Graham-Little Syndrome

Graham-Little Syndrome (GLS): Description, Causes and Risk Factors:Abbreviation: GLS.GLSGLS is an unusual type of lichen planus called lichen planopilaris (LPP) that affects the hair follicles. This rare lichenoid dermatosis is characterized by scarring alopecia, the loss of pubic and axillary hairs, and the progressive development of spinous or accuminate follicular papules on the trunk and extremities. It is a very rare disease but the exact prevalence is not known. GLS predominantly affects women. The duration of the illness varies from 6 months to 10 years.The disease is considered a variant of Lichen Planus (LP) consisting of follicular LP of the body and/or scalp, and lichen planopilaris of the scalp. Comparable to LP, the disease is probably the result of a T-cell-mediated immune response of unknown etiology.In GLS, T-lymphocytes recognize an unknown antigen expressed on the surface of keratinocytes located in the bulge area of the follicle. The bulge-isthmus area serves as a reservoir for follicular stem cells. Keratinocyte antigen recognition initiates lymphocyte activation and the subsequent destruction of the follicular stem cells, preventing anagen hair follicle regeneration. This may explain the scarring nature of GLS.Risk Factors May Include:HLA-DR (human leukocyte antigen-DR) is one of several HLA subtypes associated with lichen planus (LP) and GLS.
  • Hepatitis B.
  • Androgen insensitivity syndrome.
  • Neuropsychological stress.
  • Vitamin A deficiency.
Symptoms:Signs & symptoms may include:Cicatricial alopecia of the scalp.
  • Noncicatricial alopecia of the axilla and groin.
  • Follicular lichen planus eruption of the body, scalp, or both.
  • Mild perifollicular erythema.
  • Follicular hyperkeratosis.
  • Loss of residual normal tufts and hair follicles.
Diagnosis:Differential diagnosis for GLS may include the other lymphocytic cicatricial alopecias, such as chronic cutaneous lupus erythematosus, pseudopelade of Brocq, central centrifugal cicatricial alopecia, alopecia mucinosa, and keratosis follicularis spinulosa decalvans (KFSD).The diagnosis requires a detailed clinical history coupled with histopathology, and oftentimes, direct immunofluorescence.The clinical findings of GLScan be identified with the aid of dermoscopy or videomicroscopy. These early lesions include perifollicular violaceous erythema, scaling and groups of keratotic follicular papules. The active lesions are often located in the periphery of the area of alopecia, in contrast to chronic cutaneous lupus erythematosus (CCLE), which has predominantly central activity. A positive pull test of anagen hairs, although subjective, cannot determine the exact diagnosis but it can confirm disease activity.Biopsy is recommended to confirm the diagnosis.A biopsy should be performed in the hair-bearing margin in the periphery of alopecia in an active, inflammatory lesion. Multiple biopsies are sometimes required to achieve a definitive diagnosis.Treatment:GLS is challenging disease to manage, as there are no curative therapies, and relapses are common. Early diagnosis and treatment is important to prevent inflammation and spread.GLS is treated topically or by systemic corticosteroids, retinoids, or PUVA therapy, each credited as having partial and temporary benefits. The only improvement noted was the gradual disappearance of lichenoid papules on the trunk and extremities. They resolved with only residual discoloration. This effect was especially prominent after the combination of prednisone therapy and PUVA.Further research and clinical studies are necessary for insight into the pathophysiology and effective treatment of GLS.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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