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.

Interesting Follow-up: Paronychia in a Child

In October 2007, we presented the case of an eight year old girl with chronic paronychial inflammation located on the left index finger (Paronychia in a Child). She had no other dermatoses. The patient is adopted so we have no family history. We assumed this was some kind of localized psoriasis or acrodermatitis continua. Clobetasol ointment was prescribed which she has used since. (Photo above from 10/2007)

The patient was seen in follow-up recently. The paronycial inflammation had subsided but the finger tip was still abnormal, especially on the palmar surface and there is now hypopigmentation and atrophy distal to the area of inflammation. This latter is likely secondary to the clobetasol. Her topical therapy was switched to calcipotriene cream (the ointment is no longer available in the US.)

Photos:






Questions:
1) What do you think the diagnosis is?
2) Side-effects on the fingers from super-potent topical corticosteroids are rarely reported. One suspects that they are not that unusual. When does the treatment get worse than the disease? (I should have been more diligent in follow-up)
3) Who thinks that these preparations can cause bone changes?
Your comments will be appreciated.

References:
1. Deffer TA, Goette DK.. Distal phalangeal atrophy secondary to topical steroid therapy. Arch Dermatol. 1987 May;123(5):571-2.

2. Tosti A, Fanti PA, Morelli R, Bardazzi F. Psoriasiform acral dermatitis. Report of three cases. Acta Derm Venereol. 1992;72(3):206-7.
Department of Dermatology, University of Bologna, Italy.
The authors report 3 patients affected by psoriasiform acral dermatitis, a distinctive clinical entity characterized by a chronic dermatitis of the terminal phalanges, associated with marked shortening of the nail beds of the affected fingers. The skin biopsy showed in all cases the pathological features of a subacute spongiotic dermatitis. X-ray examination of affected fingers showed no bone or soft tissue changes. Differential diagnosis of psoriasiform acral dermatitis included psoriasis, atopic or contact dermatitis and corticosteroid-induced distal phalangeal atrophy.

3. Brill TJ, Elshorst-Schmidt T, Valesky EM, Kaufmann R, Thaçi D. Successful treatment of acrodermatitis continua of Hallopeau with sequential combination of calcipotriol and tacrolimus ointments. Dermatology. 2005;211(4):351-5.
Department of Dermatology, J.W. Goethe University, Frankfurt, Germany.
Acrodermatitis continua of Hallopeau (ACH) is a rare type of pustular psoriasis affecting the digits. We report on a 43-year-old female patient who had been suffering from ACH for more than 20 years. Despite the fact that the disease was localized on one finger during the whole period, several topical and systemic treatments resulted in only temporary or partial improvement of the lesion. Although the monotherapies with calcipotriol and tacrolimus ointments gave no satisfying results in the long-term management of the disease, the combination of both agents led to a continuous improvement of the patient's skin condition. Copyright 2005 S. Karger AG, Basel.