(Figures 3.16 and 3.17A, B) - Chronic multisystem inflammatory disease characterized by non-caseating granulomas of unknown etiology
- Related to ↑ activity of cell mediated immune system
- ↑ Frequency and severity in African American patients
- Presents with cutaneous findings in approximately 30–40% patients, may be sole or initial manifestation
- Presents typically with non-scaly, skin-colored to red-brown circinate or annular infiltrated papules/plaques on face, lips, neck, trunk, extremities; cutaneous sarcoidosis may develop within pre-existing scars
- Hypopigmented lesions not uncommon in African-American patients
- Sarcoidal plaques may appear psoriasiform
- Less common presentations include ichthyosis over lower legs, hypopigmentation, scarring alopecia, and ulcerations
- Variants (listed below)
- Histology: superficial and deep sharply-defined naked epithelioid granulomas, giant cells, minimal lymphocytes near granulomas, eosinophilic stellate inclusion bodies (asteroid bodies) or round basophilic laminated inclusions (Schaumann bodies) seen in giant cells
- Diagnosis (of exclusion): supported by ↑ ACE, ↑ calcium, ↑ ESR
- Treatment: topical, IL or systemic corticosteroid, hydroxychloroquine, methotrexate or other immunosuppressant
| | | | | | | Sarcoidosis Variant | | Clinical Findings | | | Lupus pernio | | Violaceous doughy infiltration on nose, cheeks or earlobes; often associated with chronic sarcoidosis of lungs, chronic | | | Darier-Roussy disease | | Also known as sarcoidal panniculitis; painless subcutaneous mobile nodules without epidermal change | | | Löfgren’s syndrome | | Acute sarcoidosis; erythema nodosum, hilar adenopathy, acute iritis, migrating polyarthritis, and fever | | | Mikulicz syndrome | | Complex of symptoms caused by a variety of systemic disorders (i.e. Sjögren syndrome, lymphoma and sometimes sarcoidosis) | | | | | Parotid and lacrimal enlargement with swelling, ± sicca symptoms | | | Parinaud
oculoglandular syndrome | | Conjunctivitis with ipsilateral lymphadenopathy | | | | | | | | | Also caused by infection (cat-scratch fever or tularemia) | | | | | | | | | | | | | | |
| | | Heerfordt’s syndrome | | ‘Uveoparotid fever’; fever, parotid gland enlargement, anterior uveitis, facial nerve palsy | | | Erythema nodosum | | Seen in acute or subacute sarcoidosis; good prognostic sign, associated with transient sarcoidosis that resolves spontaneously | | | Oral sarcoidosis | | May involve mucosa, tongue, major salivary glands, hard palate and gingival tissue | | | Ocular sarcoidosis | | Seen in 15–25%: anterior uveitis (common), lacrimal gland involvement, chronic uveitis leading to adhesions, glaucoma, and blindness | | | Non-mucocutaneous
findings | | Lung disease (alveolitis, fibrosis, hilar adenopathy), liver, spleen, bone, kidney, heart, GI involvement; hypercalcemia |
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| | Figure 3.16 Sarcoidosis
(Courtesy of Dr. Iris K.
Aronson) |
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