DermaDiagnosis
More Than a Spot of Bother
For this 5-year-old African-American boy and his family, the continual depigmentation of the skin around his congenital lesions is concerning. Can...
ANSWER
The correct diagnosis is relapsing polychondritis (RP; choice “a”). The lack of surface changes in the affected skin rules out contact dermatitis, while the lack of a positive response to antibiotics and absence of an entrance wound eliminate the possibility of an infectious etiology.
DISCUSSION
There are no tests to confirm the diagnosis of RP. It is a rare autoimmune condition that usually manifests in the later decades of life and equally affects men and women.
RP’s ability to appear in cartilage anywhere in the body and in a variety of forms makes timely diagnosis almost impossible. But this case illustrates some diagnostically useful signs to watch for.
The unexplained erythema in the ear, which very obviously spared the cartilage-free lobe, prompted a biopsy of the cartilage; this showed changes consistent with RP. A subsequent review of the patient’s ophthalmology records indicated a chronic episcleritis, most likely due to inflammation of eyelid cartilage.
Further testing was performed to rule out other explanations, such as gout, or autoimmune diseases, such as lupus. Results were negative.
The patient was then referred to a pulmonologist, who found no respiratory involvement, and a rheumatologist, for further evaluation (including blood work) to rule out other conditions and end-organ (eg, renal) involvement.
On follow-up, the patient was responding well to prednisone prescribed by her rheumatologist. Given her limited disease, her prognosis is fairly good.
