Wednesday, June 08, 2016

Alopecia Universalis in a Teenage Girl

Presented by Henry Foong, Ipoh, Malaysia

Here is a patient I saw recently.  She is a 17-year-old girl who has a history of severe alopecia since the age of 12 years.  It was abrupt and sudden with marked loss of scalp hair followed by eye brows and other body hair.  Within a month, she had developed alopecia universalis.  She was initially treated with intralesional triamcinolone and topical minoxidil but did not help.  Subsequently she had NB-UVB 3 times weekly in the local hospital but that also did not do much good.  She was advised to go to tertiary centres in KL but was disappointed with only one visit.  She could not go to NSC in Singapore because of financial constraints.  As a Malay, she always wears a tudong to cover her scalp.  There was no other systemic complaints.  She is the 4th in the family of 5 siblings.  No family history of alopecia. 

She saw me yesterday.  Am trying out DPCP diphencyprone sensitisation for her.  She had a previous sensitisation done but quit after one treatment.  She had total alopecia affecting the scalp, eyebrow, eyelash, axillary and suprapubic area. Used 0.1% DPCP concentration, left on the scalp for 24 hours and reviewed the next day.  She does have good reaction with small vesicles and plan to do it weekly till her hair grows.  The eyebrow hair loss was treated with intralesional triamcinolone acetonide injection of 10 mg/ml strength.  According to literature, topical minoxidil 5% solution, topical clobetasol ointment and weekly methotrexate 25mg/wk do help too.  Other novel therapy would include JAK inhibitors.
Comment:  Who has had real success treating patients with AU? Are there any lab tests of real value?

After DPCP sensitization


References:
1. Clinical Efficacy of Diphenylcyclopropenone in Alopecia Areata: Retrospective Data Analysis of 50 Patients.  Chiang KS, Mesinkovska NA, Piliang MP, Bergfeld WF. J Investig Dermatol Symp Proc. 2015 Nov;17(2):50-5.
Abstract: Diphenylcyclopropenone (DPCP) is widely considered the most effective topical immunotherapy for refractory or extensive alopecia areata (AA), but questions regarding how long to try DPCP therapy before terminating and what factors are prognostic of therapeutic success still remain unanswered. In this retrospective study of 50 AA patients, we evaluated DPCP efficacy and identified patient factors predictive of therapeutic success/failure. The median duration of DPCP treatment was 3 years, with 47% patients experiencing their first regrowth in the first 6 months of DPCP therapy, 20% between 6 months-1 year, and 8% between 1-2 years. In our study, treatment success, defined as 50% terminal hair regrowth, was reached in 71% of alopecia totalis patients and in 56% of alopecia universalis patients. Three factors were statistically significant predictors of poor treatment outcome-extent of hair loss before DPCP treatment, history of thyroid disease, and extent of body hair involvement. Relapse was observed in 44% of patients and significantly associated with history of thyroid disease. Common side effects were itching, rash, and local lymphadenopathy. The results of this study support our belief that DPCP therapy is a viable treatment option, can be successfully accomplished at home, and should not be terminated before 2 years.

2.  Pulse corticosteroid therapy for alopecia areata: study of 139 patients.
Nakajima T1, Inui S, Itami S. Dermatology. 2007;215(4):320-4.
Author information
Abstract: Of the patients, 72.7% had hair loss on > 50% of their scalp area. Among the recent-onset group (duration of AA < or = 6 months), 59.4% were good responders (> 75% regrowth of alopecia lesions), while 15.8% with > 6 months duration showed a good response. Recent-onset AA patients with less severe disease (< or = 50% hair loss) responded at a rate of 88.0%, but only 21.4% of recent-onset patients with 100% hair loss responded. No serious adverse effects were observed.

3. Association between vitamin D levels and alopecia areata.
Mahamid M, Abu-Elhija O, Samamra M, Mahamid A, Nseir W. Isr Med Assoc J. 2014 Jun;16(6):367-70.
RESULTS: Mean CRP values were significantly higher in the AA group than the control group (1.1 +/- 0.7 mg/dl vs. 0.4 +/- 0.8 mg/ dl, P < 0.05). Vitamin D levels were significantly decreased in the AA group (11.32 +/- 10.18 ng/ml vs. 21.55 +/- 13.62 ng/ml in the control group, P < 0.05). Multivariate analysis showed that CRP (odds ratio 3.1, 95% confidence interval 2.6-4.2, P = 0.04) and serum vitamin D levels < 30 ng/ml (OR 2.3, 95% CI 2.2-3.1, P = 0.02) were associated with AA.
CONCLUSIONS: We found a significant correlation between AA and vitamin D deficiency. Vitamin D deficiency can be a significant risk factor for AA occurrence.

4. Pulse corticosteroid therapy with oral dexamethasone for the treatment of adult alopecia totalis and universalis JAAD, May 2016  Link.








4 comments:

  1. This is a challenging condition and so difficult to treat. I would recommend starting her on vitamin d3 5,000 a day unless her test shows that she is really low in vitamin d and then I would load with 10,000 a day for a couple of weeks. I know that vitamin d tests are very expensive so treat her empirically. Our own Dr Litt has alopecia total is and miraculously started to regrow hair at age 90 when his vitamin d level was corrected!

    ReplyDelete
  2. From Michael Radowsky, Baltimore, MD "In reply to the virtual grand rounds article on alopecia areata Article at a recent Maryland dermatology conference a patient with near total AA a patient was presented with an dramatic response to Ruxolitinib – Started at the dose of 5 mg twice daily for the first two weeks and then slowly titrate up with two total daily dose of 30 mg.daily.The patient began to have a modest regrowth of hair on the scalp,eyelashes and genitals just one month after starting treatment . After eight months of therapy she was found to have complete regrowth of hair on the scalp and elsewhere in the body.

    ReplyDelete
  3. From Yoon Cohen:
    This is one of the most challenging conditions in Dermatology.
    In my training, I have not seen DCP use, however, squaric acid dibutylester (SADBE) has been frequently used for AA, AT, or AU. We usually sensitize patients with SADBE 5% solution, and prescribe for home treatment based on their skin reaction. Frequently SADBE 1% or 2% are prescribed. We usually recommend once weekly, but if they can tolerate, we can go up to every other day, or even every day. But, mostly the patient seem to tolerate 2-3 times weekly base. Because there is no proven studies in pediatric population, biologics are not used. Sometimes some attendings seem to use pulse dose of predsolone (or prednisone).

    We just had journal review last month on AU on pulse corticosteroid therapy. Dexamethasone 0.1mg/kg/day 2 times weekly. It seems there is no clear guideline for how much corticosteroid and how frequently it should be given for the condition.

    For the eyebrows and eyelashes, we tend to use protopic with variable results.

    It is a tough condition to treat and live with. A lot of times, we are there as a supportive role than really curing the condition as in many conditions.

    Please keep us posted on your patient's progress with DCP treatment.

    ReplyDelete
  4. From Doug Johnson, Honolulu: "I would not hesitate to use CysA 100 bid. Can see growth within 8 weeks but sometimes
    6 mos.
    In my experience, more than half respond.
    After near complete response, transition to sulfasalazine 1500 bid and taper this if response continues."

    ReplyDelete

We welcome your comments. We endeavor to serve your patients and you. If you want us to respond, please add your name and email address. Some people have trouble uploading comments. In that case, please send comments directly to djelpern@gmail.com. Thank you.