School of Medicine and Health Sciences Poster Presentations

Going Skin Deep: A Case of Nodulo-Pustular Lesions in Acute Myeloid Leukemia

Poster Number

141

Document Type

Poster

Publication Date

3-2016

Abstract

Case description: A 59 year old female with acute myeloid leukemia M7 variant presented with one week of progressive facial, head, and neck lesions preceded by a small right thigh macular lesion that had developed into a larger papulo-pustular lesion. Subsequently, a number of similar papular lesions erupted across her forehead and grew into painful nodulo-pustular lesions. Associated right sided periorbital edema prompted her hospitalization.

She has a history of recurrent upper respiratory infections, pulmonary aspergillosis, staphylococcus epidermidis vertebral osteomyelitis, diabetes mellitus, and genital herpes. For AML, she had received two remission induction cycles of idarubicin/cytarabine, then high dose ara-C five months prior to admission. For persistent neutropenia, she required daily granulocyte-colony stimulating factor. Other medications included carvedilol, lisinopril, gabapentin, lorazepam, oxycodone, and valacyclovir.

Admission vital signs were unremarkable. Examination revealed cervical lymphadenopathy and multiple nodulo-pustular lesions scattered across the forehead and neck, with a similar lesion on her right thigh. Lesions ranged from papules with surrounding erythema to 2 cm indurated erythematous nodules with central ulcerations and drainage. Right sided periorbital edema was present without visual field deficits, conjunctival injection, or extraocular movement pain. Labs included: WBC 3,720 with ANC of 2,976, hemoglobin 9.4 g/dL, platelets of 80,000, HCO3 21 mEQ/L without anion gap, glucose 115 mg/dL, and lactate 1.5 mEq/L. A skin biopsy was obtained, and IV vancomycin, acyclovir and voriconazole were initiated due to concern for superinfection. Lesions continued to progress to pustules that eventually crusted. Biopsy revealed acute neutrophilic dermatitis. Antibiotics were discontinued and prednisone was initiated with further improvement. Two months later, she was rehospitalized for bilateral pneumonia with skin lesion recurrence, now on her chest and left wrist. Skin biopsy again confirmed acute neutrophilic dermatitis.

Discussion: Sweet Syndrome is associated with acute myeloid leukemia. Though typically presenting as well-demarcated papules and plaques with surrounding erythematous base, this presentation was atypical, as lesions were predominantly nodulo-pustular. She had multiple risk factors for Sweet Syndrome that may have contributed including chronic intermittent upper respiratory infections. Even after she steroid treatment at initial presentation, she had recurrence in the setting of pneumonia, suggesting an infectious precipitant. She also received daily tbo-filgrastim, a growth factor associated with Sweet Syndrome when neutrophils rapidly increase. The patient’s M7 variant of AML also provides a unique aberration that may have made her more susceptible. In patients with a combination of risk factors including URIs, AML, and G-CSF use, Sweet Syndrome may present atypically. Skin biopsy should be considered in such patients to confirm diagnosis and initiate appropriate treatment with corticosteroids.

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Presented at: GW Research Days 2016

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Going Skin Deep: A Case of Nodulo-Pustular Lesions in Acute Myeloid Leukemia

Case description: A 59 year old female with acute myeloid leukemia M7 variant presented with one week of progressive facial, head, and neck lesions preceded by a small right thigh macular lesion that had developed into a larger papulo-pustular lesion. Subsequently, a number of similar papular lesions erupted across her forehead and grew into painful nodulo-pustular lesions. Associated right sided periorbital edema prompted her hospitalization.

She has a history of recurrent upper respiratory infections, pulmonary aspergillosis, staphylococcus epidermidis vertebral osteomyelitis, diabetes mellitus, and genital herpes. For AML, she had received two remission induction cycles of idarubicin/cytarabine, then high dose ara-C five months prior to admission. For persistent neutropenia, she required daily granulocyte-colony stimulating factor. Other medications included carvedilol, lisinopril, gabapentin, lorazepam, oxycodone, and valacyclovir.

Admission vital signs were unremarkable. Examination revealed cervical lymphadenopathy and multiple nodulo-pustular lesions scattered across the forehead and neck, with a similar lesion on her right thigh. Lesions ranged from papules with surrounding erythema to 2 cm indurated erythematous nodules with central ulcerations and drainage. Right sided periorbital edema was present without visual field deficits, conjunctival injection, or extraocular movement pain. Labs included: WBC 3,720 with ANC of 2,976, hemoglobin 9.4 g/dL, platelets of 80,000, HCO3 21 mEQ/L without anion gap, glucose 115 mg/dL, and lactate 1.5 mEq/L. A skin biopsy was obtained, and IV vancomycin, acyclovir and voriconazole were initiated due to concern for superinfection. Lesions continued to progress to pustules that eventually crusted. Biopsy revealed acute neutrophilic dermatitis. Antibiotics were discontinued and prednisone was initiated with further improvement. Two months later, she was rehospitalized for bilateral pneumonia with skin lesion recurrence, now on her chest and left wrist. Skin biopsy again confirmed acute neutrophilic dermatitis.

Discussion: Sweet Syndrome is associated with acute myeloid leukemia. Though typically presenting as well-demarcated papules and plaques with surrounding erythematous base, this presentation was atypical, as lesions were predominantly nodulo-pustular. She had multiple risk factors for Sweet Syndrome that may have contributed including chronic intermittent upper respiratory infections. Even after she steroid treatment at initial presentation, she had recurrence in the setting of pneumonia, suggesting an infectious precipitant. She also received daily tbo-filgrastim, a growth factor associated with Sweet Syndrome when neutrophils rapidly increase. The patient’s M7 variant of AML also provides a unique aberration that may have made her more susceptible. In patients with a combination of risk factors including URIs, AML, and G-CSF use, Sweet Syndrome may present atypically. Skin biopsy should be considered in such patients to confirm diagnosis and initiate appropriate treatment with corticosteroids.