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Black and white autoimmunity: early warning colours of adrenal crisis
Clinical Picture
Black and white autoimmunity: early warning colours of adrenal crisis Thomas Cuny, Marc Klein
A 31-year-old woman was admitted to the Department of Endocrinology, University Hospital of Nancy, with abdominal pain and vomiting that had been ongoing for 3 days. She had noticed a darkening of her skin colour, with some areas spared, over the previous 3 months and reported having asthenia, episodic diarrhoea, and weight loss for more than 12 months. Physical examination showed a non-segmental acrofacial vitiligo with an intense black and white contrast (figure A, D) and a cervical thyroidectomy scar as a result of surgery to treat Grave’s disease (figure C). Both low plasma cortisol (19·2 nmol/L [normal range 185–624]) and high adrenocorticotropic hormone (74·1 pmol/L [1–10·7]) concentrations confirmed a primary adrenal insufficiency. This diagnosis was supported by visualisation of atrophy of the adrenal glands on abdominal CT (figure E, arrows). Presence of 21-hydroxylase autoantibodies confirmed an autoimmune cause, and associated Grave’s disease and vitiligo suggested a diagnosis of autoimmune polyglandular syndrome (APS) type 2. Melanodermia resulted from high melanocyte-stimulating hormone concentrations released concomitantly A
B
with adrenocorticotropic hormone during the chronic adrenal insufficiency phase, with progression over time reflecting progressive deterioration of endogenous cortisol production. Melanodermia, especially on the face, gradually faded once hydrocortisone replacement therapy was started (figure A, B, C). Although rare, sharply contrasting vitiligo and melanodermia should lead clinicians to suspect adrenal insufficiency. In our patient, the contrasting presentation of the two simultaneous clinical signs of autoimmune disease prompted us to reach a diagnosis of primary adrenal insufficiency, which is almost always found at presentation of APS type 2.
Lancet Diabetes Endocrinol 2016 Published Online October 10, 2016 http://dx.doi.org/10.1016/ S2213-8587(16)30117-6 Endocrinology, University Hospital of Nancy, Vandœuvre-lès-Nancy, France (T Cuny MD, Prof M Klein MD) Correspondence to: Dr Thomas Cuny, Endocrinology, University Hospital of Nancy, 54500 Vandœuvre-lès-Nancy, France [email protected]
Contributors TR did the clinical assessment and investigation and took the pictures of the patient and the CT scan. Both authors were responsible for the care of the patient. TC prepared the draft of this Clinical Picture and MK reviewed and revised it. Both authors approved the final submitted version. Written consent to publish was obtained from the patient. Declaration of interest We declare no competing interests.
C
D
E
Figure: Clinical and CT findings in autoimmune polyglandular syndrome type 2 Sharp contrast between vitiligo and melanodermia a few days before the occurrence of the decompensated adrenal insufficiency (A), and 6 months (B) and 12 months (C) after hydrocortisone therapy substitution. Mild signs of Cushing’s syndrome suggest an overtreatment (C). (D) Vitiligo of the extremities. (E) Axial sequences of abdominal CT scan showing a severe atrophy of the two adrenal glands (arrows).
www.thelancet.com/diabetes-endocrinology Published online October 10, 2016 http://dx.doi.org/10.1016/S2213-8587(16)30117-6
1
Black and white autoimmunity: early warning colours of adrenal crisis Thomas Cuny, Marc Klein
A 31-year-old woman was admitted to the Department of Endocrinology, University Hospital of Nancy, with abdominal pain and vomiting that had been ongoing for 3 days. She had noticed a darkening of her skin colour, with some areas spared, over the previous 3 months and reported having asthenia, episodic diarrhoea, and weight loss for more than 12 months. Physical examination showed a non-segmental acrofacial vitiligo with an intense black and white contrast (figure A, D) and a cervical thyroidectomy scar as a result of surgery to treat Grave’s disease (figure C). Both low plasma cortisol (19·2 nmol/L [normal range 185–624]) and high adrenocorticotropic hormone (74·1 pmol/L [1–10·7]) concentrations confirmed a primary adrenal insufficiency. This diagnosis was supported by visualisation of atrophy of the adrenal glands on abdominal CT (figure E, arrows). Presence of 21-hydroxylase autoantibodies confirmed an autoimmune cause, and associated Grave’s disease and vitiligo suggested a diagnosis of autoimmune polyglandular syndrome (APS) type 2. Melanodermia resulted from high melanocyte-stimulating hormone concentrations released concomitantly A
B
with adrenocorticotropic hormone during the chronic adrenal insufficiency phase, with progression over time reflecting progressive deterioration of endogenous cortisol production. Melanodermia, especially on the face, gradually faded once hydrocortisone replacement therapy was started (figure A, B, C). Although rare, sharply contrasting vitiligo and melanodermia should lead clinicians to suspect adrenal insufficiency. In our patient, the contrasting presentation of the two simultaneous clinical signs of autoimmune disease prompted us to reach a diagnosis of primary adrenal insufficiency, which is almost always found at presentation of APS type 2.
Lancet Diabetes Endocrinol 2016 Published Online October 10, 2016 http://dx.doi.org/10.1016/ S2213-8587(16)30117-6 Endocrinology, University Hospital of Nancy, Vandœuvre-lès-Nancy, France (T Cuny MD, Prof M Klein MD) Correspondence to: Dr Thomas Cuny, Endocrinology, University Hospital of Nancy, 54500 Vandœuvre-lès-Nancy, France [email protected]
Contributors TR did the clinical assessment and investigation and took the pictures of the patient and the CT scan. Both authors were responsible for the care of the patient. TC prepared the draft of this Clinical Picture and MK reviewed and revised it. Both authors approved the final submitted version. Written consent to publish was obtained from the patient. Declaration of interest We declare no competing interests.
C
D
E
Figure: Clinical and CT findings in autoimmune polyglandular syndrome type 2 Sharp contrast between vitiligo and melanodermia a few days before the occurrence of the decompensated adrenal insufficiency (A), and 6 months (B) and 12 months (C) after hydrocortisone therapy substitution. Mild signs of Cushing’s syndrome suggest an overtreatment (C). (D) Vitiligo of the extremities. (E) Axial sequences of abdominal CT scan showing a severe atrophy of the two adrenal glands (arrows).
www.thelancet.com/diabetes-endocrinology Published online October 10, 2016 http://dx.doi.org/10.1016/S2213-8587(16)30117-6
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