Gougerot–Blum syndrome

Overview

Gougerot–Blum syndrome, also known as pigmented purpuric lichenoid dermatitis, is a rare chronic skin disorder characterized by reddish-brown patches, purpura (small bleeding spots), and lichenoid (flat-topped) papules primarily affecting the lower limbs. First described by Henri Gougerot and Paul Blum in 1925, it is considered a variant of pigmented purpuric dermatoses (PPD), a group of benign inflammatory skin conditions involving capillary inflammation and leakage of red blood cells into the skin.

Causes

The exact cause of Gougerot–Blum syndrome remains unknown. It is believed to be related to inflammation of small blood vessels in the skin (capillaritis), resulting in leakage of blood and pigment deposition. Several contributing factors have been proposed, including:

• Immune-mediated inflammation

• Venous hypertension or stasis

• Allergic reactions to medications or foods

• Systemic diseases: Such as diabetes mellitus or liver disease

• Genetic predisposition: Though not well established

The condition is more common in middle-aged men but can affect individuals of any age or gender.

Symptoms

The hallmark features of Gougerot–Blum syndrome are primarily dermatological. Common symptoms include:

• Reddish-brown macules and lichenoid papules: Usually clustered and localized on the lower legs or ankles

• Purpura: Small, non-blanching red or purple spots due to bleeding under the skin

• Itching or mild irritation: Common but not always present

• Chronicity: Lesions persist and may gradually spread but do not cause scarring

The rash is typically bilateral and symmetrical, and systemic symptoms (like fever or fatigue) are absent, distinguishing it from other more serious vasculitic conditions.

Diagnosis

Diagnosis of Gougerot–Blum syndrome is primarily clinical but can be confirmed with histopathological examination:

• Clinical evaluation: Characteristic appearance and distribution of lesions

• Dermoscopy: May reveal petechiae, pigment granules, and vascular changes

• Skin biopsy: Shows perivascular lymphocytic infiltrate, hemosiderin deposition, and extravasated red blood cells with lichenoid interface changes

• Laboratory tests: Usually normal; performed to exclude systemic vasculitis or other causes of purpura

Treatment

There is no definitive cure for Gougerot–Blum syndrome, and treatment focuses on managing symptoms and preventing progression. Options include:

• Topical corticosteroids: Reduce inflammation and itchiness

• Emollients: Help soothe dry or irritated skin

• Compression stockings: Improve venous return and reduce capillary leakage

• Systemic therapies: Such as pentoxifylline, vitamin C, or bioflavonoids may be considered in persistent cases

• Avoidance of triggers: Including medications or prolonged standing

Patient education and reassurance are important, as the condition is chronic but benign.

Prognosis

The prognosis for Gougerot–Blum syndrome is generally good. It is a chronic but non-life-threatening skin disorder that does not lead to systemic involvement or serious complications. While the skin lesions may persist or recur over time, they typically do not scar or progress to more severe disease. Most individuals can manage the condition effectively with topical treatments and lifestyle adjustments.