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SPECIAL REPORT Advances in dermatology and venereology ActaDV Acta Dermato-Venereologica ActaDV
Granuloma Faciale Treatment: A Systematic Review
Claudia LINDHAUS and Peter ELSNER Department of Dermatology and Allergology, University Hospital Jena, Jena, Germany
Granuloma faciale is an uncommon benign chronic dermatosis characterized by reddish-brown to violaceous asymptomatic plaques appearing predominantly on the face. The pathogenesis of granuloma faciale remains unclear, and it is frequently unresponsive to therapy. This systematic review aims to summarize all recent publications on the management of granuloma faciale. The publications are mainly individual case reports, small case series and a few retrospective studies. Treatment options included topical, intralesional and systemic corticosteroids, topical pimecrolimus and tacrolimus, topical and systemic dapsone, systemic hydroxychloroquine, clofazimine, and tumour necrosis factor-alpha inhibitors. More invasive therapies using lasers as well as cryosurgery and surgery were also reported. Topical glucocorticosteroids and tacrolimus remain treatments of first choice, possibly supplemented by topical dapsone.
Key words: tacrolimus; dapsone; review. Accepted Sep 7, 2017; Epub ahead of print Sep 7, 2017 Acta Derm Venereol 2018; 98: 14 – 18.
Corr: Claudia Lindhaus, Department of Dermatology and Allergology, University Hospital Jena, Erfurter Strasse 35, DE-07743 Jena, Germany. E-mail: Claudia. lindhaus @ med. uni-jena. de
Granuloma faciale( GF) is an uncommon inflammatory dermatosis with characteristic clinical and histological features. The term granuloma faciale was coined by Wigley in 1945, referring to the condition as an eosinophilic granuloma of the skin( 1). Clinically, GF presents as reddish-brown to violaceous plaques, often with follicular accentuation and superficial telangiectasias( 2). Plaques are situated almost solely on the face, but occasionally may appear on the trunk, extremities, or in the nasal cavity( extrafacial GF)( 3).
Diagnosis is confirmed by skin biopsy, which is often necessary to rule out other skin diseases with a similar appearance: rosacea, sarcoidosis, lupus vulgaris, fungal infection, mycobacteriosis, and discoid lupus erythematosus( 2).
Erythema elevatum diutinum( EED) is an important differential diagnosis for GF, especially in its extra-facial presentation. Both lesions are variants of leukocytoclastic vasculitis. The main differences between them are clinical; therefore diagnostic difficulties are increased in atypical locations. EED manifests with multiple lesions on the extensor surface of the joints, while GF manifests typically with isolated lesions, predominantly on the face( 4).
The histopathological diagnosis of GF may be challenging, as precise histopathological criteria have not been defined. Several features, such as the presence of many eosinophils in the infiltrate, are thought to be characteristic of GF. In a retrospective analysis of 66 patients and 73 skin specimens, Ortonne et al.( 3) demonstrated that the most frequent histopathological features of GF were the presence of a grenz zone, infiltration of neutrophils, and telangiectasia. However, some features usually considered to be of diagnostic value for GF were absent in a proportion of cases. In particular, there were cases with absent or diminished numbers of eosinophils.
Although vascular changes appeared to be frequent, concentric fibrosis around small blood vessels may be demonstrated( 4), but necrotizing vasculitis with vessel wall fibroid necrosis is rare, indicating that vessels may be involved in the pathogenesis of GF in a manner different from that seen in necrotizing vasculitis( 3). Occasionally, the presence of IgG, IgA, IgM, C3c and C1q deposits surrounding cutaneous skin vessels in GF suggests that activation of complement via the classical pathway may participate in the development of vasculitis( 5).
Acute and chronic features are often linked, which suggests that GF follows a chronic course with recurrent acute phases, rather than having distinct acute and chronic stages( 3).
The pathogenesis of GF remains unclear. It has been suggested that it is mediated by interferon( IFN)-γ produced by CD4 + T-helper cells. In GF lesions, immunohistochemistry reveals a predominance of CD4 + lymphocytes, responsible for producing IFN-γ, a mediator that acts to express molecules such as ICAM-1( intercellular adhesion molecule 1) on the surface of keratinocytes, promoting the chemotaxis of lymphocytes( 6). Interestingly, in GF lesions, basal keratinocytes do not express ICAM-1, restricting the migration of inflammatory cells into the epidermis, and forming the characteristic grenz zone( 6).
Further proposed, but yet unproven, factors that may contribute to the development of GF are hypersensitivity reactions, infection, trauma, actinic exposure, and radiation( 4).
Management of GF may be difficult, with multiple topical, systemic and mechanical treatment regimens proposed, and variable clinical responses.
The aim of this review is to summarize recent experience in management of GF, in order to help other physicians choose a suitable treatment. doi: 10.2340 / 00015555-2784 Acta Derm Venereol 2018; 98: 14 – 18
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica.