Race for the Case answers

Diagnose this new case by Jessica Forbes Kaprive, DO

A 47-year-old African American patient with a medical history of hypertension and major depressive disorder presented with a chief complaint of rash, located on the face. She describes the rash as asymptomatic and had been enlarging over the past year, involving multiple areas of her face including the bilateral medial canthus, nasolabial sulcus, forehead, and right cheek. She has used topical hydrocortisone periodically. She denied any history of prior skin conditions, recent travel, autoimmune disease, new hygiene products, and has no relevant positive review of systems.

1. What is the most likely diagnosis?
Answer: Cutaneous sarcoidosis. African Americans have the highest disease incidence and also a more severe/progressive clinical course. Females are more likely affected than males with a bimodal incidence peak at 25-35 yo and 45-65 yo.

2. Describe the classic histopathologic findings of this condition.
Answer: The classic histopathologic findings include superficial and deep dermis packed with nodules of well-formed, non-caseating, “naked epithelioid granulomas” lacking significant surrounding lymphocytes. Asteroid bodies (star-shaped eosinophilic inclusions of collagen) and Schaumann bodies (basophilic calcium and protein inclusions) are commonly seen in cutaneous Sarcoidosis.

3. What are common conditions that one could consider on the differential diagnosis of pink facial papules/nodules?
Answer: A non-exhaustive differential diagnosis for red, facial papules and nodules include granulomatous rosacea, cutaneous lupus, B-cell lymphoproliferative disorders, pseudolymphoma, tuberculosis, cutaneous leishmaniasis, lymphocytic infiltrate of Jessner, granuloma faciale, lupus miliaris disseminatus faciei.

4. What is the relevant work-up for this condition?
Answer: The primary outcome is to determine if there is internal organ involvement. Routine laboratory investigation is recommended including a CBC with differential, CMP with liver function tests, ESR, serum calcium, 24-hour urine calcium and serum ACE level. Chest radiography should be performed which will often reveal bilateral hilar adenopathy. Common lab findings include hypercalcemia and lymphopenia. High resolution CT may be more sensitive at determining the extent of disease. PFT (pulmonary function tests) may show decreased vital capacity, diffusion capacity and total lung capacity. It is also pertinent to as patients may experience heart block or arrhythmia. It is also important to recommend patients schedule regular eye exams.

5. What is the clinical course and treatment?
Answer: Cutaneous involvement of sarcoidosis occurs in about 20 to 35 percent of the patients with systemic disease and may be the initial manifestation of the disease. Cutaneous sarcoidosis lesions may be the only clinical manifestation, however the disease can also involve the lymph nodes, lungs, eyes, liver, heart, joints and brain. In a small cohort study, almost 25 % of patients displayed sarcoid skin lesions prior to systemic organ involvement. Regular follow up with a dermatologist is necessary to monitor disease activity and to allow early detection of systemic involvement in cases of sarcoidosis limited to the skin. For patients with severe or progressive skin lesions and/or widespread cutaneous involvement, systemic corticosteroids may be required. Steroid-sparing agents such as hydroxychloroquine and methotrexate are used for chronic, skin-predominant disease.

References

1. Alihkan A, Hocker TLH. Review of Dermatology, 3.6 Granulomatous/Histiocytic Disorders, General Dermatology. Elsevier; 2017

2. Boch K, Langan EA, Zillikens D, Ludwig RJ, Kridin K. Evaluation of clinical and laboratory characteristics of patients with cutaneous sarcoidosis: A single-center retrospective cohort study. Front Med (Lausanne). 2022 Oct 10;9:980507. doi: 10.3389/fmed.2022.980507. PMID: 36300194; PMCID: PMC9589039.

3. Drent M, Crouser ED, Grunewald J. Challenges of sarcoidosis and its management. N Engl J Med. (2021) 385:1018–32. 10.1056/NEJMra2101555

4. Yanardag H, Tetikkurt C, Bilir M, Demirci S, Iscimen A. Diagnosis of cutaneous sarcoidosis; clinical and the prognostic significance of skin lesions. Multidiscip Respir Med. 2013 Mar 22;8(1):26. doi: 10.1186/2049-6958-8-26. PMID: 23521826; PMCID: PMC3614473.