A Case of Concurrent Erythematotelangiectatic Rosacea and Cutaneous Lupus Erythematosus

Rosacea is a chronic inflammatory disorder of the central facial skin.¹ It primarily presents with flushing, facial redness, and telangiectasias. Papules, pustules, and phymatous skin changes can also occur.² 

Rosacea is a condition that is oftentimes diagnosed clinically, based on characteristic cutaneous findings. These clinical manifestations may mimic other facial skin disorders, one of which is cutaneous lupus erythematosus (CLE). CLE is a less common disorder and can be characterized by facial erythema, a malar rash, discoid lesions, oral or nasopharyngeal ulcers, and various other findings. 

CLE can present as a skin disease alone or may occur alongside systemic lupus erythematosus (SLE). SLE is an autoimmune condition that causes diverse symptoms, including fever, fatigue, and joint pain. CLE is further divided into subclasses: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE). Approximately 80% of CLE is classified as CCLE and the majority of CCLE is classified as discoid lupus erythematosus (DLE).³ A case of concurrent erythematotelangiectatic rosacea and cutaneous lupus erythematosus is presented below.

Case Presentation

A 50-year-old Caucasian male presented to the dermatology department for evaluation of an erythematous malar rash. He denied fever, chills, headaches, fatigue, dysuria, diarrhea, stomach upset, hair loss, oral or digital ulcers, history of blood clots, psoriasis, chest pain, shortness of breath, heart palpitations, numbness/tingling of extremities, dactylitis, uveitis, back pain, joint swelling, and morning stiffness. 

During the visit, background erythema and subtle acneiform papules were visualized on the patient’s bilateral cheeks and nose (Figure 1A). The patient noted these signs and symptoms had occurred intermittently for 7 years. The patient was prescribed twice daily application of topical metronidazole cream and twice daily oral doxycycline 100 mg and was instructed to follow up in 1 month.

One month after his previous visit, the patient presented again with no improvement. Reexamination still revealed background erythema and subtle acneiform papules on the bridge of the nose and bilateral cheeks. A biopsy was obtained from the left nasal ala. Pathology revealed a perifollicular infiltrate of lymphocytes and some neutrophils surrounding follicles, which was read as compatible with acne rosacea with features of background seborrheic dermatitis.

The patient was instructed to discontinue doxycycline and metronidazole. He was prescribed a course of once nightly application of topical ivermectin cream and twice daily oral sulfamethoxazole–trimethoprim 400-80. The patient noted no improvement after 6 weeks. 

A subsequent course of once-daily application of topical brimonidine 0.4%–niacinamide 4% gel was given, with no improvement noted. This was followed by a course of twice daily application of azelaic acid 15% gel, with no improvement noted.

Lab workup revealed an antinuclear antibody (ANA) titer of 1:80, with negative extractable nuclear antigens (ENA).  Complete blood count (CBC), comprehensive metabolic panel (CMP), erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), complement components 3 (C3) and 4 (C4), and double-stranded DNA (dsDNA) antibodies were all without abnormalities.

The rheumatology department was consulted to rule out systemic involvement. Rheumatology noted a malar facial rash that crossed the nasolabial fold, normal inflammatory markers, and the absence of lymphopenia, and ultimately concluded a very low suspicion of SLE.

When the patient followed up with dermatology, the rash presentation had changed. Scattered red to violaceous, indurated, broad plaques located on the face on a background of erythema were visualized on physical exam (Figure 1B). A repeat biopsy was obtained from the right nasal sidewall (approximately 6 months after the first biopsy was obtained). 

Pathology revealed hyperkeratosis, follicular plugging and atrophy, liquefaction degeneration, and perivascular and perifollicular infiltration of lymphocytes and histiocytes in the dermis (Figure 2A and 2B), with findings consistent with cutaneous lupus erythematosus. 

The patient was started on twice daily application of topical tacrolimus ointment and twice daily oral hydroxychloroquine 200 mg. Approximately 6 months later, the patient presented for follow-up.

All cutaneous symptoms had resolved and normal skin changes were visualized on physical exam (Figure 1C). Patient history, clinical findings, pathology, and response to treatment, all contributed to the diagnosis of concurrent erythematotelangiectatic rosacea and cutaneous lupus erythematosus.

Discussion

The 2017 National Rosacea Society criteria for the diagnosis of rosacea include at least 1 diagnostic or 2 major phenotypes.² A diagnosis of rosacea can be made with the presence of 1 of the following cutaneous signs: fixed centrofacial erythema or phymatous skin changes. When no diagnostic phenotype is present, 2 or more major features are considered diagnostic.

