Lichen planus pigmentosus-inversus

Published Date: 19th July 2016

Publication Authors: Ngan K, Sharma N

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Abstract

A 50-year-old Caucasian woman presented with a four-month history of asymptomatic, linear hyperpigmentation along the skin creases on the left side of her neck, right axilla and groins. No preceding trigger factor was reported and she had no relevant medical or drug history.

Clinical examination revealed multiple, well-demarcated hyperpigmented macules and patches distributed linearly along the intertriginous regions of her neck, groins and right axilla. There was no evidence of acute inflammation. The rest of her skin examination was unremarkable, with no mucosal or nail involvement.

Histological examinations of punch biopsies obtained from her neck and right groin both revealed prominent basal vacuolar change, colloid bodies and moderate lichenoid chronic inflammation with conspicuous pigment incontinence. A diagnosis of lichen planus pigmentosus-inversus (LPPI) was made. The patient is undergoing treatment with topical 0.1% tacrolimus ointment, and is due back for review in clinic to assess treatment response.

LPPI is a rare variant of lichen planus (LP) with less than 20 cases reported to date. It is characterized by brown or violaceous, smooth, macules and patches up to a few centimeters affecting the intertriginous regions. Larger lesions adopt a linear or annular configuration which follows body creases. They are mostly asymptomatic, but may be associated with mild pruritus. Skin lesions in keeping with classic LP are infrequently found, though the scalp, mucosal membranes and nails are typically spared. An association with Hepatitis C infection has been suggested, but almost all reported cases had no relevant medical or drug history. The exact cause of LPPI remains unknown, but it is postulated to be secondary to T-lymphocyte-mediated cytotoxic activity against basal keratinocytes.

Diagnosis is confirmed by skin biopsy and histological findings of lichenoid inflammation alternating with areas (in the superficial dermis) of regression, melanophagia and prominent pigmentary incontinence.

Spontaneous regression of LPPI after a few months have been observed, but the disease can remain active for many years. Success rates of treatment trials with topical calcineurin inhibitors, high potency corticosteroids and oral prednisolone have been limited. Reassurance of this benign condition may be all that is necessary, though if symptomatic or distressing to patients, topical agents such as calcineurin inhibitors may be considered as a first measure.

Wong, JLC; Sharma, N; Ngan, K. (2015).  Lichen Planus Pigmentosus-Inversus: A Rare Varient of Lichen Planus . Journal of the American Academy of Dermatology. 19 (5 Suppl 1), AB239

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