Variability of Sarcoid Skin Manifestations Is Illustrated in a Patient With Multiple Concurrent Pinna Lesions

Sarcoidosis is a systemic autoimmune disease characterized by the presence of noncaseating epithelioid granulomas. While the exact etiology of sarcoidosis is unclear, a currently favored theory states that sarcoidosis is caused by an environmental trigger in an individual with a genetic predisposition. ^1-2 Certain HLA polymorphisms are associated with the development of sarcoidosis, suggesting that aberrant antigen presentation plays a role in the generation of an inappropriate immune response. ^2-3

Sarcoidosis can involve almost any organ and tissue type, though there is a predilection for certain organs and anatomic sites. Otolaryngologic findings include cervical adenopathy, skin and cartilage lesions of the pinna, and parotid gland swelling. ^{1,4 -6} A rare presentation of sarcoidosis is Heerfordt-Waldenström syndrome (uveoparotid fever), which is characterized by a combination of parotid gland enlargement, facial palsy, anterior uveitis, and a low grade fever.

A 51-year-old man with a known history of sarcoidosis presented to our otolaryngology clinic for evaluation of recurrent pinna lesions and aural fullness. He previously had a computed tomography of the chest showing multiple calcified hilar lymph nodes and a perilymphatic distribution of micronodules. Physical examination revealed a left pinna with an ulcerated cutaneous lesion in the concha. The right ear demonstrated a very different appearing tragal and preauricular raised skin lesion (Figures 1 and 2). Neither lesion was tender or pruritic. The patient had been taking low-dose prednisone tablets daily, but the lesions did not resolve.

Figure 1.

An ulcerated cutaneous lesion involving the left pinna.

Figure 2.

Raised tragal and preauricular skin lesions as well as a smaller conchal skin lesion.

The mainstay of treatment of sarcoidosis is oral corticosteroids. Treatment of skin lesions with topical or intralesional corticosteroids was attempted by other researchers with limited success. ⁶ Other immunosuppressants such as methotrexate and tumor necrosis factor-α antagonists may be used in refractory cases. ⁷

It is important to note that sarcoidosis has variable expression among individuals and usually follows a waxing and waning disease course that may resolve spontaneously within a few years of onset, or be lifelong. A significant proportion of patients with sarcoidosis have a mild or “forme fruste” disease. They do not seek any treatment and their diagnosis is often made based on incidental findings. Skin manifestations may imitate other diseases, making sarcoidosis particularly challenging to diagnose. ^2,7 Even synchronous sarcoidosis-related skin lesions in the same patient can vary greatly in appearance, as this case demonstrates. A high level of clinical suspicion for sarcoidosis is needed, and the presence of unexplained pinna lesions should prompt further investigation.