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Diagnosis and Treatment > Signs and Symptoms > Poikiloderma

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Natasha Shrikrishnapalasuriyar Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Mirena Noyvirt Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Philip Evans Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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Bethan Gibson Department of Intensive Care, Royal Glamorgan Hospital, Llantrisant, UK

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Elin Foden Department of Intensive Care, Royal Glamorgan Hospital, Llantrisant, UK

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Atul Kalhan Department of Diabetes and Endocrinology, Royal Glamorgan Hospital, Llantrisant, UK

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A 54-year-old woman was admitted to hospital with a presumed allergic reaction to a single dose of amoxicillin given for a suspected upper respiratory tract infection. She complained of chest tightness although there was no wheeze or stridor. On examination, she was pyrexial, tachycardic, hypertensive and had a diffuse mottled rash on her lower limbs. Her initial investigations showed raised inflammatory markers. She was treated in the intensive care for a presumed anaphylactic reaction with an underlying sepsis. Further investigations including CT head and CSF examination were unremarkable; however, a CT abdomen showed a 10 cm heterogeneous right adrenal mass. Based on review by the endocrine team, a diagnosis of pheochromocytoma crisis was made, which was subsequently confirmed on 24-h urinary metanephrine measurement. An emergency adrenalectomy was considered although she was deemed unfit for surgery. Despite intensive medical management, her conditioned deteriorated and she died secondary to multi-organ failure induced by pheochromocytoma crisis.

Learning points:

  • Pheochromocytoma have relatively higher prevalence in autopsy series (0.05–1%) suggestive of a diagnosis, which is often missed.

  • Pheochromocytoma crisis is an endocrine emergency characterized by hemodynamic instability induced by surge of catecholamines often precipitated by trauma and medications (β blockers, general anesthetic agents, ephedrine and steroids).

  • Pheochromocytoma crisis can mimic acute coronary syndrome, cardiogenic or septic shock.

  • Livedo reticularis can be a rare although significant cutaneous marker of underlying pheochromocytoma crisis.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Hormone, Adrenaline, Dopamine, Metanephrines, Noradrenaline, Normetanephrine, Condition/ Syndrome, Phaeochromocytoma, Phaeochromocytoma crisis, Patient Demographics, Age, Adolescent/young adult, Gender, Female, Ethnicity, White, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Agitation, Altered level of consciousness, Atrial fibrillation, Breathing difficulties, Hypertension, Hypotension, Poikiloderma, Pyrexia, Rash, Sepsis, Tachycardia, Investigation, Adrenaline, Alanine aminotransferase, Bilirubin, Blood pressure, Catecholamines (24-hour urine), C-reactive protein, Creatinine, CT scan, Dopamine, Echocardiogram, Kidney function, Liver function, Metanephrines (urinary), Noradrenaline, Normetanephrine, Urea and electrolytes, White blood cell count, Intervention, Fluid repletion, Medication, Alpha-blockers, Amoxicillin, Antibiotics, Epinephrine, Glucocorticoids, Hydrocortisone, Norepinephrine, Phenoxybenzamine, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-17-0170
Volume/Issue:
Volume 2018: Issue 1
Open access