Several rheumatologic diseases have cutaneous manifestations. At the ODAC Dermatology, Aesthetic and Surgical Conference in Orlando, Florida, Erik Stratman, MD, presented “Tips and Tricks in the Diagnosis and Management of Cutaneous Lupus and Other Cutaneous Rheumatologic Diseases.”1 Dr Stratman is a dermatologist at Marshfield Clinic Health System in Marshfield, Wisconsin.
During his presentation, Dr Stratman discussed cutaneous lupus, dermatomyositis, and morphea. He shared diagnostic tips and clinical pearls for treating these diseases in an interview with The Dermatologist.
The Dermatologist: What distinctive features can help with the diagnosis of these diseases? Are there any other tips you discussed for identifying rheumatologic conditions?
Dr Stratman: For discoid lupus, lesion morphology can be very helpful, particularly for identifying the early signs of lesional scarring and follicular accentuation of scale. The distribution can also be helpful because discoid lupus predominantly affects the sun-exposed face and neck, dorsal hands and forearms, and upper trunk. Other helpful places to examine on the body, which might otherwise be forgotten on a standard skin exam, are the conchal bowls of the ears.
For dermatomyositis, I demonstrated that some of the scalp findings on dermoscopy have the same glomeruloid vessel findings as seen on the proximal nail folds, particularly in patients with longstanding scalp involvement.
In addition, I discussed the differences between plaque, generalized, and linear morphea. It is important to discuss genital symptoms with adult patients and offer genital examinations because there is a significant rate of genital lichen sclerosis, and patients may not disclose this unless asked.
You may also like...
Q&A: High Herpes Zoster Risk in Inflammatory Skin Diseases
The New ACR/NPF Guidelines: A Rheumatologist’s Perspective
Diagnosing Psoriatic Arthritis in the Dermatology Clinic
The Dermatologist: What clinical pearls did you share for managing these conditions in a dermatology practice?
Dr Stratman: I discussed the value of getting an antinuclear antibody (ANA) and performing a thorough rheumatologic review of systems in patients presenting with discoid lupus because of the low, but real, possibility of discoid lupus being a sign of systemic lupus. Patients with higher numbers of discoid lesions seem to have a higher risk of systemic lupus development.
In addition, I suggested waiting to perform an extractable nuclear antigen (ENA) test only after the ANA is positive rather than ordering both tests simultaneously, given the high cost of ENA and very low rate of patients with ANA-negative–ENA-positive results. At my institution, for example, the ENA costs about 10 times more than the ANA. Also, when ordering ANA, I recommend ordering an extra tube blood in order to perform the ENA afterwards if the ANA is positive. Due to the fact that this is typically a chronic disease and not an emergency, an extra couple of days to wait for the ENA result does not make a difference in the management of the patient. However, it can save the patient or health system a large amount of money in unnecessary testing.
Also, repeating ANA tests over time is not usually necessary and should be avoided unless the clinical picture of the patient has changed significantly.
For dermatomyositis, I discussed newer myositis antibody panels, particularly the value of anti-melanoma differentiation-associated gene 5 (anti-MDA–5) testing, which is now commercially available. The anti-MDA–5 panel helps to predict those at greatest risk of developing interstitial lung disease. Another valuable test for anti-transcription intermediary factor–1 gamma (also commercially available), which helps predict those at higher risk of internal malignancy, particularly elderly patients.
Simply sticking to age-appropriate health screening will miss a fair amount of malignancies occurring in young patients with dermatomyositis. To that end, I typically recommend CT of the chest, abdomen, and pelvis in addition to comprehensive history and physical, including a pelvic exam in women, complete blood count, liver function tests, urinalysis, colonoscopy (or fecal occult blood test if colonoscopy is not indicated), chest X-ray, age- and gender-appropriate Pap test, testicular self-examination, mammography, and colonoscopy.
The Dermatologist: Do you have any other recommendations for treating these patients, such as best ways to obtain disease control, pain management, etc?
Dr Stratman: In patients with amyopathic dermatomyositis who are unable to achieve disease control with potent topical steroids alone, I discussed the therapeutic ladder of systemic treatment. While hydroxychloroquine is an early step of the therapeutic ladder, I often jump over that rung in the ladder due to the higher rate of drug eruptions among dermatomyositis patients treated with hydroxychloroquine. I start with methotrexate therapy if they have no contraindications to that medicine. Typically, I would plan on methotrexate 15 mg weekly, pushing higher if unresponsive.
In addition, I reviewed hydroxychloroquine therapy and the relatively new ophthalmology monitoring guidelines.2 The guidelines recommend a baseline eye exam for all patients; however, I will start the medicine while scheduling a baseline eye exam to be performed soon after.
In the new guidelines, the dosing is based on actual weight rather than ideal weight. Under the previous guidelines, the recommended dose was less than 6.5 mg/kg of ideal body weight. Now, the recommended dose is less than 5 mg/kg of actual body weight. This is important particularly for patients with low body weight. Among this patient population, the previous dosing was associated with a somewhat higher risk of developing retinopathy from hydroxychloroquine. The new guidelines suggest following up with patients who are otherwise healthy and without high risk for retinopathy after a cumulative of 5 years of therapy. I think many dermatology providers may be pushing for more frequent follow-up of 6 months or yearly, which may be unnecessary for otherwise healthy patients.
That said, it remains important to recognize patients at higher risk for retinopathy and screen them at sooner intervals than the 5-year recommended interval. Patients at higher risk include those with lower-than-normal glomerular filtration rates, those previously dosed higher than 5 mg/kg of actual body weight, those taking tamoxifen therapy, and those who show macular pathology at the baseline eye exam. These patients require closer follow-up, with eye exams at least yearly.
1. Stratman E. Tips and tricks in the diagnosis and management of cutaneous lupus and other cutaneous rheumatologic diseases. Presented at: ODAC Dermatology, Aesthetic and Surgical Conference; Orlando, FL; January 17, 2020.
2. Marmor MF, Kellner U, Lai TYY, Melles RB, Mieler WF, the American Academy of Ophthalmology. Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 Revision). Ophthalmology. 2016;123(6): 1386-1394. doi:10.1016/j.ophtha.2016.01.058