Hyperpigmentation mimicking Laugier syndrome, levodopa therapy and Addison's disease

Abstract

The Laugier-Hunziker syndrome is an acquired, idiopathic, benign mucocutaneous hypermelanosis that usually occurs on the lips and oral mucosa, although it may appear at other sites. Nails are frequently involved, mainly forming longitudinal hyperpigmented bands. We report the case of a patient that presented a typical picture of this entity, nearly 1 year after the beginning of treatment with levodopa. Two years after the first lesions occurred, she developed Addison's disease. The patient suffered from a diffuse discrete hyperpigmentation (it was more remarkable on exposed areas) and an intensification of the melanotic macules that were previously noticeable before in oral and genital mucosa, fingers, toes and nails. Hormonal replacement treatment enabled the control of laboratory and general manifestations and to decrease the degree of mucocutaneous hyperpigmentation considerably, despite initial hyperpigmented lesions persisting in described areas.

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“…2 Mild acanthosis has been described in some reports. 2 Therefore, it is a functional change in the melanocyte of an as yet unknown cause; however, it is probably a consequence of chronic stimulation due to enzymatic hyperreactivity in the biosynthesis of melanin-tyrosin, which subsequently results in an increase in the number of melanosomes. 2 Various conditions should be included in the differential diagnosis of mucocutaneous pigmentary disorders such as the following syndromes: McCune-Albright, LEOPARD, Addison, LAMB, Gardner, Cronkhite-Canada, neurofibromatosis and Peutz-Jeghers, as well as racial pigmentation, which is a benign disorder and the principal cause of longitudinal melanonychia.…”

“…1 This original concept of this acquired pigmentary disorder was later modified to idiopathic lenticular mucocutaneous pigmentation after the condition was detected in other areas where a similar histology was found. 2,3 The etiopathogenesis of this condition is unknown, although different theories have been proposed to explain the mechanism behind the melanocytic dysfunction. 2,3 It is most prevalent in women and in general begins between the ages of 30 and 50 years.…”

“…2,3 The etiopathogenesis of this condition is unknown, although different theories have been proposed to explain the mechanism behind the melanocytic dysfunction. 2,3 It is most prevalent in women and in general begins between the ages of 30 and 50 years. It occurs almost exclusively in whites, unlike the patient in the current report, who is brown.…”

“…1 The pathology of this condition consists of hyperpigmentation of the basal layer, an increase in melanophages and the presence of pigmentary incontinence in the papillary dermis, with no hyperproliferation or melanocytic atypia. 2,6,7 The disorder is more evident in the areas most affected. 2 Mild acanthosis has been described in some reports.…”

“…2,6,7 The disorder is more evident in the areas most affected. 2 Mild acanthosis has been described in some reports. 2 Therefore, it is a functional change in the melanocyte of an as yet unknown cause; however, it is probably a consequence of chronic stimulation due to enzymatic hyperreactivity in the biosynthesis of melanin-tyrosin, which subsequently results in an increase in the number of melanosomes.…”