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.

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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.

Alopecia Areata and Twenty Nail Distrophy

Abstract: 18 year old man with two year history of alopecia areata and six month history of dry lusterless nails.

HPI:  The patient is an 18 year-old college student who has had alopecia areata for the past 2 years.  He is well otherwise and has had no other problems until a number of months ago when he developed a nail dystrophy.   He takes no medications by mouth.  There is no family history of alopecia or autoimmune problems.  Treatment to date for alopecia has been intralesional triamcinlone with regrowth, however, new areas continue to evolve.

O/E:  There are 6 - 7 alopecic areas measuring from 2 to 6 cm in diameter widely scattered over the scalp.  He has some areas of alopepcia on his abdomen.
19 of his nails are dystrophic.  They are lusterless and many are greyish white in color, rough and friable.  One of his toenails appears normal

Clinical Photos:

Lab: All studies have been normal.  Records of these have been requested.

Diagnosis:  Alopecia areata and trachyonychia (aka 20 Nail Dystrophy)

Discussion and Questions: The patient has read about immunotherapy with DNCB and related chemicals.  Has anyone treated a patient with this modality who experienced long-lasting remission?  Do you know of good treatments for his nail dystrophy?

References: 
Alopecia universalis with twenty-nail dystrophy (trachyonychia).

Chien P Jr, Kovich OI.

Dermatol Online J. 2008 May 15;14(5):24.

Department of Dermatology, New York University, USA.

Abstract

A 43-year-old man presented with long-standing trachyonychia of all 20 nails, which worsened after the onset of alopecia universalis 18 months ago. Trachyonychia can be associated with alopecia universalis although the treatment strategies of both conditions differ. The principle of treating trachyonychia may involve regulating the differentiation of keratinocytes and/or reducing inflammation in the nail fold or nail matrix while treatment of alopecia universalis involves immunomodulation.

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Indian J Dermatol Venereol Leprol. 2011 Nov-Dec;77(6):640-5.

Trachyonychia: a comprehensive review.

Gordon KA, Vega JM, Tosti A.

Source

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida 33136, USA. kagordon@med.miami.edu

Abstract

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.  Available full text

Vañó-Galván S, et. al. Sudden hair loss associated with trachyonychia. Cleve Clin J Med. 2008 Aug;75(8):567-8
Department of Dermatology, Ramón y Cajal Hospital, University of Alcalá, Madrid, Spain. sergiovano@yahoo.es  Free Full Text

UTILITY OF DERMOSCOPY IN ALOPECIA AREATA

Mandar Mane, Amiya Kumar Nath, and Devinder Mohan Thappa

Indian J Dermatol. 2011 Jul-Aug; 56(4): 407–411  F  Free Full Text

Twenty-nail dystrophy of alopecia areata.

Horn RT Jr, Odom RB.

Arch Dermatol. 1980 May;116(5):573-4

Abstract

We describe here a patient with dystrophy of all 20 nails, which has persisted for five years after the resolution of alopecia areata. We feel that the term "20-nail dystrophy" is best used to describe a clinical entity that can have several causes.

Alopecia Universalis

HPI: The patient is a 77-year-old woman who was seen for alopecia, which has been present for about eight months now. This followed chemotherapy for nonhodgkin’s lymphoma. She has a history of alopecia areata decades ago which resolved on its own.

O/E: The examination shows that this patient has alopecia universalis. She has a few eyelashes but no eyebrows, no body hair, no scalp hair.

DX: Alopecia universalis following chemotherapy. This is unusual. There is one report of alopecia universalis following treatment for hepatitis C with ribavirin and interferon.

PLAN: I am going to get a list from her of the medications she was treated with for NHL and see if there are any reports on this. I will also run this be some colleagues.

Question: Has anyone seen a similar patient?

References: