Lupus Miliaris Disseminatus Faciei (LMDF)

đź’ˇ
Uncommon, chronic, inflammatory dermatosis
AKA lupus miliaris disseminatus faciei, facial idiopathic granulomas with regressive evolution (FIGURE)
 
Etimologia e histĂłria
  • Referred to as “rosacea-like tuberculid” by Lewandowsky → clinical appearance similar to that of a papular form of rosacea + yeallow-brown “apple-jelly” nodules on diascopy and tuberculoid granulomas on histologic examination
    • Hence, earlier discribed as lupus miliaris disseminatus faciei
  • Later it was noted that granulomas can be seen in typical rosacea → rosacea may be manifested by a clinical and histologic spectrum that includes granuloma formation in some patients
 
Apresentação clínica
  • More monomorphous and persistent
  • Skin-color to dull red-brown facial papules, dome-shaped
  • Favor the central face
  • Classic rosacea signs may be absernt
  • Characterized by red-to-yellow or yellow-brown papules of the central face, particularly on and around the eyelids
  • Lesions may occur singly or in crops
 
Epidemiology
  • Variant in both children and adults that may resolve spontaneously
  • Predominantly affects middle-aged women
  • More common in Asians (japanese)
 
Etiologia
  • Unknown etiology & pathogenesis
  • Once considered a tuberculid because of the histology
  • Many authors consider it an extreme variant of granulomatous rosacea
  • Others believe it is a distinct (characteristic histopathology and occasional involvement of noncentral facial areas)
  • Possible link with Demodex mites
  • Molecular studies indicate a role for metalloproteinases, UV radiation and AMPs
  • Immunologic factors: chronic inflammatory reaction induced by various triggers (UV light, heat, stress, etc)
  • Immune dysregulation: possible abnormalities in immune system function, leading to an exaggerated inflammatory response
 
Prognosis
  • Active disease usually involves a 1- to 3-year course and may resolve spontaneously
  • Risk of significant disfiguring scarring if left untreated
 
Histopathology
  • Non-caseating epithelioid granulomas
  • Perifollicular or associated with sebaceous glands within the dermis
  • Mixed inflammatory infiltrate surrounding the granulomas, consisting of lymphocytes, histiocytes and occasionally plasma cells
  • Diagnosis is dependent upon the histopathologic finings
  • Superficial perivascular and periappendageal lymphocytic infiltrates with a few histiocytes and neutrophils
  • Fully developed lesions show round granulomas, often with caseation necrosis
  • Mixtures of sacoidal and tuberculoid granulomas
  • Late lesions show fibrosis with scattered lymphocytes, histiocytes and neutrophils and may show epidermal thinning
 
Treatment
  • Evidence is limited to case series and retrospective reviews
  • Treatment should be considered due to scarring even though the disease course is usually self-limited
  • There is no standard treatment
  • Oral antibiotics are usually used (tetracycline or doxycycline)
  • Topical treatment
    • Azelaic acid
    • Benzoyl peroxide
    • Metronidazole
    • Pimecrolimus
    • Corticosteroids
  • Systemic corticosteroids
  • For recalcitrant granulomatous rosacea, isotretinoin may be attempted
  • Options
    • Intralesional triamcinolone
    • Dapsone (100mg daily)
    • Tetracyclines
    • Antimalarials
    • Vitamins (eg. ribofravin, pyridoxine)
    • Isotretinoin
    • Low-dose prednisone (starting at 10mg daily)
      • Last resort
      • Temporary improvement, followed by chronic flaring