Abstract
A 17-year-old girl presented with persistent swelling and erythema of the midportion of the helix of the pinnas, with no associated history of pain, fever, or hearing loss. The area was erythematous, with crusting and a minimal amount of pus. Her otologic examination was otherwise normal. A culture grew Staphylococcus (nonaureus). Over a 9-month period, she had flair-ups of the lesions with minimal response to topical and oral antibiotics. A dermatology consultation was obtained, and she was started on topical steroids, with no clinical improvement. She then developed raised erythematous lesions. Blood work showed no obvious immune disorder or evidence of an infectious process. A full-thickness skin biopsy was then taken, and pathologic examination found noncaseating granulomatous dermatitis; the histopathologic diagnosis was sarcoidosis. The patient was then referred to rheumatology for further evaluation and treatment. Although sarcoidosis is rare in the pediatric population, skin lesions that do not respond to medical therapy should be considered for biopsy.
1
Introduction
Sarcoidosis is a disorder of unknown etiology that is characterized by noncaseating granulomatous lesions. It can present in any organ of the body, although 80% to 90% of cases include respiratory involvement . Diagnosis is made based on a combination of histopathologic and clinical presentation. The classical presentation of sarcoidosis includes hilar lymphadenopathy in association with elevated serum angiotensin-converting enzyme (ACE) levels. Epidemiologically, rates of sarcoidosis are higher in women, African Americans, and those in the third decade of life .
Approximately 10% to 15% of patients with sarcoidosis have otolaryngologic signs or symptoms, but these are rarely the presenting complaint . There have been rare mentions of cutaneous external ear involvement, mainly consisting of raised nodular skin lesions of the auricular helix as well as anecdotal reports of sarcoidosis in the middle ear and auditory nerve . Middle ear involvement is typically secondary to eustachian tube dysfunction caused by obstruction from nasopharyngeal symptoms; direct involvement of the mastoid or middle ear is uncommon . Fewer than 1% of patients with sarcoidosis experience hearing loss, and when hearing loss occurs, its presentation in terms of the type (sensorineural, conductive, or mixed), degree (mild, moderate, or severe), and laterality (unilateral or bilateral) is variable .
2
Case report
A 17-year-old girl presented for evaluation of swelling of the helix of the pinnas, with no recent history of fever, hearing loss, or pain. Her medical and surgical history was remarkable for Chiari malformation for which she underwent a craniotomy and headaches for which she takes propranolol. The midportion of her helix was erythematous with crusting and a minimal amount of pus. The external auditory canal and tympanic membrane were normal. The remainder of the head and neck examination was normal with no palpable lymph nodes. A culture was obtained and demonstrated Staphylococcus , nonaureus. Over a period of approximately 9 months, she had flair-ups of the lesions that included increased swelling and pain with minimal response to topical as well as oral antibiotic therapy. The patient was then seen by dermatology, and no definitive diagnosis was given. She was started on topical steroids with no resolution of the lesions. The patient then developed raised purple and erythematous bumps. She had no other otologic symptoms or any systemic complaints; blood work looking for an infectious or immune disorder was negative (see Fig. 1 for laboratory values). She underwent a full-thickness skin biopsy to the level of the perichondrium ( Fig. 2 ). Pathologic examination found noncaseating granulomatous dermatitis, with no microorganisms. The histopathologic diagnosis was sarcoidosis. She was referred to rheumatology for further systemic evaluation and treatment.
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