In late summer 2017, a 21-year-old African American woman without previous medical problems or sexual activity presented with 6 weeks of intermittent high-grade fevers, painful vulvar lesions, 20-lb weight loss, fatigue, and malaise. At symptom onset, she experienced tender acneiform-like nodules on the face and sternum, and painful blistering of the lower lip that had healed by time of presentation. Physical examination revealed a cachectic woman with a disheveled appearance. There was no evidence of iritis or uveitis, though bilateral retinal vasculitis with small focal areas of retinal hemorrhage were visualized on ophthalmological examination. Oral examination did not reveal any aphthous ulcers, though dry mucous membranes were discovered. There was no alopecia. A careful palpatory evaluation was performed in conjunction with a 30-second Chapman's reflex (CR) visceral screening examination.
4 Notable findings included anterior cervical and inguinal lymphadenopathy. Lymph nodes were painful, warm, and moderately enlarged, measuring approximately 1 to 2 cm. She was also found to have multiple vulvar ulcerations. The 30-second visceral screening examination was without acute findings. Given the patient's lymphadenopathy of the neck, the physician (J.A.) decided to evaluate for specific CR corresponding to this region of the body. The physician applied firm digital pressure to the right medial humerus. The soft tissue in this area was both edematous and ropy, measuring about the size of an almond. The area overlying this CR was granular in quality and accompanied by slight pain endorsed by the patient without radiation. Given establishment of the positive anterior CR, a posterior CR was palpated for confirmation. Similar findings were demonstrated at the left transverse process of T3-T5, though pain was less intense. No radiation of pain was endorsed by the patient. Given the location in the soft tissue, palpatory quality, and distinct pain characteristics, a CR was validated (
Figure 1).
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Initial screening labs were obtained (
Table). Computed tomography (CT) of the patient's neck revealed enlargement of multiple cervical lymph nodes, including an enlarged right supraclavicular lymph node and a right level 2b node, demonstrating a low-density center concerning for a neoplastic process (
Figure 2). An excisional cervical lymph node biopsy was performed with simultaneous biopsies of the vulvar ulcerations and nodular skin lesion overlying the sternum. Histology of the right cervical lymph node revealed architecture distorted by large areas of histiocytes with prominent karyorrhectic debris (
Figure 3). Histiocytes were noted to have abundant pink cytoplasm and frequent crescent-shaped nuclei in combination with a paucity of neutrophils and eosinophils. Other areas of the lymph node had mildly expanded paracortical T-cell zones with scattered immunoblasts and plasma cells. By paraffin immunohistochemistry, the histocytes were positive for CD68, CD163, and myeloperoxidase. CD123 highlighted an increased number of plasmacytoid dendritic cells present singly and in discrete aggregates. Findings were consistent with KFD; however, lupus lymphadenitis (LL) was also considered histologically.
Shave biopsy of the left posterior labia minora was noted to have brisk dermal neutrophilic infiltrate; sebaceous glands were noted to be very edematous (
Figure 4). These histologic findings were not specific. Flow cytometry was negative, and malignancy was excluded. Rheumatologic work up revealed positive serologies for antinuclear antibody (ANA), anti-Smith antibody, and low complement. A positive ribonucleoprotein (RNP) was also noted (
Table). Pathergy test was negative. Infectious etiologies were considered but ruled out based on absence of positive laboratory findings or blood cultures. Rheumatic conditions considered included KFD, SLE, subacute cutaneous lupus erythematosus (SCLE), Behcet's disease, mixed connective tissue diseases, and periodic fever syndrome.
Given the histological findings on biopsy in correlation with the patient's physical exam and history, a provisional diagnosis of KFD was made, and she was treated with colchicine, hydroxychloroquine, and prednisone, resulting in improvement of her symptoms. At follow up 5 months after presentation, she was found to have new nonscarring alopecia and lymphopenia, further indicating systemic lupus erythematosus (SLE) based on 2012 diagnostic criteria and new diagnostic criteria published by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) in 2019.
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