Butterfly Rash (Malar): Telltale Lupus Symptom?

The butterfly rash is a reddish purple facial rash that appears on the faces of many Lupus Warriors. 31% – 65% of people with lupus experience the skin symptom.

Many associations, funds, bloggers, and lupus advocates include a butterfly in their logos. This symbol is common as a reference to the butterfly rash (also known as the malar rash). Named from the Latin word ‘mala’ meaning jaw or cheek-bone, the rash takes the shape of a butterfly. It appears on the:

• cheeks
• bridge of the nose
• NOT on the smile/laugh lines on the side of the nose that run to the corner of the mouth
  • called the nasolabial folds

The rash can be raises or flat, blotchy or solid. In severe cases, the butterfly rash can be painful. Typically, it is a slightly itchy rash that may feel “hot.”

The American College of Rheumatology lupus diagnosis rubric includes the butterfly rash. So it is part of the evaluation for getting a lupus diagnosis. However, not all Lupus Warriors will experience the rash. How common is the rash? Because the answer was not clear after reviewing the existing research, LupusCorner conducted a poll.

LupusCorner butterfly rash poll methods

The poll was posted on LupusCorner and our Facebook page in 2016. There were 2 ways to answer the poll:

1. From the poll widget
   • Do you ever experience the butterfly rash (malar rash): Yes/No/I do not have lupus
2. In free-text responses in the Facebook comments
   • Any comments that could not be coded were removed from the sample

Poll results

There were 969 total responses. 53.4% of people with lupus experienced the butterfly rash at some point in their battle. Ten people did not have lupus and were removed from the calculation.

As a result of this poll, we have some knowledge of the self-report baselines for the LupusCorner community. While this is useful, it does not take additional lupus factors into consideration.

Butterfly rash & lupus: current context

The goal of the poll was to replicate current studies with patient-volunteered information. Because of this, some potentially relevant factors were not added the poll (or to existing research) including:

• disease severity
• racial/ethnic background
• age of lupus onset

There is some current research suggesting that the above factors may be relevant to the butterfly rash prevalence. For example, late-onset SLE (defined as onset of the disease after age 50) was more benign, with a significantly lower incidence of the malar rash according to one study conducted with a Chinese cohort of participants.

So, how does this poll compare to other prevalence statistics? Wikipedia reports that the butterfly rash is “present in approximately 46-65% of lupus sufferers and varies between populations.“

Butterfly rash prevalence in 2 distinct US Hispanic subpopulations

A study by Vilá, L. M., et al., examined two US Hispanic subgroups – focusing on 105 Hispanic SLE patients from Texas and 81 SLE patients from Puerto Rico. The average ages, in years, were 33.1 and 37.5 for the Texas population and Puerto Rican population respectively.

These two groups of participants has significant differences between them. The Texas cohort experienced the rash in 45.7% of participants while the Puerto Rican cohort experienced the rash in 65.4% of participants. Wikipedia uses these numbers to describe the butterfly rash prevalence.

While this finding is interesting, the researchers also found 7 other clinical differences were and 3 different autoantibody measures  between the groups. Additional research is necessary to better understand why these differences exist — with particular interest paid to nature/nurture differences and access to healthcare services.

Though not completed in the original study, we combined the population subgroups to find the total prevalence percentage, regardless of cohort. Interestingly, the prevalence was 54%, nearly identical to the finding of our poll

Malar rashes in Tunisian populations with lupus

This study by Houman, M H., et al., focused on a Tunisian population. The authors noted at the time of publication (2004) there were few studies on Arab populations and effectively 0 studies on North Africans/Tunisians.

First, the researchers retroactively enrolled 100 SLE Tunisian participants (92 women and 8 men) into the study. The average participant age was 32 years old. 19 of the patients were over 50 upon diagnosis, but age-of-onset figures do not appear in the results section for variability of butterfly rash.

This study found that 63 of the participants experienced the butterfly rash (63%). Also, Wikipedia cited this figure.

Cutaneous and non-cutaneous lupus symptoms

A study published in 1992 by Wysenbeek, A. J., et al., focused on all types of rashes. The study followed 81 patients with lupus at the Beilinson Medical Center in Israel. No additional information regarding the demographics of the participants was provided. Still, this study identified that 40 of the 81 (49%) people with lupus experienced a malar rash.

So there’s a consensus on prevalence of the butterfly rash? Not so fast.

While the top two studies are referenced by Wikipedia and the third is discoverable via a Google search, they are not exhaustive reviews of the research. The goal of these studies was not to determine the prevalence of the butterfly rash, though it was included in the analysis. Contradicting these findings, a larger study found a lower prevalence of the lupus symptom.

Cervera, R., et al. (2003) conducted a multi-center research study that lasted 10 years. 1,000 people participated from 7 European countries. “A total of 350 patients were from Spain, 250 from Italy, 248 from the United Kingdom, 50 from Poland, 50 from Turkey, 37 from Norway, and 15 from Belgium.” In addition to being comprised of varying ethnic and racial populations, these countries have varying cultures and availability of healthcare. These factors can change findings. So future studies should include these elements. Despite this consideration, the results presented are determined by combining all of these groups into a single cohort.

During the course of the 10-year study, 311 of the 1000 (31.1%) participants with lupus experienced the butterfly rash. Also, the study noted that the presence of malar rash, as well as other clinical manifestations like arthritis, was higher during the first 5 years of the study as compared to the second 5 years.

So the true prevalence of butterfly rashes in lupus is…

Rashes may appear in anywhere between 31% and 65%. But, there may be more to the question.

For this reason, future research should consider additional personal and disease-history factors to better identify potential causes or risk factors for the malar rash.

The butterfly rash is not sufficient for a rheumatologist to confirm a lupus diagnosis. So, if you believe you have lupus but do not experience the rash, continue tracking other symptoms.

Updated July 8, 2019