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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 .