Granuloma Faciale - a rare and idiopathic skin disorder characterized by the development of asymptomatic, well-defined red plaques with a smooth surface on the face. ICD-10 Code: L92.2.
This disease primarily affects men between the ages of 20 and 89 (average age 53), but can also occur in children and women. People of European descent are more likely to be affected.
The etiology and pathogenesis are not fully understood. Research suggests a role for interferon-gamma and elevated levels of IL-5 in the development of the disease. Some believe that the disease is a chronic IgG4-related cutaneous vasculitis because immunologic studies often show an elevated ratio of IgG4 to IgG-containing circulating plasma cells and an increase in IgG4-positive plasma cells in the affected skin lesions.
Because of its clinical and histologic similarity to erythema elevatum diutinum, some authors consider granuloma faciale to be a clinical variant of vasculitis, but localized to the face.This condition is characterized by the appearance of painless, well-defined, hemispherical, solitary papules or infiltrated plaques ranging in size from a few millimeters to several centimeters, red in color, with a smooth surface. Dilated hair follicle openings (the "orange peel" or "peau d'orange" sign) and telangiectasias are visible on the surface. On diascopy, the elements become pale, but no specific inclusions are seen. Ulceration is never observed, and with intense sun exposure, the lesions may become edematous, more erythematous, and acquire a dark (pigmented) hue.
In about one third of patients, more than one lesion is found, but the total number of elements does not exceed 5-6. In a large series of cases, it was found that the rash was most commonly localized on the forehead (37%), cheeks (30%), nose (28%), and eyelids (11%). Extracutaneous involvement is seen in 7-10% of cases. Granuloma faciale has been described on the earlobes, scalp, shoulders, trunk, extremities, dorsum of the hands, feet, and vulva. Rare intranasal localization has also been reported.
The disease is chronic in nature and the rash elements may persist for years without visible change. Spontaneous resolution is rare. Granuloma faciale is not associated with systemic disease and never becomes malignant.- B-cell lymphoma
- Pseudolymphoma (cutaneous lymphocytoma)
- Persistent reaction to arthropod bites
- Lupus erythematosus tumidus
- Polymorphous light eruption
- Erythema elevatum diutinum
- Fixed drug eruption
- Sarcoidosis
- Granuloma annulare
- Leprosy
- Foreign body granuloma
- Granulomatous rosacea
- Jessner-Kanof lymphocytic infiltration
- Kimura's disease
- Blue rubber bleb nevus syndrome
- Juvenile xanthogranuloma
- Mastocytoma
- Insect bites
- Spitz nevus
The disease is resistant to treatment. First-line treatment is intralesional injection of triamcinolone (2.5-5 mg/ml). Less effective treatments include topical calciumneurin inhibitors (tacrolimus) and corticosteroids under occlusive dressings.
Systemic therapy options include:
- Oral dapsone at a dose of 20-150 mg once daily.
- Oral clofazimine at a dose of 300 mg once daily.
- Oral hydroxychloroquine at a dose of 200-400 mg daily.
- Oral colchicine at a dose of 0.5-1.5 mg daily or every other day.