Graham-Little-Piccardi-Lasseur Syndrome: A Case Report

February 2023 | Volume 22 | Issue 2 | 210 | Copyright © February 2023


Published online January 27, 2023

Arda Celen MSc, Kristen Lo Sicco MD, Nayoung Lee MD, Shane Meehan MD, Jason Weed MD

The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY

Abstract
Graham-Little Piccardi-Lasseur syndrome is a rare dermatosis that affects the hair follicles throughout the body and often presents with a progressive cicatricial alopecia of the scalp that is unresponsive to medical therapy. While treatment options are limited, prompt recognition through a careful physical exam aided by dermoscopy can facilitate early intervention. Here we present a patient with GLPLS, discuss pertinent morphologic and dermoscopic findings, and review the current literature.

J Drugs Dermatol. 2022;22(2):210-216. doi:10.36849/JDD.6926

Citation: Celen A, Lo Sicco K, Lee N, et al. Graham-Little-Piccardi-Lasseur Syndrome: A case report. J Drugs Dermatol. 2023;22(2):210-211.doi:10.36849/JDD.6926

INTRODUCTION

Graham-Little-Piccardi-Lasseur Syndrome (GLPLS) is a rare clinical subtype of lichen planopilaris (LPP) that manifests as a triad of scarring alopecia of the scalp, nonscarring alopecia of the axillary and the pubic skin, and widespread lichenoid follicular papules.1 GLPLS more commonly affects women (male-to-female ratio ≃ 1:4), with the classic patient being a middle-aged Caucasian woman.2 While GLPLS is considered to be a manifestation of an immunologic disorder, predisposing factors are unclear and may be multifactorial. However immunosuppressive therapy confers limited and variable benefit, underscoring a longstanding need for therapeutic advances. The typical clinical course features progressive, scarring alopecia that may cause significant psychological distress to the patient.

CASE

A 58-year-old woman presented to the outpatient dermatology clinic with a tender and severely pruritic scalp skin eruption that began a few weeks before presentation. She described initially noticing welt-like lesions on her neck and face, along with a burning sensation in her scalp accompanied by hair loss. A dermoscopic exam revealed numerous peripilar casts and erythema on the scalp and hyperkeratotic erythematous follicular papules throughout the lumbosacral area, abdomen, and thighs (Figures 1, 2). Perifollicular erythema was seen on the axillae. A scalp punch biopsy revealed a perifollicular lichenoid lymphocytic infiltrate with fibrosis and a decreased number of terminal anagen hair follicles, some of which lacked sebaceous gland lobules. An abdominal punch biopsy revealed a perifollicular lymphocytic infiltrate with interface changes, hypergranulosis, and follicular plugging (Figure 3). A colloidal iron stain failed to reveal increased mucin deposition, and a PAS-D stain showed no evidence of basement membrane thickening or fungal infection. Overall, the clinical and histopathological findings were consistent with a diagnosis of GLPLS. The patient was instructed to apply topical clobetasol propionate 0.05% solution and minoxidil 5% solution twice daily on the scalp and augmented betamethasone dipropionate cream 0.05% BID on the body.

At a follow-up visit one month later, the patient reported resolution of her scalp pain and improvement of involved skin on the trunk and extremities. Tacrolimus ointment 0.1% for the scalp and the body twice daily on weekends and oral doxycycline 20 mg BID twice daily were added to the medication regimen, and topical corticosteroid treatments were restricted