Sarcoidosis with Lupus Pernio in an Afro-Caribbean Man

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A 54-year-old man of Afro-Caribbean ancestry presented with a 2-month history of nonproductive cough, 10-day history of constant subjective fevers, and a 1-day history of bilateral cheek swelling. He was a nonsmoker and had no environmental exposure. He had a remote history of right-sided monocular vision loss 2 years prior, successfully treated empirically with corticosteroids. Examination revealed warm skin, regular tachycardia, bilateral enlarged nontender parotid glands, and a nodular violaceous rash at the tip of his nose (Figure). Blood testing for complete blood count, complete metabolic profile, liver, renal, and thyroid functions, and lactate dehydrogenase were within normal limits. His erythrocyte sedimentation rate was mildly elevated at 42 mm/h. Human immunodeficiency virus and tuberculin skin testing were negative. Computed tomography scan of the chest, abdomen, and pelvis showed multiple enlarged paratracheal, subcarinal, and bilateral matted hilar lymph nodes; peri-lymphatic micronodularity throughout both lungs (Scadding stage 2); multiple enhancing lesions in the spleen and liver; and several enlarged lymph nodes in the porta hepatis. Furthermore, there were focal areas of cortical hypoenhancement in the upper pole of the left kidney and a subtle lace-like pattern in a few bilateral upper ribs. Overall, radiologic findings were suggestive of multisystemic sarcoidosis. A magnetic resonance imaging scan of the brain showed no evidence of neurosarcoidosis. Electrocardiogram and transthoracic echocardiogram were normal. A biopsy of the nodular rash at the tip of his nose was taken, which confirmed extensive noncaseating epithelioid granulomata. A firm diagnosis of sarcoidosis on the background of lupus pernio and parotid fever was made. Differentials of this presentation would include lymphoma, cutaneous pseudolymphoma, Kaposi's sarcoma, melanoma, histiocytoma, skin tuberculosis, xanthomas, necrobiosis lipoidica, and lupus erythematosus. As per the EpiSarc study, lupus pernio with multisystemic organ involvement is a rare, distinct phenotype of sarcoidosis. 1 Lhote R Annesi-Maesano I Nunes H et al. Clinical phenotypes of extrapulmonary sarcoidosis: an analysis of a French, multi-ethnic, multicentre cohort. Eur Respir J. 2021; 572001160 Crossref Scopus (7) Google Scholar Although American Thoracic Society guidelines state that lymph node or other tissue sampling is not recommended in patients with high clinical suspicion, 2 Crouser ED Maier LA Wilson KC et al. Diagnosis and detection of sarcoidosis. An Official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020; 201: e26-e51 Crossref PubMed Google Scholar critical clinical findings such as lupus pernio can be easily missed in patients of darker skin tones. This presentation is reminiscent of Heerfordt-Waldenström syndrome, a clinical spectrum of uveitis, parotid swelling, chronic fever, and facial nerve palsy. 3 Fraga RC Kakizaki P Valente NYS Portocarrero LKL Teixeira MFS Senise PF Do you know this syndrome? Heerfordt-Waldenström syndrome. An Bras Dermatol. 2017; 92: 571-572 Crossref PubMed Scopus (0) Google Scholar Our patient lacked uveitis and facial nerve palsy, but his monocular vision loss 2 years prior was likely a sarcoid manifestation.