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Granuloma Faciale


BASICS


DESCRIPTION


Granuloma faciale (GF) is an uncommon benign and chronic inflammatory skin disease characterized by single/multiple facial cutaneous nodules.  

EPIDEMIOLOGY


Incidence
  • Rare; precise incidence and prevalence are unknown.
  • Primarily a disease of middle-aged white men with rare reports of granuloma faciale in Japanese, blacks, and children.

ETIOLOGY AND PATHOPHYSIOLOGY


Some cases are idiopathic. Production of interleukin-5 by a clonal T-cell population may lead to attraction of eosinophils to the affected area (1). A γ-interferon-mediated process has also been proposed (2). Sun exposure may play a role, as lesions are more common in sun exposed areas and sunlight may darken lesions.  

DIAGNOSIS


HISTORY


Granuloma faciale is typically asymptomatic. Tenderness and itching of the lesions are rarely described.  

PHYSICAL EXAM


Lesion characteristics:  
  • Location:
    • Most commonly located on the forehead
    • Cheeks, nose, eyelid, ear, scalp, or temple may also be involved.
    • Extra-facial lesions occur in up to 20% of cases, typically in areas of sun exposure.
  • Description:
    • Smooth, well-circumscribed, elevated dull red/brown/blue/purple papules, plaques, or nodules
  • Size:
    • Lesions can range in size from millimeters to centimeters.
  • Additional findings:
    • Lesions may contain telangiectasias.
    • Lesions darken with sun exposure.
    • Lesions may have a smooth surface with prominent follicular orifices, creating a peau d'orange effect.

DIFFERENTIAL DIAGNOSIS


  • Fixed drug eruption
  • Sarcoidosis
  • Polymorphous light eruption
  • B-cell lymphoma, cutaneous pseudolymphoma, cutaneous T-cell lymphoma
  • Granuloma annulare
  • Jessner lymphocytic infiltration of the skin
  • Granulomatous rosacea
  • Discoid lupus erythematous
  • Nodular cutaneous localized amyloidosis
  • Pseudolymphoma/lymphocytoma cutis
  • Leprosy
  • Foreign body granuloma
  • Erythema elevatum diutinum
  • Mastocytoma
  • Basal cell carcinoma
  • Tinea faciei

DIAGNOSTIC TESTS & INTERPRETATION


Diagnostic Procedures/Other
  • Granuloma faciale is a clinical diagnosis.
  • Dermoscopy is used to enhance the clinical diagnosis.
  • Biopsy is used for confirmation.

Test Interpretation
  • The histology of granuloma faciale is diagnostic.
  • A grenz zone of uninvolved tissue is located just below the epidermis created by sparing of the upper papillary dermis.
  • Beneath the grenz zone is a polymorphous inflammatory infiltrate consisting of lymphocytes, plasma cells, eosinophils, and neutrophils. This is located in the papillary and mid dermis. Granulomas are not present and the epidermis is not involved.
  • Vasculitic changes include perivascular inflammation with nuclear wall dust and vessel damage.
  • Direct immunofluorescence demonstrates deposition of IgG and fibrin, IgM, C3, and C1q around vessels and at the basement membrane.
  • Electron microscopy reveals excessive perivascular eosinophils, Charcot-Leyden crystals, and degranulation.
  • Dermoscopy reveals pink background with some areas blackened, white striations in different directions, and prominent follicular orifices (3)[C].

TREATMENT


MEDICATION


  • No double-blinded randomized control trials exist for standardized treatment; data is mostly based on case reports and small case series.
  • Treatment is not necessary and is often for cosmetic reasons.

