Unstable Vitiligo in the Setting of Diffuse Hypermelanosis: A Peculiar Presentation of Schmidt’s Syndrome

  • Rui Tavares Bello Unidade de Dermatologia/Dermatology Department, Hospital dos Lusíadas, Lisboa, Portugal
  • Carlos Tavares Bello Serviço de Endocrinologia/Endocrinology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal
  • João Sequeira Duarte Serviço de Endocrinologia/Endocrinology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal
  • Carlos Vasconcelos Serviço de Endocrinologia/Endocrinology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal
Keywords: Melanosis, Polyendocrinopathies, Autoimmune, Vitiligo


Vitiligo is an acquired prevalent pigmentary disorder characterized by its chronicity, refractoriness and significant psychosocial burden. Concerning its pathogenesis, several genetic, environmental, oxidative stress mechanisms and immune disturbances combine in an intricate, complex way. Diverse immunemediated comorbidities do occur in a variable yet significant manner, so as to fully justify the careful approach and follow-up of these patients. polyglandular autoimmune syndrome type II – Schmidt`s syndrome – is defined by the association of Addison`s disease with immunemediated thyroid disease and/or type 1 diabetes mellitus. The case of a 64year old Caucasian female patient with a long lasting history of several immunemediated disorders (namely Hashimoto thyroiditis and hypothyroidism, atrophic gastritis, lichen planus and stable acrofacial vitiligo) is reported. who For the last 3 months her vitiligo had suffered significant extension and morphologic changes – confettilike lesions, trichromic vitiligo and Koebner`s phenomenon – along with discrete and subtle generalized hypermelanosis. Laboratory work up disclosed a state of hypocortisolism –further confirmed by functional, immunological and image studies: autoimmune Addison`s disease in the context of Schmidt`s syndrome. An effective endocrine insufficiency correction prompted for a quick patient`s recovery as far as hydroelectrolytic and metabolic metabolism, with a concomitant reversion of the hypermelanosis and a stabilization of the vitiligo. In this case it is noteworthy the rapid expansion of a previous stable vitiligo along with the acquisition of peculiar new morphologic features, in the context of a subtle diffuse hypermelanosis in a patient with a past history of several immunemediated disorders. We underline the need to acknowledge the possible association of vitiligo with immunemediated comorbidities, particularly whenever atypical semiological or evolutive features are present.


Download data is not yet available.


Krüger C, Schallreuter KU. A review of the worldwide prevalence

of vitiligo in children/adolescents and adults. Int

J Dermatol.2012; 51:120612.

TaïebA, Picardo M, VETF Members. The definition and

assessment of vitiligo: a consensus report of the Vitiligo

European Task Force. Pigment Cell Res. 2007; 20:2735.

Sheth VM, Guo Y, Qureshi AA. Comorbidities associated

with vitiligo: a tenyear

retrospective study. Dermatology.

; 227:3115.

Laddha NC, Dwivedi M, Mansuri MS, Gani AR, Ansarullah

M, Ramachandran AV, et al. Vitiligo: interplay between

oxidative stress and imune system. Exp Dermatol.

; 22:24550.

Spritz RA. The genetics of vitiligo. J Invest Dermatol.

; 131:E18E20.

Le Poole IC, Das PK, van den Wijngaard RM, Bos JD, Westerhof

W. Review of the ethiopathomechanism of vitiligo:

A convergence theory. Exp Dermatol- 1993; 2:14553.

Gauthier Y, CarioAndré

M, Taïeb A. A critical appraisal

of vitiligo etiologic theories. Is melanocyte loss a

melanocytorrhagia? Pigment Cell Res. 2003; 16:3227.

Ezzedine K, Sheth V, Rodrigues M, Eleftheriadou V, Harris

J, Hamzavi IH, et al. Vitiligo is not a cosmetic disease. J

Am Acad Dermatol. 2015; 73:8835.

Passeron T. Leucodermies. EMC – Dermatologie. 2016;


Lee H, Lee MH, Lee DY, Kang HY, Kim KH, Choi GS, et

al. Prevalence of Vitiligo and Associated Comorbidities in

Korea. Yonsei Med J. 2015; 56:71925.

Carey RM. The changing clinical spectrum of adrenal insufficiency.

Ann Intern Med. 1997; 127:11035.

Odkers W. Adrenal insufficiency. N Engl J Med. 1996,


Eason RJ, Croxon MS, Perry MC, Somerfield SD.

Addison`s disease, adrenal autoantibodies and computerized

adrenal tomography. NZ Med J. 1982; 95:56973.

Laureti S, Vecchi L, Santeusiano F, Falorni A. Is the prevalence

of Addison's disease underestimated? J Clin Endocrinol

Metab. 1999; 84:1762.

Stewart PM, Krone NP. The adrenal cortex. Williams Textbook

of Endocrinology. Amsterdam: Elsevier; 2011.

Hagg E, Asplund K, Lithner F. Value of basal plasma cortisol

assays in the assessment of pituitaryadrenal


Clin Endocrinol. 1987; 26:2216.

