An approach to hypopigmentationBMJ 2017; 356 doi: https://doi.org/10.1136/bmj.i6534 (Published 12 January 2017) Cite this as: BMJ 2017;356:i6534
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Although not specifically highlighted in the practice point(1), the prevalence of vitiligo in autoimmune Addison's disease is sufficiently high (10.6%-18%) (2)(3) to justify a high index of suspicion for this endocrine disorder during evaluation of patients with vitiligo.
In one study, among 322 patients with autoimmune polyglandular syndrome type 2 (APS-2) (in which Addison's disease is always present) (4), the prevalence of vitiligo was 10.6%, and the prevalence of type 1 diabetes mellitus (DM1) was 15.8% (2). In a previous study, among 125 patients in whom Addison's disease was associated with a variety of autoimmune disorders, the prevalence of vitiligo was 14% (3). In the presence of both vitiligo and DM1 the index of suspicion for Addison's disease should be especially high, given the prevalence of 10.6% and 15.8%, respectively, for vitiligo and DM1 in the study of 322 patients with APS-2 (2).
Among patients with DM1 the onset of Addison's disease may be heralded by recurrent unexplained hypoglycaemia (5)(6). In one such patient, who also had vitiligo, Addisonian hyperpigmentation had a truncal distribution (5), and in another, hyperpigmentation only had a scrotal distribution (6). In fact, patients with Addison's disease need not be hyperpigmented, a clinical observation documented as long ago as 1964 (7).
(1) Hill JP., Batchelor JM. An approach to hypopigmentation. BMJ 2017;356:i6534
(2) Betterle C., Scarpa R., Garelli S et al. Addison's disease: a survey of 633 patients in Padova. European Journal of Endocrinology 2013;169:773-784
(3) Kasperlik-Zaluska AA., Migdalska B., Czarnocka B et al. Association of Addison's disease with autoimmune disorders- a long term observation of 180 patients
Postgrad Med J 1991;67:984-987
(4) Amerio P., Tracanna M., De Remigis P et al. Vitiligo associated with other autoimmune diseases: polyglandular autoimmune syndromes types 3B+C and 4. Clinical and Experimental Dermatology 2006;31:746-749
(5) Mc Aulay V., Frier BM. Addison's disease in type 1 diabetes presenting with recurrent hypoglycaemia. Postgrad Med J 2000;76: 230-232
(6) Passanisi S., Timpanaro T., Lo Presti D., Caruso-Nicoletti M. Recurrent hypoglycaemia in type 1 diabetes mellitus may unravel the association with Addison's disease: case report. BMC Research Notes 2014;7:634
(7) Allison MF., Bailey IS., Curtin DC. Crisis following corticotrophin in Addison's disease without hyperpigmentation. Postgrad Med J 1964;40:26-28
Competing interests: No competing interests