Graham-Little syndrome

Overview

Graham-Little syndrome, also known as Graham-Little-Piccardi-Lassueur syndrome, is a rare form of lichen planopilaris (a variant of lichen planus) that primarily affects the hair and skin. It is characterized by a triad of clinical features: progressive scarring alopecia of the scalp, non-scarring hair loss in the axillary and pubic regions, and widespread follicular keratotic papules on the trunk and limbs. The condition predominantly affects middle-aged women but can also occur in men. It is chronic and can lead to permanent hair loss if not managed early.

Causes

The exact cause of Graham-Little syndrome is unknown, but it is believed to be an autoimmune disorder. The immune system mistakenly attacks hair follicles and skin cells, leading to inflammation and subsequent damage. Factors that may contribute to the onset include:

• Autoimmune predisposition: Similar to other forms of lichen planus

• Genetic susceptibility: Though familial cases are extremely rare

• Triggers: Such as infections, stress, or certain medications

Histologically, the condition resembles lichen planus with perifollicular inflammation and destruction of hair follicles.

Symptoms

Graham-Little syndrome is defined by a characteristic triad of symptoms:

• Scarring alopecia of the scalp: Progressive, patchy hair loss with irreversible destruction of hair follicles

• Non-scarring alopecia in the axillary and pubic regions: Smooth, hairless skin without signs of inflammation

• Follicular keratotic papules: Small, rough, spiny papules primarily on the trunk and extremities, resembling keratosis pilaris

Other possible symptoms may include itching, burning, or tenderness in affected areas. Oral mucosal involvement is rare but may occur in some cases as part of generalized lichen planus.

Diagnosis

Diagnosis of Graham-Little syndrome is primarily clinical, supported by histological examination of skin or scalp biopsies. Diagnostic steps include:

• Clinical evaluation: Identification of the classical triad of symptoms

• Dermoscopy: Reveals perifollicular erythema, scaling, and follicular dropout

• Scalp or skin biopsy: Shows interface dermatitis, lichenoid inflammation, and destruction of hair follicles typical of lichen planopilaris

• Laboratory tests: May be used to exclude other causes of alopecia or systemic autoimmune disorders

It is important to differentiate Graham-Little syndrome from other scarring alopecias, such as discoid lupus erythematosus or folliculitis decalvans.

Treatment

Treatment of Graham-Little syndrome aims to control inflammation, relieve symptoms, and prevent progression of scarring alopecia. Common treatment strategies include:

• Topical corticosteroids: High-potency steroids to reduce local inflammation

• Intralesional corticosteroid injections: For localized scalp lesions

• Oral corticosteroids: For severe or rapidly progressing disease

• Immunosuppressive agents: Such as hydroxychloroquine, methotrexate, or cyclosporine for long-term control

• Retinoids: Oral acitretin or isotretinoin may be helpful in controlling follicular hyperkeratosis

• Antimalarials: Such as hydroxychloroquine, used for their immunomodulatory effects

Treatment response varies, and long-term follow-up is required to monitor disease progression and treatment side effects.

Prognosis

The prognosis of Graham-Little syndrome is variable. While the condition is not life-threatening, it is chronic and can lead to irreversible scarring alopecia if not treated promptly. Early diagnosis and aggressive management can help slow or halt progression, though complete regrowth of hair in scarred areas is not possible. The follicular papules may persist or recur, and relapses are common. With consistent treatment and dermatologic care, patients can manage symptoms and maintain quality of life.