First Line
  • If medical therapy is chosen, intralesional triamcinolone 2.5 to 5 mg/mL; may be repeated every 4 to 6 weeks (4)[C]
  • Topical tacrolimus 0.1% twice daily (5)[C]

Second Line
  • Dapsone 50 to 150 mg/day
  • Clofazimine 300 mg/day
  • Colchicine
  • Hydroxychloroquine
  • Isoniazid

SURGERY/OTHER PROCEDURES


  • Pulsed dye laser is recommended when topical and/or systemic therapies have failed or are contraindicated (6)[A].
    • 585- or 595-nm pulse dye laser (6)[A]
  • CO2 laser
  • Argon laser
  • Excision
  • Cryosurgery
  • Electrosurgery
  • Dermabrasion
  • Topical psoralen with UVA radiation

ONGOING CARE


PROGNOSIS


  • Lesions do not spontaneously resolve.
  • Granuloma faciale has the propensity to relapse after treatment.

REFERENCES


11 Gauger  A, Ronet  C, Schnopp  C, et al. High local interleukin 5 production in granuloma faciale (eosinophilicum): role of clonally expanded skin-specific CD4+ cells. Br J Dermatol.  2005;153(2):454-457.22 Smoller  BR, Bortz  J. Immunophenotypic analysis suggests that granuloma faciale is a gamma-interferon-mediated process. J Cutan Pathol.  1993;20(5):442-446.33 Teixeira  DA, Estrozi  B, Ianhez  M. Granuloma faciale: a rare disease from a dermoscopy perspective. An Bras Dermatol.  2013;88(6)(Suppl 1):97-100.44 Radin  DA, Mehregan  DR. Granuloma faciale: distribution of the lesions and review of the literature. Cutis.  2003;72(3):213-219.55 Caldarola  G, Zalaudek  I, Argenziano  G, et al. Granuloma faciale: a case report on long-term treatment with topical tacrolimus and dermoscopic aspects. Dermatol Ther.  2011;24(5):508-511.66 Erceg  A, de Jong  EM, van de Kerkhof  PC, et al. The efficacy of pulsed dye laser treatment for inflammatory skin diseases: a systematic review. J Am Acad Dermatol.  2013;69(4):609-615.e8. doi:10.1016/j.jaad.2013.03.029.

ADDITIONAL READING


  • Barnadas  MA, Curell  R, Alomar  A. Direct immunofluorescence in granuloma faciale: a case report and review of the literature. J Cutan Pathol.  2006;33(7):508-511.
  • Dourmishev  L, Ouzounova-Raykova  V, Broshtilova  V, et al. Granuloma faciale effectively treated with topical pimecrolimus. Acta Dermatovenerol Croat.  2014;22(4):305-307.
  • Dowlati  B, Firooz  A, Dowlati  Y. Granuloma faciale: successful treatment of nine cases with a combination of cryotherapy and intralesional corticosteroid injection. Int J Dermatol.  1997;36(7):548-551.
  • Lallas  A, Argenziano  G, Apalla  Z, et al. Dermoscopic patterns of common facial inflammatory skin diseases. J Eur Acad Dermatol Venereol.  2014;28(5):609-614.
  • Marcoval  J, Moreno  A, Bordas  X, et al. Granuloma faciale: treatment with topical tacrolimus. J Am Acad Dermatol.  2006;55(5)(Suppl):S110-S111.
  • Ortonne  N, Wechsler  J, Bagot  M, et al. Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol.  2005;53(6):1002-1009.
  • Roustan  G, S ¡nchez Yus  E, Salas  C, et al. Granuloma faciale with extrafacial lesions. Dermatology (Basel).  1999;198(1):79-82.
  • Thiyanaratnam  J, Doherty  SD, Krishnan  B, et al. Granuloma faciale: case report and review. Dermatol Online J.  2009;15(12):3.
  • Tomson  N, Sterling  JC, Salvary  I. Granuloma faciale treated successfully with topical tacrolimus. Clin Exp Dermatol.  2009;34(3):424-425.

CODES


ICD10


L92.2 Granuloma faciale [eosinophilic granuloma of skin]  

ICD9


701.8 Other specified hypertrophic and atrophic conditions of skin  

SNOMED


Granuloma faciale (disorder)  

CLINICAL PEARLS


  • Granuloma faciale is primarily a disease of middle-aged white men.
  • Sun-exposed areas are more commonly affected and sunlight may darken lesions.
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