A Rare Case of Graham-Little-Piccardi-Lassueur Syndrome

Introduction Graham Little-Piccardi-Lassueur syndrome (GLPLS) is an extremely rare clinical subtype of lichen planopilaris (LPP), defined by a triad of cicatricial scalp alopecia, non-scarring hair loss in the axillae and pubic area and lichenoid papules, particularly on the torso and limbs [1]. Case Presentation A 77-year-old female presented with a five-month history of the body rash and patches of scalp alopecia. Her family history was negative for skin diseases. A first clinical examination revealed scarring alopecia with inflammatory erythema and scabs on the scalp, also non-scarring alopecia of the axillae and neck. Diffusely spreading scaly and itchy papules on the torso were observed. A biopsy performed from the scalp and the back confirmed LPP. The histopathological findings, anamnesis and the triad of clinical symptoms allowed to make the diagnosis of GLPLS. The prescribed treatment topical glucocorticoids and systemic hydroxychloroquine had a positive effect: the rashes on the torso and scalp completely disappeared and the hair started to grow back slightly. Discussion GLPLS primarily affects older patients, especially postmenopausal women, and it is associated with hormonal imbalances, such as androgen insensitivity and hypothyroidism, dyslipidemia, depression and hypertension [2–5]. The pathogenesis of GLPLS appears to involve T cell-mediated immunity, but the specific trigger for the inflammatory response remains unknown. Immunological factors, such as the presence of HLA-DR may play a role, as evidenced by familial cases [4]. GLPLS has to be differentiated from classical LPP and frontal fibrosing alopecia [6]. The classic treatment includes systemic and topical corticosteroids, though retinoids, phototherapy, tacrolimus, hydroxychloroquine and cyclosporine may also be used but with temporary effects [7]. Conclusions Early biopsy is essential to differentiate GLPLS from other skin conditions ensuring accurate diagnosis and preventing disease progression. This case highlights that the right treatment can help control the symptoms, regardless of the chronicity of the disease.