphoblastic Leukemia (ALL), although the latter accounts for the majority of acute leukemia cases in childhood. We report a child who presented with a rare and unusual cutaneous pattern of angioedema like lesion caused by leukemia cutis.
Leukemia Cutis (LC); Acute Myeloid Leukemia (AML); Acute Lymphoblastic Leukemia (ALL); Pancytopenia
Leukemia is the commonest childhood malignancy which accounts for 30% of all cancers diagnosed in the pediatric population. Although ALL makes up for the majority of cases of acute leukemia in childhood, AML is more frequently associated with LC. In children, the onset of LC usually happens concomitantly or after systemic disease has been diagnosed. At times, LC may precede the development of leukemic cells in the peripheral blood or bone marrow. This condition is known as aleukemic lukemia cutis [1-7]. the occurrence of leukemia cutis is not only confined to children with high risk leukemia but also occurs in children with standard and low risk leukemia. LC appears to occur more commonly in children as compared to adults with AML [8]. We report a case of aleukemic leukemia cutis with an unusual presentation of angioedema like features in a child.
This is a previously well 5 years old girl who presented to the emergency department with a history of fever and epistaxis for 2 days duration. Upon examination, she appeared to be mildly pale with hepatomegaly. There was no lymphadenopathy. Full Blood Count (FBC) showed pancytopenia with Hemoglobin (Hb) 9.8 g/dL, total White Count (TWC) 1.9 x 109 /L (absolute neutrophil count of 0.06) and platelet count 22 x 109/L. Peripheral Blood Film (PBF) showed anemia with leucoerythroblastic picture and increased schistocytes. No blasts or abnormal lymphoid cells were seen. During her hospital stay she became hypotensive and was admitted to the Paediatric Intensive Care Unit (PICU) for neutropenia septic shock, requiring intravenous antibiotics and inotropic support. Blood culture done revealed no growth of organism. Ultrasound abdomen did not show any intra abdominal collection. Bone Marrow Aspiration and Trephine biopsy (BMAT) reported edematous marrow with an excess of blasts cells which are likely to be haematogones. BMA Immunophenotyping showed the presence of 4.69-6.98% of CD10+ B-lymphoblast. No cytogenetic abnormalities were detected. BMA culture did not grow any organism. Cerebral Spinal Fluid (CSF) examination was clear from blasts or abnormal lymphoid cells. She was discharged home after 3 weeks of hospital stay when her fever subsided, and her clinical condition improved. Serial blood count monitoring revealed persistent bicytopenia (lymphopenia with anaemia). Approximately 7 weeks from her initial presentation, she started to develop progressive bilateral and symmetrical erythematous swellings over both lower eyelids. This was followed by perioral and lip oedema, resembling a child with angioedema (Figures 1 and 2). There was no involvement of the oral mucosal. There was no associated wheezing, shortness of breath, skin pruritus or pain. There was no family history of angioedema. At the same time, she was found to have developed generalized lymphadenopathy with hepatosplenomegaly. Repeated FBC showed bicytopenia with mild anaemia (Hb 10 g/dL) and leucopenia (TWC 3.3 x 109/L, ANC 1.48). Her platelet counts were normal (PLT 205 x 109/L). Repeated PBF did not reveal evidence of blast cells. MRI brain and orbit was done showing bilateral eyelid thickening with normal brain structure. A decision was made to perform a skin biopsy at the left lower eyelid as well as a cervical lymph node biopsy. The histopathological findings from the biopsy showed infiltration of malignant cells (Figures 3 and 4). Approximately 4 weeks after her biopsy, repeated BMAT showed the presence of excessive blasts cells of 85% in the bone marrow, which were morphologically consistent with leukemic cells, confirming a diagnosis of B cell ALL. She was started on chemotherapy following the all AEIOP BFM 2009 (International collaborative treatment protocol for children and adolescents with acute lymphoblastic leukemia). The per orbital and perioral swelling improved significantly within 5 days of starting chemotherapy [9,10].
Figure 1: Clinical picture of patient showing bilateral symmetrical firm swellings involving the lower eye lids and the lips as well as the chin, mimicking angioedema.
Figure 2: Marked improvement and reduction of perioral and periorbital swelling after 5 days of chemotherapy.
Figure 3: Lower eyelid biopsy: Neoplastic cells diffusely infiltrating the fibro collagenous tissue and muscle fibers of the lower eyelid. H and E staining.
Figure 4: Higher magnification shows lymphoblasts in between the muscle fibers. these cells have round to oval to convoluted nuclei, inconspicuous nucleoli and scant cytoplasm. H and E staining.
Leukemia cutis has been reported to present with a variety of manifestations which includes macules, papules, patches, infiltrated plaques, nodules, petechiae or purpura. These lesions have a propensity to develop over the head, neck and scalp areas. In newborns, the term “blueberry muffin” is often used to describe LC which presents as multiple erythematous to lilaceous skin nodules. Current published data in the literature reveals that about one third of cases of leukemia presents simultaneously with LC. It was found that less than 10% of cases of leukemia start with an initial skin presentation of aleukemic leukemia cutis. Although acquired angioedema has been reported to be associated with lymphoproliferative diseases in adulthood, to our best knowledge, LC mimicking angioedema in children has not been reported in the literature to date. In the case study reported above, the child was found to have pancytopenia during her initial presentation. However, the first PBF and BMAT done did not reveal the presence of blasts cells. Repeated serial PBF remained clear from malignant cells despite the development of angioedema like features. Skin biopsy over the eyelid and lymph node biopsy from the cervical region finally clinched the diagnosis of malignancy, showing malignant lymphoid tissues with mitotic. Differential diagnoses of hereditary angioedema and acquired angioedema were considered in this case. Given the lack of a positive family history of angioedema, it was thought that acquired angioedema due to C1 inhibitor (AAE-C1-INH) deficiency was more likely to be the diagnosis. We were unable to exclude the diagnosis C1 inhibitor deficiency in this patient as the laboratory testing was not available in our clinical setting. However, the progressive swelling of her eyelids and lips over a period of 2 weeks did not fit into the typical scenario seen in AAE-C1-INH, of which, symptoms typically resolve within 2-5 days. Skin biopsy eventually allowed for a diagnosis of malignancy in our patient. To date, there is no consensus of the association between the presence of LC and the prognosis of leukemia in childhood. Some authors have considered LC to be an indicator of poorer prognosis or even an indicator of higher chance of relapse of disease. Our patient was classified as having standard risk leukemia and was started on chemotherapy accordingly. Her facial swelling resolved with no residual dyspigmentation or scarring after commencement of treatment. More studies are required to determine whether aleukemic cutis is associated with poorer prognosis in childhood leukemia.
Acute leukemia in children is known to be associated with a variety of cutaneous findings. To the best of our knowledge, LC presenting as angioedema like facial lesions has not been reported in children previously. Our case not only adds to the new skin presentation of acute leukemia but also illustrates the importance of performing skin biopsy for any atypical skin presentation associated with abnormal blood counts because LC can precede the diagnosis of acute leukemia in childhood.
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Citation: Wong HL, Singh JKA, Tan SC, Hamid NSA (2022) Acute Lymphoblastic Leukemia Presenting as Angioedema like Cutaneous lesion in a Child. Clin Pediatr Dermatol. 8:29
Copyright: © 2022 Wong HL, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
