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Sarcoidosis with lupus pernio in an Afro-Caribbean male

Published:January 13, 2023DOI:https://doi.org/10.1016/j.amjmed.2022.12.019
      A 54-year-old male of Afro-Caribbean ancestry presented with a 2-month history of non-productive cough, 10-day history of constant subjective fevers, and a 1-day history of bilateral cheek swelling. He is a non-smoker and had no environmental exposure. He had a remote history of right-sided monocular vision loss two years prior, successfully treated empirically with corticosteroids. Examination revealed warm skin, regular tachycardia, bilateral enlarged non-tender parotid glands, and a nodular violaceous rash at the tip of his nose (Figure 1). 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/hr. 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. An MRI Brain showed no evidence of neurosarcoidosis. ECG and transthoracic echocardiogram were normal. A biopsy of the nodular rash at the tip of his nose was taken, which confirmed extensive non-caseating epitheloid 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
      • Lhote R
      • Annesi-Maesano I
      • Nunes H
      • et al.
      Clinical phenotypes of extrapulmonary sarcoidosis: an analysis of a French, multi-ethnic, multicentre cohort.
      . Though ATS guidelines state that lymph node or other tissue sampling is not recommended in patients with high clinical suspicion
      • Crouser ED
      • Maier LA
      • Wilson KC
      • et al.
      Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline.
      , 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
      • Fraga RC
      • Kakizaki P
      • Valente NYS
      • Portocarrero LKL
      • Teixeira MFS
      • Senise PF.
      Do you know this syndrome? Heerfordt-Waldenström syndrome.
      . Our patient lacked uveitis and facial nerve palsy, but his monocular vision loss two years prior was likely a sarcoid manifestation.
      Figure 1
      Figure 1Nodular, violaceous rash on patient's nose; lupus pernio

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      References

        • Lhote R
        • Annesi-Maesano I
        • Nunes H
        • et al.
        Clinical phenotypes of extrapulmonary sarcoidosis: an analysis of a French, multi-ethnic, multicentre cohort.
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        Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline.
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        • 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: 571-572https://doi.org/10.1590/abd1806-4841.20175211