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Solar urticaria and possible autoimmune disease

Question:

6/26/2019
A 55 year-old female with solar urticaria. Seen originally on 12/16 at rheum/derm for muscle weakness and rash with mild increase CPK and atelase, ANA 1-40 and normal CBD. Antihistone and typical SLE w/u negative. Plaquenil no help. Tests then showed +++ in visible spectrum and less in UVA spectrum. (-) UVB and porphyrins. Uses/used various physical sun blocks, SPF clothing, tinted car windows, UVA filters at work, and UV-spectrum umbrellas. Repeated biopsies= subacute spongiotic dermatitis its dermal inflammation. Last seen by derm - 114 patch tests (-) and started on XOLAIR 150 mg q four weeks since 6/2018. Slight improvement but now needed leave of absence since 2/2019. Issues are Xolair is causing alopecia and bilateral hip pain. Other w/u include (-) skin testing.

Other concerns include continued muscle weakness especially if extremely tired. She can only be exposed to outdoors for 10 minutes with sunblock. She develops a rash on sun exposed areas which is erythematous based with slight "welts." Once she goes inside, she will need 3-4 days for her skin to return to normal. OTC antihistamines of no value. She is on Turmeric.

Current plan is to stay on Xolair through summer and then a break. I suggested she speak wither dermatologist re: PUVA, Dupixent, or Cyclosporine. I question the diagnosis due to the prolonged time to have rash improve and her muscle weakness. I reviewed the other articles you sent on a previous solar urticaria derm question. Thanks in advance for any suggestions.

Answer:

Your question was forwarded to Dr. Karen Hsu Blatman, an instructor at Harvard Medical School and expert in urticaria. Her response is below.

“I agree with your assessment that your patient’s presentation is atypical for the classic definition of solar urticaria.

The reasons are as follows (as you have mentioned): the repeated biopsies reveal subacute spongiotic dermatitis, and the prolonged rash, as solar urticaria should resolve within 24 hours.

As she does not seem to have urticaria, has had minimal improvement for more than one year, and has significant side effects associated with omalizumab, I would discontinue omalizumab. There are case reports of transient hair loss and alopecia associated with omalizumab.

As I understand from your question, she is only taking turmeric and omalizumab at this time, no other medications or supplements. Do you know if the phototesting results were immediate? I wonder if EMG and muscle biopsy may be helpful to evaluate for dermatomyositis, which may be present with a photosensitive rash.

While I would defer to dermatology and rheumatology for further work-up, I would agree with considering a steroid sparing immunosuppressant for treatment, such as mycophenolate mofetil or cyclosporine.”

St John’s Wort ingestion has been reported to cause a photoallergic dermatitis. The differential of light induced dermatoses is long and includes polymorphous light eruption, actinic prurigo, musk and sandlewood contact sensitivity, atopic dermatitis, cutaneous T cell lymphoma, disseminated actinic porokeratosis, reticular erythematous mucinosis, photoallergic reaction to dandelion or common tansey or other weeds (1-4).

1. The natural history of chronic actinic dermatitis.
Dawe RS, Crombie IK, Ferguson J
Arch Dermatol. 2000;136(10):1215.
OBJECTIVE To determine the prognosis for resolution of abnormal cutaneous photosensitivity in patients with chronic actinic dermatitis (also known as the photosensitivity dermatitis and actinic reticuloid syndrome).
DESIGN Historical cohort study involving follow-up of patients for up to 24 years from diagnosis.
SETTING A Scottish tertiary referral center for investigation of photodermatosis.
PATIENTS One hundred seventy-eight patients with chronic actinic dermatitis, 62% of a cohort of 285 living patients identified in the Photobiology Unit database.
INTERVENTIONS Recall for repeated clinical assessment and monochromator phototesting. All patients underwent patch testing when initially assessed; this was repeated at follow-up in a subgroup of patients.
MAIN OUTCOME MEASURES Resolution of abnormal photosensitivity, defined as clinical resolution and return of phototest responses to within normal population limits. In addition, possible prognostic factors for resolution of photosensitivity were examined.
RESULTS The probability of abnormal photosensitivity resolving by 10 years from diagnosis is 1 in 5. Particularly severe abnormal UV-B photosensitivity (minimal erythema dose at 305+/-5 nm half-maximum bandwidth,
CONCLUSIONS Newly diagnosed patients can be told that most of them will improve with appropriate UV/visible light and allergen avoidance and that there is hope that their photosensitivity will completely resolve.

