Graham-Little-Piccardi-Lasseur Syndrome

Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is a rare variant of lichen planopilaris—a follicular form of lichen planus. The condition was first described by Piccardi in 1913 and further characterized by Ernst Graham Little 2 years later in a mutual patient with Lasseur.  The 3 presentations of lichen planopilaris include GLPLS, frontal fibrosing alopecia, and classic lichen planopilaris. Classic lichen planopilaris is a chronic inflammatory process manifesting as patchy progressive cicatricial scalp alopecia. Frontal fibrosing alopecia presents with band-like cicatricial alopecia on the scalp's frontal and temporal zones. GLPLS is characterized by multifocal, patchy, cicatricial alopecia on the scalp, noncicatricial alopecia in the axillae and perineum, and follicular hyperkeratosis on the trunk and extremities. GLPLS symptoms may occur in any order, often starting with hyperkeratotic papules on the trunk and extremities before alopecia develops in any location (see  Lichen Planopilaris). GLPLS constitutes a small fraction of lichen planopilaris cases, predominantly affecting White women who are typically aged between 30 and 70. Correlations exist with various factors such as hepatitis B vaccination, androgen insensitivity syndrome, human leukocyte antigen (HLA)-DR-1 positivity (observed in a mother and daughter), vitamin A deficiency, hormonal dysfunction, and neuropsychological issues. Managing GLPLS focuses on immune response reduction to prevent follicular scarring, although reliable therapeutic regimens are still lacking. Research indicates that Janus kinase (JAK) inhibition with tofacitinib may normalize interferon (IFN)-mediated T-cell chemotaxis, potentially preventing infundibular inflammation. Despite theoretical advancements, healthcare professionals face challenges in achieving complete patient remission, underscoring the need for better preventive measures to mitigate long-term complications. Hair follicles consist of upper (permanent) and lower (transitory) segments. The upper segment comprises the infundibulum, extending from the epidermis to the sebaceous duct, and the isthmus, which houses stem cells at the bulge region. The lower segment is divided into the trunk (stem) and the bulb, with the trunk extending from the insertion of the arrector pili muscle to the cornified part of the bulb (see . The Common Integument, Section of the Skin). The bulb becomes completely keratinized beyond this region, known as Adamson's fringe A, stretching to the hair follicle's base. The follicular bulb houses the dermal papilla, providing blood supply and nutrients through a central capillary. The bulb also contains progenitor cells, dendritic melanocytes, and 3 internal hair sheath layers—Henle, Huxley, and cuticle—along with the external root sheath and vitreous membrane (basement membrane). The hair shaft has a cuticle, cortex, and medulla and becomes visible at higher levels of the follicle (see . Transverse Section of a Hair Follicle). The internal hair sheath merges into a single, fully keratinized stratum, while the external root sheath thickens with additional layers. Desquamation occurs in the upper trunk portions of the internal hair sheath, and keratinization takes place in the external root sheath. At the level of the infundibulum, the follicle undergoes keratinization similar to the epidermis, featuring a distinct granular layer and stratum corneum. Hair can be classified into 3 types—lanugo, vellus, and terminal. Lanugo hair is fine and covers the fetus during development, typically disappearing after birth. Vellus hair is less than 1 cm long with a diameter of less than 0.03 mm, lacking a medulla, and its bulb is located in the upper dermis. Terminal hair is longer than 1 cm, with a diameter greater than 0.06 mm. This hair type possesses a cortex and a medulla, and its bulb is located in the subcutaneous tissue. Terminal hair is found in androgen-dependent (eg, scalp, beard, chest, axillae, and pubic region) and androgen-independent areas (eg, eyebrows and eyelashes). Androgens do not influence vellus hair. The hair follicle's lower segment is involved in the hair growth cycle. About 85% to 100% of scalp hairs are typically in the anagen phase, which lasts 2 to 7 years. Around 1% of scalp hairs exist in the catagen phase—a period lasting 2 to 3 weeks. Matrix cell apoptosis and thinning of the lower segment occur during this period. In addition, the vitreous membrane and lower hair shaft thicken during this phase, surrounded solely by the external root sheath, creating a "club hair" appearance. Telogen is the resting phase, which involves 0% to 15% of scalp hairs, and about 100 days before the initiation of new hair growth and beginning of another cycle.