The 'butterfly' sign. A clue to generalized pruritus in a patient with chronic obstructive hepatobiliary disease

Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases?

“…Pruritus is not alleviated by scratching and eventually becomes more diffuse and intractable. 3 Other cutaneous manifestations include the butterfly sign (generalized hyperpigmentation with sparing of the interscapular and scapular areas of the back), 4 and stigmata of chronic liver disease, including xanthomas, palmar erythema, and spider angiomas. 5 Jaundice is less common and is usually a manifestation of late-stage disease.…”

Old scratch

“…Repeated skin damage can also lead to postinflammatory hyperpigmentation that is distinct from the green discoloration of the skin that has been attributed to bile pigment deposition at the sweat pores 25. The contrast that can occur in pigmentation between the areas of skin that are scratched and those that are not is well illustrated in the sign, first reported by Reynolds26 and confirmed by others,27 of a “butterfly” of normal pigmentation set on the dark background of skin that has been repeatedly abraded (Fig. 1, top).…”

British Association of Dermatologists’ guidelines for the investigation and management of generalized pruritus in adults without an underlying dermatosis, 2018

“…Pruritus is a common symptom in patients with various hepatobiliary disorders, including cholestasis of pregnancy . The skin in hepatic pruritus is often generally hyperpigmented and excoriated . The hands and feet are often the worst‐affected areas .…”