Betterle C, Dal Pra C, Mantero F, Zanchetta R. Autoimmune

adrenal insufficiency and autoimmune polyendocrine

syndromes: autoantibodies, autoantigens, and their

applicability in diagnosis and disease prediction. Endocr

Rev. 2002; 23: 32764.

Addison T. On the constitutional and local effects of disease

of the suprarrenal capsules. In a collection of the

published writing of the late Thomas Addison, MD., physician

to Guy`s Hospital. London: New Sydenham Society;

Zelissen, PM, Bast EJ, Croughs RJ. Associated autoimmunity

in Addison’s disease. J Autoimmun. 1995; 8:12130.

Gürkan E, Çetinarslan B, Güzelmansur İ, Kocabaş B. Latent

polyglandular autoimmune syndrome type 2 diagnosed

during a shock manifestation. Gynecol Endocrinol.

; 32:521-3.

Kahaly GJ. Polyglandular autoimmune syndrome type II.

Presse Med. 2012; 41:e66370.

Ditmar M, Kahaly GJ. Polyglandular autoimmune syndromes:

immunogenetics and longterm

follow-up. J Clin

Endocrinol Metab. 2003; 88:298392.

Michels A, Gottlieb P. Autoimmune polyglandular syndromes.

Nat Rev Endocrinol. 2010; 6:2707.

Trence DL, Morley JE, Handwerger BS. Polyglandular autoimmune

syndromes. Am J Med. 1984; 77:10716.

McDermott MT. Endocrine Secrets. 6th ed. Philadelphia:

Saunders; 2013.

Bain A, Stewart M, Mwamure P, Nirmalaraj K. Addison's

disease in a patient with hypothyroidism: autoimmune

polyglandular syndrome type 2. BMJ Case Rep. 2015;

pii: bcr2015210506.

Kemp EH, Waterman EA, Hawes BE, O'Neill K, Gottumukkala

RV, Gawkrodger DJ,et al. The melaninconcentrating

hormone receptor1,

a novel target of autoantibody responses in vitiligo. J Clin Invest. 2002;


Taïeb A. Immune/Inflammatory Aspects. In: Picardo M,

Taïeb A, editors. Vitiligo. Berlin: SpringerVerlag;

p. 239-47.

Gill L, Zarbo A, Isedeh P, Jacobsen G, Lim HW, Hamzavi

I. Comorbid autoimmune diseases in patients with vitiligo:

A cross-sectional study. J Am Acad Dermatol. 2016;


Liu JB, Li M, Yang S, Gui JP, Wang HY, Du WH,et al. Clinical

profiles of vitiligo in China: an analysis of 3742

patients. Clin Exp Dermatol. 2005; 30:327-31.

Mollet I, van Geel N, Lambert J. Autoimmune/inflammatory

and other diseases associated with vitiligo. In: Picardo

M, Taïeb A, editors. Vitiligo. Berlin: SpringerVerlag;

p. 79-90.

Vrijman C, Kroon MW, Limpens J, Leeflang MM, Luiten

MR, van der Veen JP, et al.The prevalence of thyroid disease

in patients with vitiligo: a systematic review. Br J

Dermatol. 2012; 167:1224-35.

Sardana K, Sharma RC, Koranne RV, Mahajan S. An interesting

case of colocalization of segmental lichen planus

and vitiligo in a 14-year-old-boy. Int J Dermatol. 2002;


Amerio P, Tracanna M, De Remigis P, Betterle C, Vianale

L, Marra ME, et al. Vitiligo associated with other autoimmune

diseases: polyglandular autoimmune syndrome

types 3B+C and 4. Clin Exp Dermatol. 2006; 31:746-9.

Njoo MD, Das PK, Bos JD, Westerhof W. Association of

the Kobner phenomenon with disease activity and therapeutic

responsiveness in vitiligo vulgaris. Arch Dermatol.


Sahni K, Parsad D. Stability on vitiligo: is there a perfect

way to predict it? J Cutan Aesth Surg. 2013; 6:77-82.

Sosa JJ, Currimbhoy SD, Ukoha U, Sirignano S, O'Leary

R, Vandergriff T, t al. Confettilike

depigmentation: A potential

sign of rapidly progressing vitiligo. J Am Acad

Dermatol. 2015; 73:272-5.

Harning R, Cui J, Bystrin JC.

Relation between the incidence

and level of pigment cell antibodies and disease

activity in vitiligo. J Invest Dermatol. 1991; 97:1078-80.

How to Cite
Tavares Bello, R., Tavares Bello, C., Sequeira Duarte, J., & Vasconcelos, C. (2017). Unstable Vitiligo in the Setting of Diffuse Hypermelanosis: A Peculiar Presentation of Schmidt’s Syndrome. Journal of the Portuguese Society of Dermatology and Venereology, 75(1), 65-71. https://doi.org/10.29021/spdv.75.1.720
Case Reports