Leukemia Cutis in a 60 Year Male with Underlying Acute Myeloblastic Leukemia

Leukemia cutis is not a common manifestation of leukemia but when present it indicates poor prognosis. Here we report a case of an atypical presentation of acute myeloblastic leukaemia who presented with neurological features and skin eruption. Keyword: AML, Leukaemia Cutis, Poor Prognosis 1. Assistant Professor, Dhaka Medical College, Dhaka. 2. Assistant Professor, Central Medical College, Comilla. 3. Assistant Professor, Dhaka Medical College, Dhaka. 4. Assistant Registrar, Dhaka Medical College, Dhaka. 5. Medical Officer, Dhaka Medical College, Dhaka. 6. Assistant Professor, Central Medical College, Comilla. Correspondence : Dr. ZulfiqarHossain Khan, Assistant Professor, Dhaka Medical College, Dhaka. myelogenous leukemia cutis may produce an angiocentric infiltrate, although interstitial permeation among collagen bundles within the reticular dermis soon supervenes . Vague nodules may form as the dermis becomes progressively expanded by malignant cells, which express myeloperoxidase. Malignant myeloid cells forming the dominant cell type of acute myeloid leukemia cutis tend to appear monotonous and homogeneous cytologically. Nuclei are round to oval, chromatin may be evenly dispersed, and nucleoli are multiple but potentially inconspicuous. On rare occasion, immature eosinophils containing typical cytoplasmic granules in the absence of bilobed nuclear contours may be helpful in assigning a presumptive diagnosis3.The prognosis of leukemia cutis is directly related to the prognosis for systemic disease.Therapy is usually directed at the leukemia itself2 Case report: A 60 year old male was admitted in the department of medicine in DMCH with multiple skin coloured papules over scalp,face,trunk and proximal extremities for 03 months with generalized weakness and acute retention of urine. Initially the patient noticed sudden eruption of multiple papules on scalp and face. Rapidly lesions spread throughout the trunk and proximal upper extremities.Lesions are painless and nonpruritic.There is no history of fever,weight loss,gum bleeding or bony tenderness. Physical examination revealed severe anemia.There is no lymphadenopathy.Examination of the integumentary system revealed multiple skin coloured papules on scalp, face, proximal upper extremities and throughout the trunk. Lesions are nontender and hard in consistency. Abdominal examination revealed moderate hepatomegaly but no splenomegaly or ascitis. Other systemic examination revealed no abnormalities. J MEDICINE 2013; 14 : 91-94

myelogenous leukemia cutis may produce an angiocentric infiltrate, although interstitial permeation among collagen bundles within the reticular dermis soon supervenes .Vague nodules may form as the dermis becomes progressively expanded by malignant cells, which express myeloperoxidase.Malignant myeloid cells forming the dominant cell type of acute myeloid leukemia cutis tend to appear monotonous and homogeneous cytologically.Nuclei are round to oval, chromatin may be evenly dispersed, and nucleoli are multiple but potentially inconspicuous.On rare occasion, immature eosinophils containing typical cytoplasmic granules in the absence of bilobed nuclear contours may be helpful in assigning a presumptive diagnosis 3 .The prognosis of leukemia cutis is directly related to the prognosis for systemic disease.Therapy is usually directed at the leukemia itself 2

Case report:
A 60 year old male was admitted in the department of medicine in DMCH with multiple skin coloured papules over scalp,face,trunk and proximal extremities for 03 months with generalized weakness and acute retention of urine.Initially the patient noticed sudden eruption of multiple papules on scalp and face.Rapidly lesions spread throughout the trunk and proximal upper extremities.Lesions are painless and nonpruritic.There is no history of fever,weight loss,gum bleeding or bony tenderness.
Physical examination revealed severe anemia.There is no lymphadenopathy.Examination of the integumentary system revealed multiple skin coloured papules on scalp, face, proximal upper extremities and throughout the trunk.Lesions are nontender and hard in consistency.Abdominal examination revealed moderate hepatomegaly but no splenomegaly or ascitis.Other systemic examination revealed no abnormalities.The patient was given 2 units of blood transfusion for correction of severe anemia.Unfortunately he was discharged with risk bond and failed to treat and followed up properly.

Discussion:
A literature review showed that leukaemia cutis confined to the skin is extremely rare and commonly misdiagnosed. 8,9ts a poor prognostic sign.Cutaneous involvement with acute leukaemia is fairly uncommon and usually a relatively late event.Skin lesions in acute leukaemia may be specific, called leukaemia cutis, or non-specific, called leukaemids that include leukocytoclastic vasculitis, Sweet's syndrome, urticaria, Erythroderma and erythema nodosum.In a study of 42 patients with specific leukaemic infiltrates, Su et al 5 found that the most common lesions to be multiple papules and nodules (60%) andinfiltrated plaques (26%), with less frequent occurrence of macules, ulcers, ecchymoses, and palpable purpura.Leukaemia cutis occurs in about 20% of patientswith acute monocyticleukaemia(FAB-M5), 6 and is uncommonly seen in other forms of AML.
Ittypically manifests as red or violaceous papules or nodules, mainly on the face, and can become purpuric with coexisting thrombocytopenia, and is generallyasymptomatic.Skin infiltration favours the lower dermis and subcutaneous fat with prominent involvement of adnexal structures, nerves, vessels of the superficial and deep plexus as noted in the present case.Skin involvement is not related tothe circulating white-cell count but the skin can act as a sanctuary for leukaemic cells.Leukaemia cutis generally carries a poor prognosis, with a high incidence of extramedullary disease at other sites including the meninges, and with imminentbone marrow relapses and serial skin relapses.As observed in a previous report, standard induction chemotherapy for AML is less effective in patients who have cutaneous involvement.Nevertheless, with curative therapy directed at skin, bone marrow, and other sites of extramedullary involvement,longterm disease-free survival is possible. 7 herein described a patient with AML who presented with asymptomatic extensive skincoloured papules on scalp,face,trunk and proximal upper extremities as the initial clinical manifestation of the disease.This report represents one of the few cases of AML presenting with leukemia cutis.

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Fig.-1: Multiple skin coloured infiltrated papules throughout the scalp