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Cutaneous Clues of Systemic Disease
Critical Care SESSION TITLE: Student/Resident Case Report Poster - Critical Care IV SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
Cutaneous Clues of Systemic Disease Ravi Patel MD*; and Stephen Clum MD University of South Florida, Tampa, FL INTRODUCTION: Systemic mastocytosis is a mast cell disorder with variable clinical manifestations ranging from isolated cutaneous lesions to systemic mastocytosis and mast cell leukemia. A comprehensive history and physical examination can yield findings that allow for recognition of systemic mastocytosis. CASE PRESENTATION: A 63-year-old female presented after cardiopulmonary arrest following lidocaine prior to a routine dental procedure. Following injection, she reported nausea, became unresponsive, and was found to be in pulseless electrical activity. Return of spontaneous circulation was achieved after advanced cardiac life support was administered. Her past medical history was significant for poor dentition and unspecified allergies. On arrival to the MICU, she was spontaneously moving all extremities, but did not follow commands. She had cutaneous lesions over her torso and lower extremities suggestive of urticaria pigmentosa. Diagnostic testing for cardiopulmonary causes for the patient’s circulatory collapse yielded no definitive etiology. Neurologic imaging showed no evidence of ischemia or hemorrhage, and electroencephalographic evaluation showed no evidence of seizure activity. DISCUSSION: Given her history of unspecified allergies and prominent cutaneous lesions, systemic mastocytosis was suspected. She had a positive Kit (c-kit) D816V mutation in peripheral blood, along with elevated serum tryptase of 53 ng/mL (normal reference range 2-10 ng/mL), supporting the diagnosis of systemic mastocytosis. She was treated with first and second-generation antihistamines, a leukotriene receptor antagonist, and a short course of low dose glucocorticoids. Her persistent neurologic dysfuction was felt to be due to anoxic injury during the cardiopulmonary arrest. She remained intubated for the duration of the hospitalization, and ultimately required a tracheostomy. Her family deferred confirmatory diagnosis of systemic mastocytosis via bone marrow biopsy, as it would not have provided any prognostic or clinical benefit.
Reference #1: Pardanani, Animesh. “Systemic Mastocytosis in Adults: 2015 Update on Diagnosis, Risk Stratification, and Management.” Am. J. Hematol. American Journal of Hematology 90.3 (2015): 250-62. Reference #2: Bains, Sonia N., and Fred H. Hsieh. “Current Approaches to the Diagnosis and Treatment of Systemic Mastocytosis.” Annals of Allergy, Asthma & Immunology 104 (2010): 1-10. Jan. 2010. Reference #3: Hennessy, Bryan, et al. “Management of Patients With Systemic Mastocytosis: Review of M. D. Anderson Cancer Center Experience.” American Journal of Hematology 77.3 (2004): 209-14. Nov. 2004. DISCLOSURE: The following authors have nothing to disclose: Ravi Patel, Stephen Clum No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.458
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
journal.publications.chestnet.org
445A
CRITICAL CARE
CONCLUSIONS: This case illustrates the complexity and severity of systemic mastocytosis. The anaphylactic reaction followed by complete vascular collapse resulted in a devastating neurological injury. Recognition of the dermatological manifestation of systemic mastocytosis can facilitate a rapid diagnosis as well as expedited intervention to anaphylactic reactions should this occur.
Cutaneous Clues of Systemic Disease Ravi Patel MD*; and Stephen Clum MD University of South Florida, Tampa, FL INTRODUCTION: Systemic mastocytosis is a mast cell disorder with variable clinical manifestations ranging from isolated cutaneous lesions to systemic mastocytosis and mast cell leukemia. A comprehensive history and physical examination can yield findings that allow for recognition of systemic mastocytosis. CASE PRESENTATION: A 63-year-old female presented after cardiopulmonary arrest following lidocaine prior to a routine dental procedure. Following injection, she reported nausea, became unresponsive, and was found to be in pulseless electrical activity. Return of spontaneous circulation was achieved after advanced cardiac life support was administered. Her past medical history was significant for poor dentition and unspecified allergies. On arrival to the MICU, she was spontaneously moving all extremities, but did not follow commands. She had cutaneous lesions over her torso and lower extremities suggestive of urticaria pigmentosa. Diagnostic testing for cardiopulmonary causes for the patient’s circulatory collapse yielded no definitive etiology. Neurologic imaging showed no evidence of ischemia or hemorrhage, and electroencephalographic evaluation showed no evidence of seizure activity. DISCUSSION: Given her history of unspecified allergies and prominent cutaneous lesions, systemic mastocytosis was suspected. She had a positive Kit (c-kit) D816V mutation in peripheral blood, along with elevated serum tryptase of 53 ng/mL (normal reference range 2-10 ng/mL), supporting the diagnosis of systemic mastocytosis. She was treated with first and second-generation antihistamines, a leukotriene receptor antagonist, and a short course of low dose glucocorticoids. Her persistent neurologic dysfuction was felt to be due to anoxic injury during the cardiopulmonary arrest. She remained intubated for the duration of the hospitalization, and ultimately required a tracheostomy. Her family deferred confirmatory diagnosis of systemic mastocytosis via bone marrow biopsy, as it would not have provided any prognostic or clinical benefit.
Reference #1: Pardanani, Animesh. “Systemic Mastocytosis in Adults: 2015 Update on Diagnosis, Risk Stratification, and Management.” Am. J. Hematol. American Journal of Hematology 90.3 (2015): 250-62. Reference #2: Bains, Sonia N., and Fred H. Hsieh. “Current Approaches to the Diagnosis and Treatment of Systemic Mastocytosis.” Annals of Allergy, Asthma & Immunology 104 (2010): 1-10. Jan. 2010. Reference #3: Hennessy, Bryan, et al. “Management of Patients With Systemic Mastocytosis: Review of M. D. Anderson Cancer Center Experience.” American Journal of Hematology 77.3 (2004): 209-14. Nov. 2004. DISCLOSURE: The following authors have nothing to disclose: Ravi Patel, Stephen Clum No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.458
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
journal.publications.chestnet.org
445A
CRITICAL CARE
CONCLUSIONS: This case illustrates the complexity and severity of systemic mastocytosis. The anaphylactic reaction followed by complete vascular collapse resulted in a devastating neurological injury. Recognition of the dermatological manifestation of systemic mastocytosis can facilitate a rapid diagnosis as well as expedited intervention to anaphylactic reactions should this occur.