Granuloma faciale (GF) is a rare, benign, inflammatory skin disease, usually presenting as isolated, well-defined reddish-brown to violaceous asymptomatic papules, nodules or plaques showing follicular accentuation and telangiectasia. It was first described as 'eosinophilic granuloma' in 1945 by Wigley JE. Granuloma faciale is most commonly seen in middle-aged white males (between the second and seventh decades of life), but it has been reported in childhood too. It usually appears as a single lesion on the face, but occurrence can be as multiple lesions and/or extrafacial. Extrafacial GF localizations are possible, including localization on the scalp, trunk, nasal cavity or extremities. The facial lesions most commonly present on the forehead, nose, or cheeks.
The etiology of granuloma faciale is unknown, but because of the localization on mainly sun-exposed areas of the body, the thinking is that the condition is related to actinic damage. Other possible etiologies include allergy and trauma. Radiation therapy is considered to be another potential trigger.
The pathogenesis of GF is not well established. It is considered to be a variant of chronic cutaneous vasculitis possibly secondary to an underlying localized Arthus phenomenon.
Specific histopathologic features have do not yet exist for granuloma faciale. The most frequent histologic features reported are the presence of Grenz zone, infiltration of neutrophils and telangiectasia.
The inflammatory infiltrates in the dermis are usually separated from the overlying epidermis by a narrow Grenz zone of the uninvolved dermis. Dilated follicular ostia and/or follicular plugs are frequently observed.
Vascular changes are frequent presentations, and they consist mainly of perivascular infiltrates which could penetrate the vascular wall and causing leukocytoclasis. However necrotizing vasculitis is very rare. Moreover, the presence of hemosiderin and red blood cell deposits are consistent with vascular injury.
Direct immunofluorescence is not positive in all cases and not pathognomonic for granuloma faciale. Positive findings seen on direct immunofluorescence include granular deposits of IgG with less intense deposits of IgM, and in some cases, IgA, C3, and C1q localized in the perivascular areas or the basement membrane zone.
Granuloma faciale typically presents as a chronic, progressive, asymptomatic, isolated, well-defined reddish-brown to a violaceous asymptomatic papule, nodule or plaques showing follicular accentuation and telangiectasia. The most common sites are the face and other sun-exposed areas. Dermoscopy is a useful tool for the clinical evaluation of GF. Dermoscopy typically reflects the clinical and histological features. The most common dermoscopic features include: linear, arborizing vessels, dilated follicular openings and brown dots/ globules. The brown dots/ globules are considered to reflect hemosiderin deposition.
Granuloma faciale has a progressive, chronic course with recurrent acute phases, rather than having a distinct acute and chronic phase. It is therefore difficult to comment on the chronicity based on histopathology as cases might show overlapping features of acute and chronic inflammation. Laboratory evaluation is normal except for mild blood eosinophilia. Granuloma faciale is a benign condition, but spontaneous healing is rare, so most cases require therapeutic intervention, but presently available treatment options associated with significant chances of recurrence.
The treatment of granuloma faciale include is difficult, with variable clinical outcomes. Treatment modalities reported to be useful in granuloma faciale include:
Several cutaneous conditions present with an appearance similar to granuloma faciale include. Clinical differential diagnosis includes sarcoidosis, discoid lupus erythematosus, rosacea, mycobacterial infections cutaneous deep fungal infections, cutaneous lymphoma, and basal cell carcinoma.
Erythema elevatum diutinum (EED) is a histopathological differential diagnosis for granuloma faciale include and can show similar histological features like fibrosing vasculitis. It is possible that both EED and GF have a common underlying pathogenic mechanism. The main difference between these two entities is clinical, as EED usually presents as multiple lesions on the extensor surfaces of the joints, and GF usually a single facial lesion. The diagnosis is more challenging when EED presents on the face, or in the case of extra facial localization with GF. However, a high number of eosinophils strongly favors the diagnosis of GF. On the other hand, the granulomatous nodules in the histopathologic examination are present in some cases of EED. Additionally, EED commonly correlates with some underlying disease, mainly hematological abnormalities, autoimmune conditions, HIV infections, other infectious diseases, and insect bites. However, granuloma faciale include is rarely associated with other systemic diseases.
Topical treatments are considered to be first-line therapy in granuloma faciale include:
Surgical and other therapies:
Primary care providers and nurse practitioners who see facial lesions should refer these patients to the dermatologist for definitive workup. It is essential to evaluate granuloma faciale along with the dermatopathologist to ensure the accuracy of diagnosis because of the large number of clinical and histological differentials. an interprofessional team approach that includes physicians, nurse practitioners, PAs, and pharmacists gives the best opportunity for successful case management and patient care.
Surgical treatment, especially for facial lesions, needs to be planned in collaboration with a plastic surgeon.
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