Granuloma faciale

European Journal of Dermatology. Volume 10, Number 4, 313-4, June 2000, Votre diagnostic !

Author(s) : Deniz DEMIRCIOGLU, Gül BUKULMEZ, Aytaç GOKOZ.

Summary : A 29 year-old male patient presented with a 2-month history of asymptomatic brown papules and plaques on the face, which had been gradually increasing in number. The patient's medical history was unremarkable for any other skin or systemic disease and he was not on any long-term medication. Examination of the skin revealed 8-10 well-circumscribed, dusky red-brown, indurated, 0.5-1.5 cm papules and plaques on the forehead and both cheeks (Fig. 1). Physical examination was otherwise normal. Histopathological examination of an excision biopsy specimen showed a dense mixed dermal infiltrate composed predominantly of neutrophils and eosinophils, also of plasma cells, lymphocytes and histiocytes. The subepidermal region and the adnexal structures remained uninvolved, thus the infiltrate was separated from the epidermis by a Grenz zone (Fig. 2). In the dermis some vessel walls were also infiltrated (Fig. 3). The epidermis was normal except for focal spongiosis and exocytosis.

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ARTICLE

Discussion

Granuloma faciale is an uncommon skin disorder presenting as reddish brown papules, nodules and plaques on the face. Lesions are usually asymptomatic although occasionally pruritus and tenderness have been reported. The disorder typically affects healthy middle-aged white men, but it may also occur in women and even in children [1]. The color of the lesions ranges from normal skin color to purple and surface changes are usually limited to follicular dilatation. Extrafacial lesions, with or without co-existing facial lesions, have been reported [2, 3].

The etiology and exact pathogenesis of the disorder remain unknown. Diagnosis is usually established through the histopathological findings that are quite distinctive. In spite of its name skin biopsies do not show any granuloma formation or an infiltrate rich in histiocytes. Distinctive histopathological features include a dense polymorphous dermal infiltrate consisting of eosinophils, neutrophils, plasma cells, lymphocytes and histiocytes. The infiltrate is separated from both the epidermis and the adnexal structures by the characteristic Grenz zone. There may be some degree of vasculitis involving the papillary and middermal capillaries. Perivascular deposition of immunglobulins (usually IgG) and complement has been reported on direct immunofluorescence examination. Based on these observations some authors consider granuloma faciale as a variant of chronic leukocytoclastic vasculitis confined to the skin [4, 5]. Immunophenotypic analysis in an attempt to define the nature of the lymphocytic infiltrate in granuloma faciale has shown the cells to be almost exclusively T-helper lymphocytes. Since they express interleukin-2 receptors, ICAM-1, lymphocyte functional antigen (LFA)-1 and HLA-DR, these lymphocytes have been suggested as producing gamma-interferon. Under the influence of gamma-interferon, ICAM-1 expression on keratinocytes, which causes migration of lymphocytes to the epidermis, is expected to occur. In granuloma faciale, however, it has been observed that there is a lack of expression of ICAM-1 by basal keratinocytes and this has been suggested as resulting in the formation of the Grenz zone [6].

The clinical differential diagnosis includes sarcoidosis, chronic cutaneous lupus erythematosus, polymorphous light eruption, pseudolymphoma, lymphoma/leukemia cutis, mycosis fungoides, insect bite reaction and fixed drug eruption.

The clinical course of granuloma faciale is usually chronic with exacerbations and remissions. Occasionally permanent spontaneous clearing may occur. Multiple treatment modalities have been employed for the disease, but none has been consistently successful. Drugs that have been proposed include dapsone [7], antimalarials, colchicine, topical, intralesional or systemic corticosteroids and clofazimine. Physical modalities such as surgical excision, dermabrasion [8], carbon dioxide [8] and argon laser, electrodesiccation [8], topical PUVA, cryotherapy and combination of cryotherapy and intralesional corticosteroid injection [9] have also been used with variable results.

Further studies are necessary to define the pathophysiology of granuloma faciale and to establish the effective mode of treatment.

Article accepted on 16/1/00

REFERENCES

1. Pedace FJ, Perry H. Granuloma faciale. A clinical and histopathologic review. Arch Dermatol 1966; 94: 387-95.

2. Sears JK, et al. Extrafacial granuloma faciale. Arch Dermatol 1991; 127: 742-3.

3. Castano E, et al. Granuloma faciale entirely in an extrafacial location. Br J Dermatol 1997; 136: 978-9.

4. Ackerman AB. Leukocytoclastic vasculitis. In: Ackerman AB, Chongchitnant N, Sanchez J, Guo Y, Bennin B, Reichel M, Rondall MB, eds. Histologic diagnosis of inflammatory skin diseases. Baltimore, Maryland: Williams & Wilkins, 1997: 489.

5. Smoller BR, et al. The natural history of vasculitis. What the histology tells us about pathogenesis. Arch Dermatol 1990; 126: 84-9.

6. Smoller BR, Bortz J. Immunophenotypic analysis suggests that granuloma faciale is gamma-interferon-mediated process. J Cutan Pathol 1993; 20: 442-6.

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8. Dinehart SM, et al. Granuloma faciale. Comparison of different treatment modalities. Arch Otolaryngol Head Neck Surg 1990; 116: 849-51.

9. Dowlati B, et al. Granuloma faciale: successful treatment of nine cases with a combination of cryotherapy and intralesional corticosteroid injection. Int J Dermatol 1997; 36: 548-51.