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Sarcoidosis - Submitted on: Tuesday May, 13, 2003
Contributed by:Bernadette Liegl, M.D., N/A
Sarcoidosis is one member of a large family of granulomatous disorders in which the common denominator is the histological demonstration of epitheloid cell granulomas. The cause is unknown although it seems to be associated with several immunological abnormalities.

Definition:
Sarcoidosis is one member of a large family of granulomatous disorders in which the common denominator is the histological demonstration of epitheloid cell granulomas. The cause is unknown although it seems to be associated with several immunological abnormalities.

Clinical Features:
This systemic disease may involve any organ of the body, but most commonly it affects the lung, lymph nodes, skin and eyes. Young adults are predominantly affected. 25% of the patients with systemic sarcoidosis have cutaneous lesions. Sarcoidosis can also occur in association with erythema nodosum (Loefgren syndrome). It is already known that granulomatous diseases are cosely linked to a deregulated TNF-alpha production. Recent analyses provide evidence that erythema nodosum associated with sarcoidosis might be pathogenically linked to altered TNF-alpha production due to a genetic promotor polymorphism. Sarcoidosis has also be reported in association with HIV infection. Helper T-lymphocyte depletion may attenuate granuloma formation. Active sarcoidosis may develop after highly active antiretroviral therapy (HAART).
There are different clinical forms of cutaneous sarcoidosis.
·        Papules, plaques and nodules in an annular or      
       serpiginous pattern.
·        Maculopapular eruptions associated with acute
       lymphadenopathy, uveitis or pulmonary involvement.
·        Plaques with marked telangiectasia (angiolupoid
       sarcoidosis).
·        Lupus pernio (violaceous nodules on the nose,   
       cheeks and ears).
·        Subcutaneous nodules of sarcoidosis are quite rare
       and occur in association with other lesions or
       alone. They were first described by Darier and
       Roussy.
·        Uncommon variants are cicatricial alopecia,
       ulcerative necrotizing and mutilating forms,
       ichtiosiform sarcoidosis, verrucous, psoriasiform,
       hypopigmented lesions and erythroderma.

The majority of the lesions resolve without scarring, however fibrosis and scarring can occur.


Histology:
Figures
(Click on an image for a larger view)
Sarcoidosis. Low magnification.
Sarcoidosis. Non-necrotizing naked granuloma
Sarcoidosis histologically a non-necrotizing granulomatous dermatitis. (Figure 1) As such it is a diagnosis of exclusion exclusion and depends of clinicopathological correlation.

The dermal granulomatous infiltrates may be limited to the superficial dermis or can involve the entire dermis and subcutis.

The granulomas are composed of epitheloid histiocytes, multinucleated giant cells, usually of Langhans type, with a sparse infiltrate of lymphocytes in the periphery.

Perigranulomatous fibrosis may be present. The classical appearance is typically refered to as “naked granulomas”. The giant cells can contain Asteroid bodies (star shaped eosinophilic structures) or Schaumann bodies ( round or oval, laminated and calcified especially at there periphery), both of which are unspecific inclusions.
In some granulomas small foci of necrosis, fibrinous or granular material may be seen. In rare cases polarizable particles are associated with granuloma formations in the skin in patients with sarcoidosis. Marked dermal scarring is only seen in lupus pernio, necrotizing and ulcerative lesions. In verrucous lesions, epidermal hyperplasia is a feature where the ichtiosiform variant shows hyperkeratosis. Other epidermal changes may include parakeratosis, acanthosis, atrophy, spongiotic reaction, lichenoid tissue reaction, epidermal vesiculation.

Differential Diagnosis
Sarcoid- like tissue reaction to foreign material like silica, tattoo, pigments, zirconium, berillium and certain man- made fibres introduced into the skin may induce a granulomatous reaction which resembles sarcoidosis. The giant cells may contain foreign material, which is difficult to detect in the routine microscopy but may be birefringent in polarized light. Especially silica can produce sarcoid- like foreign body reaction in scars many years after trauma.   


Treatment:
Corticosteroids are the first line treatment.
Particularly, they are indicated for uveitis, disfiguring skin lesions, worsening chest roentgenogram, myocardial and neurological involvement. Alternative treatments are Oxyphenbutazone for cases of active exsudative sarcoidosis and chloroquine and potassium para- aminobenzoate in chronic fibrotic sarcoidosis.

Bibliography:
  • Sarcoidosis. Patel,Greer, J-Am-Acad-Dermatol.2001 44(5):725-43.
  • Epidermal changes in cutaneous lesions of sarcoidosis. Okamoto et all. Am-J-dermatopathol.1999 21(3): 229-33.
  • Foreign bodies in sarcoidosis. Kim, Triffet,Gibson. Am-J-Dermatopath. 2000 22(5) 408-12.
  • Sarcoidosis in a patient with AIDS: a manifestation of immune restoration syndrome. Mirmirani et all.J-Am-Acad- Dermatol. 1999 41(2 Pt 2): 285-6.
  • Sarcoidosis .An update review. Kerdel FA, Moschella SL. J Am Acad Dermatol 1984; 11: 1-19.
  • Cutaneous sarcoidosis. Samtsov AV. Int J Dermatol 1992; 31: 385-391.
  • Sarcoidosis presenting as multiple subcutaneous nodules. Shidrawi RG. Clin Exp. Dermatol 1994; 19: 356-358.
  • Tumor necrosis factor-alpha promotor polymorhism in erythema nodosum.Labunski et all. Acta-dermato-venerologica. 2001 Jan-Fab; 81(1):18-21.
     

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