Photobiology Unit, Department of Dermatology, Ninewells Hospital and Medical School, Dundee, DD1 9SY, Scotland. rob.dawe@dial.pipex.com

2. Chronic actinic dermatitis: an analysis at a single institution over 25 years.
Que SK, Brauer JA, Soter NA, Cohen DE
Dermatitis. 2011;22(3):147.
BACKGROUND Chronic actinic dermatitis (CAD) is a rare photosensitivity disorder with scant epidemiologic data.
OBJECTIVE To evaluate demographic data and results of photopatch and patch tests over a 25-year period.
METHODS Retrospective chart review of patients with CAD from 1993 to 2009.
RESULTS Forty patients
had a mean age of 57.8 years, and 27 (67.5%) were men. Twelve patients (30%) were skin types I and II, and 17 (42.5%) were skin types V and VI. Nine patients (22.5%) were younger than 50 years, and 4 of these (44.4%) were men. One of the nine patients (11.1%) was skin type I, and 4 (44.4%) were skin types V and VI. Carba mix and para-phenylenediamine were the two most commonly positive agents in patch tests. Sunscreens and plants and plant derivatives were the most commonly positive agents in photopatch tests.
CONCLUSIONS Our findings suggest a trend of two new classes of North American patients at our institution being diagnosed with CAD-younger women with skin types IV to VI and older men with skin types I to III. We observed a greater-than-expected number of positive patch-test reactions to para-phenylenediamine. We suggest that patch testing and photopatch testing of individuals may be useful adjuncts in the assessment of CAD.

Photomedicine Section, Charles C. Harris Skin and Cancer Pavilion, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, NY, USA.

3. Contact allergic sensitivity to plants and the photosensitivity dermatitis and actinic reticuloid syndrome.
Frain-Bell W, Johnson BE
Br J Dermatol. 1979;101(5):503.
Contact allergic sensitivity to oleoresin extracts from Compositae plants was found to be usually present in individuals suffering from the photosensitivity dermatitis and actinic reticuloid syndrome. It was demonstrated in forty-seven out of fifty-five examples of this syndrome. These results provide support for the view that contact allergic sensitivity is an important aspect of the state of chronic photosensitivity in the middle-aged and elderly male.

4. Contact and photocontact sensitization in chronic actinic dermatitis: sesquiterpene lactone mix is an important allergen.
Menagé H, Ross JS, Norris PG, Hawk JL, White IR
Br J Dermatol. 1995;132(4):543.
Eighty-nine patients with chronic actinic dermatitis (CAD) were retrospectively studied: 69 (78%) male and 20 (22%) female, with mean ages of 66 and 64 years, respectively; nine (10%) were dark skinned. Eight (9%) were abnormally sensitive to UVB wavelengths alone, 74 (83%) to UVB and UVA, and seven (8%) to UVB, UVA and visible radiation. Eighty-six patients were patch tested to an extended standard European series of contact allergens, including in 80 cases a 0.1% mix of three sesquiterpene lactones, and photopatch tested to a standard photopatch series. Sixty-four of these 86 patients (74%) had positive patch or photopatch tests; 36% (29 of 80) were sensitive to the sesquiterpene lactone mix, 21% (18 of 86) to fragrance compounds, 20% (17 of 86) to colophony, and 14% (12 of 86) to rubber chemicals. Ten (12%) had positive photopatch tests; five (6%) to musk ambrette, six (7%) to sunscreens and one to both. Fourteen of the eighty-nine patients with CAD (16%) had preceding endogenous eczema. In 18 of 86 patients (21%), CAD occurred alone, with neither detectable contact nor photocontact allergy, nor a preceding history of endogenous eczema. This study confirms the association between Compositae (sesquiterpene lactone) dermatitis and CAD.

I hope this information is of help to you and your practice.

All our best.
Dennis K. Ledford, MD, FAAAAI
Karen Hsu Blatman, MD, FAAAAI