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A man with abdominal pain and skin pigmentation

  • Sylvia Aide Martínez-Cabriales
    Affiliations
    Department of Dermatology, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, Mexico
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  • Emmanuel I. González-Moreno
    Affiliations
    Department of Gastroenterology, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León , Monterrey, Mexico
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  • Jorge Ocampo-Candiani
    Correspondence
    Corresponding author at: Jorge Ocampo Candiani, Chairman of Dermatology Division, Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León. Av. Madero and Gonzalitos S/N, 64460 Mitras Centro Monterrey, México.
    Affiliations
    Department of Dermatology, University Hospital “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, Mexico
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Published:August 30, 2016DOI:https://doi.org/10.1016/j.ejim.2016.08.006

      1. Case description

      A 52-year-old man presented to the Emergency department because of 1-week history of nausea and abdominal pain. The patient referred generalized muscle pain, fatigue, hyporexia and 13 kg weight loss since 18 months ago. He also noticed a brown-colored pigmentation in his skin during the last year. Laboratory data showed an elevated white blood cell count (11,300/μL, 7900 neutrophils), hemoglobin 14.5 mg/dL, potassium 5.37 mmol/L, sodium 135.7 mmol/L, magnesium 2.71 mg/dL, phosphorus 4.7 mg/dL, calcium 9.4 mg/dL, glucose 70 mg/dL, BUN 21 mg/dL, VIH negative. On a physical examination blood pressure was 90/50 mm Hg, hyperpigmentation of the skin mostly in palmar creases, flexural areas, nipples, vermilion border of the lips and the buccal periodontal was noticed (Fig. 1).
      Fig. 1
      Fig. 1Hyperpigmentation of gums, lips and nipples (A and B). Hyperpigmentation on pressure surfaces and pigmented bands on nails (C-E).
      What is the diagnosis?

      2. Diagnosis

      Addison's disease
      In our patient we documented low serum cortisol level (11.7 ng/mL) and high adrenocorticotropic hormone (ACTH). Diagnosis was confirmed by stimulation with Cortrosyn test. Anti-adrenocortical antibodies against the steroidogenic enzyme, 21-hydroxylase were not detected.
      Addison's disease or primary adrenocortical insufficiency is a rare condition that causes deficiency in glucocorticoids, mineralocorticoids and androgens. It could be life-threatening if diagnosis is missed. Most cases are due to autoimmune destruction, followed by tuberculosis. It can be related to autoimmune polyendocrine syndromes type II or I. HIV, fungal infections, hemorrhage, drugs, infiltrative disorders and metastases could cause it. Onset of symptomatology, usually insidious and nonspecific, appears after >90% of the gland is destroyed, explaining why patients are diagnosed after several years. Symptoms include weakness, anorexia, weight loss, myalgia, arthralgia, hypotension, nausea, vomit, abdominal pain, constipation, diarrhea and generalized hyperpigmentation [
      • Torrejo´ N.S.
      • Webb S.M.
      • Rodrı'guez-Espinosa J.
      • et al.
      Long-lasting subclinical Addison's disease.
      ,
      • Bleicken B.
      • Hahner S.
      • Ventz M.
      • et al.
      Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients.
      ]. The latter resulted from stimulation of melanocortin receptors on keratinocytes by the ACTH produced by the corticotrophs. Hyperpigmentation rules out secondary adrenal insufficiency. Other symptoms are vitiligo, salt craving, amenorrhea, infertility, auricular calcification and depression. Patients also present with normal or low serum sodium, normal or high serum potassium, metabolic acidosis, hypoglycemia, low serum cortisol and elevated ACTH. Confirmation is achieved with cosyntropin stimulation test after suboptimal cortisol response at 30 and 60 min. Hormonal replacement must be started as soon as diagnosis is made.

      Conflict of interest

      The authors have no conflict of interest to declare.

      References

        • Torrejo´ N.S.
        • Webb S.M.
        • Rodrı'guez-Espinosa J.
        • et al.
        Long-lasting subclinical Addison's disease.
        Exp Clin Endocrinol Diabetes. 2007; 115: 530-532
        • Bleicken B.
        • Hahner S.
        • Ventz M.
        • et al.
        Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients.
        Am J Med Sci. 2010; 339: 525-531