Major features include papules and pustules, flushing, telangiectasia, and ocular manifestations. Secondary signs and symptoms include burning or stinging, edema, dry appearance, and ocular manifestations. However, the presence of secondary signs alone is not considered diagnostic.²

Furthermore, rosacea can be divided into 4 subtypes: erythematotelangiectatic, papulopustular, phymatous, and ocular. The patient was originally diagnosed with erythematotelangiectatic rosacea due to the presence of a diagnostic cutaneous sign; fixed centrofacial erythema. The patient noted his symptoms were intermittent, suggesting that this erythema periodically intensified.

Additionally, the patient met 3 major features, which are also considered diagnostic for rosacea. He presented with erythematous papules and flushing (Figure 1A) and telangiectasias (Figure 1C), based on physical exam findings during his visits in the dermatology department. The patient did not meet any secondary criteria; however, these criteria are not needed to make a diagnosis of rosacea. 

The patient was ultimately diagnosed with the erythematotelangiectatic subtype of rosacea due to characteristic physical exam findings (facial erythema, flushing (Figure 1A), and telangiectasias (Figure 1C)). These findings on the physical exam were reinforced with a confirmatory biopsy compatible with the diagnosis of rosacea. Interestingly, pathology also revealed background seborrheic dermatitis. Of note, 26 percent of patients with rosacea also have facial seborrheic dermatitis.⁴ This finding also supported a diagnosis of rosacea.

However, the patient’s lack of response to several treatments led to further investigation and exploration of alternative diagnoses. The 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for SLE include an entry criterion of an elevated ANA titer of  ≥1:80.⁵

This criterion must be followed by additive criteria grouped in 7 clinical groups (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunological groups (antiphospholipid antibodies, complement proteins, SLE-specific antibodies). Only patients accumulating ≥10 points are classified as having SLE.⁵

CLE can present as a cutaneous disease, but can also occur with SLE. A cutaneous biopsy is helpful to confirm the diagnosis of CLE, and typical pathological features include follicular plugging and perifollicular lymphocytic infiltrate.⁶ CLE can be further divided into subclasses: ACLE, SCLE, and CCLE. CCLE is the most common subtype, and discoid lupus erythematosus (DLE) comprises the majority of CCLE.³

During the patient’s initial visit, he presented with a bright red, confluent eruption in a butterfly pattern (Figure 1A), which is a typical feature of ACLE. 7 In a subsequent visit approximately 6 months later, he presented with scattered indurated scaly plaques on the face (Figure 1B), which is characteristic of DLE.⁸

This patient demonstrates that lupus erythematosus is a dynamic and evolving disease process. The ACLE features visualized at the primary visit later evolved into classic DLE features. However, both ACLE and DLE fall in the spectrum of cutaneous disease. Furthermore, pathology from the repeat biopsy revealed hyperkeratosis, follicular plugging and atrophy, liquefaction degeneration, and perivascular and perifollicular infiltration of lymphocytes and histiocytes (Figure 2A and 2B), which confirmed the diagnosis of CLE.

The patient met the entry criterion for SLE (elevated ANA titer of  ≥1:80) but failed to meet further diagnostic criteria. His lab workup was unremarkable, and he had no systemic findings that supported a diagnosis of SLE. While a diagnosis of CLE was confirmed, all diagnostic criteria for erythematotelangiectatic rosacea were met as well. In fact, many findings throughout the course of treatment revealed a significant overlap between lupus erythematosus and erythematotelangiectatic rosacea. 

The patient had an ANA titer of 1:80, which is an entry criterion for a diagnosis of lupus erythematosus.⁵ However, rosacea is a chronic inflammatory disorder, and elevated ANA titers are commonly found in rosacea patients as well.⁹ Typical pathological features of CLE are follicular plugging and perifollicular lymphocytic infiltrate, 6 which were found in the second biopsy obtained from the patient. 

However, perifollicular infiltrate of lymphocytes and neutrophils is a pathological feature of rosacea¹⁰ and this feature was found in the first biopsy. To the best of our knowledge, there are no reports in the literature that describe the simultaneous presence of erythematotelangiectatic rosacea and cutaneous lupus erythematosus; however, based on clinical and histologic findings, a concurrent diagnosis was made.

Conclusion

This case highlights that the distinction between rosacea and CLE can be difficult due to their clinical and histologic overlap which poses a diagnostic challenge. In this case, an eventual diagnosis of concurrent erythematotelangiectatic rosacea and cutaneous lupus erythematosus was made. Understanding the nuances of these two conditions, as well as the fact that they can occur concurrently, can be a valuable diagnostic tool for dermatology providers. Further research is needed to establish the relationship between these inflammatory dermatoses.

Coauthored with Brendan Thomas, MD.