Granuloma Faciale | Granuloma eosinofílico

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Benign, chronic, inflammatory skin disease
 
Clinical presentation
  • Usually presenting as isolated, well-defined reddish-brown (ferruginosas) to violaceous asymptomatic papules, nodules or plaques showing follicular accentuation and telangiectasia
    • Most commonly present on the forehead, nose or cheeks
  • Tipicamente ausência de sintomas sistémicos
 
Epidemiology
  • Slight predilection for middle-aged white males (mean age of 50 years)
 
Etiology
  • Unknown
    • Alguns autores incluem no espectro de doenças relacionadas com IgG4
    • Activação de IL-5, infiltrados eosinofílicos → dermatose eosinofílica
    • Related to actininc damage (localization mainly on sun-exposed areas)
    • Other possible etiologies: allergy, trauma, radiation therapy
    • Considered to be a variant of chronic cutaneous vasculitis possibly secondary to an underlying localized Arthus phenomenon (localized type III hypersensitivity reaction)
 
Dermoscopy
  • Linear, arborizing vessels
  • Dilated follicular openings
  • Brown dots/globules → hemosiderin deposition
 
Histopathology
  • Mixed inflammatory infiltrates in the dermis of neutrophils, lymphocytes, plasma cells (que expressam IgG4), eosinophils
    • Lesoes iniciais: mais neutrófilos, vasculite
    • Lesoes mais tardia: mais eosinófilos e plasmócitos e fibrose lamelar
  • Usually separated from the overlying epidermis by a narrow Grenz zone
  • Dilated follicular ostia and/or follicular plugs
  • Vascular changes are frequent (perivascular infiltrates → leukocytoclasis, presence of hemosiderin and red blood cell deposits)
  • Direct immunofluorescence is not positive in all cases and not pathognomonic
    • Positive finings: 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
 
Treatment
  • Difficult and often unrewarding
  • First-line therapy: topical treatment
    • Topical corticosteroids
    • Intralesional corticosteroids, every 4 weeks (can be combined with cryotherapy)
    • Tacrolimus 0,1% 2id → may require up to 2-6 months
    • Dapsone 5% gel (anti-neutrófilo e anti-eosinófilo)
    • Intralesional rituximab was tried in three cases at a dose of 10mg/mL once monthly, with relatively good results
  • Systemic treatment
    • Systemic corticosteroids have been reported to be effective but with only partial improvement in most cases
    • Dapsone at dose 50*150mg/day has demonstrated effectiveness (anti-inflammatory, antimicrobial and antiprotozoal effects)
    • Clofazimine is an anti-leprosy treatment with anti-inflammatory and anti-proliferative effects for lymphocytes and carcinoma cells
      • Dose: 300mg/day with good effect after 3-5 months of treatment
  • AVOID: cryosurgery, electrosurgery, surgical excision, dermabrasion and CO2 or PDL laser → risk of scarring
  • Surgical and other therapies in last resort
    • Cryotherapy with liquid nitrogen as single treatment
    • Pulsed dye laser (PDL) → good cosmetic outcomes, especially for superficial lesions. The time interval between laser treatments is about 2-4 months
    • Potassium-titanyl-phosphate (KTP) - 532nm has been reported to be effective with good results reported after 5-10 days of daily treatment, without significant scarring
    • Carbon-dioxide (CO2) laser
    • Argon laser at 480 to 520 nm
    • Phototherapy including topical psoralen-ultraviolet A (PUVA)
    • Surgical excision with or without grafting
    • Dermabrasion