CLINICAL MANIFESTATIONS OF DISORDERS OF ADRENOCORTICOTROPIN EXCESS

Part of “CHAPTER 14 – ADRENOCORTICOTROPIN: PHYSIOLOGY AND CLINICAL ASPECTS“

Hyperpigmentation is common when ACTH levels exceed 300 pg/mL (Fig. 14-4). The highest ACTH levels are seen in Addison disease, Nelson syndrome, and ectopic ACTH secretion. Clinically, the hyperpigmentation is found particularly in areas of increased pressure (elbows, knuckles, knees) and is accentuated in areas of normal pigmentation (areolae, genitalia, palmar creases). Surfaces (such as the mucosal) that are not normally pigmented may exhibit hyperpigmentation. Scars acquired after the onset of ACTH excess also may exhibit hyperpigmentation (see Chap. 76).

FIGURE 14-4. Pigmented lunulae in a patient with the paraneoplastic adrenocorticotropic hormone (ACTH) syndrome who developed severe hyperpigmentation of rapid onset 3 months previously. More commonly, pigmentation of the nails related to excess ACTH is longitudinal rather than transverse, as in this patient. (For other examples of hyperpigmentation related to excess ACTH, see Chap. 76 and Chap. 219.)

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