Sarcoidosis



Sarcoidosis


Elyse M. Love

Evan Rieder



BACKGROUND

Sarcoidosis is a systemic, idiopathic inflammatory condition with skin involvement in up to one-third of affected patients. Cutaneous involvement may include the skin, oral mucosa, and/or nails. Systemic involvement most commonly affects the lungs, but it may affect virtually any organ system.


PRESENTATION

Cutaneous sarcoidosis may be the first symptom of sarcoidosis or may present after the diagnosis of systemic sarcoidosis has been established. Over 20 morphological variants of cutaneous sarcoidosis exist, including papular, psoriasiform, annular, lichenoid, plaque, atrophic, scarring, and verrucous. Classically, sarcoidal lesions are red-brown symmetric papules and plaques, with a predilection for the face, especially the nasal ala, periocular, and perioral regions (Figure 14.6.1). Larger plaques may also be seen on the neck, upper trunk, and extremities. Lesions may be arcuate in distribution or follow patterns of trauma. Presentation of red-brown papules within a scar and/or tattoo is highly suspicious for sarcoidosis. Telangiectasia may be prominent.

Pressure with diascopy may turn red-brown papules into an “apple jelly” color, but this may be difficult to perceive in patients with Fitzpatrick skin types V and VI. Lesions may also appear hypopigmented, ichthyosiform, micropapular, psoriasiform, and/or ulcerative. The nails and oral mucosa may also be involved.

Sarcoidosis has a bimodal distribution with incidence peaks from 25 to 35 years of age and again from 45 to 65 years of age. In the United States, sarcoidosis has a higher incidence in individuals of African descent when compared with other races. Worldwide, sarcoidosis
has the highest incidence in Sweden and the United Kingdom.

More than half of patients experience remission within 3 years of diagnosis and two-thirds experience resolution within a decade of diagnosis.






FIGURE 14.6.1 A patient with cutaneous sarcoidosis with classic periorbital and palpebral red-brown papules. (From Goodheart HP, Goodheart’s Same-Site Differential Diagnosis: A Rapid Method of Diagnosing and Treating Common Skin Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2010.)




PATHOGENESIS

It is postulated that disease development occurs in genetically susceptible individuals after exposure to an unknown triggering antigen. Debate continues as to whether the triggering antigen is created from autoimmune mechanisms or an external exposure, such as infection.

Jun 29, 2020 | Posted by in Dermatology | Comments Off on Sarcoidosis
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