Correspondence

noma is not ADPA, and should not be confused for the latter condition. M. Asgari and S. Chen Department of Pathology and Laboratory Medicine, Staten Island University Hospital, Staten Island, New York, NY, USA; and Department of Pathology and Laboratory Medicine, Hofstra North Shore-LIJ School of Medicine, Lake Success, NY, USA E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 17 May 2013

References 1 Hsu HC, Ho CY, Chen CH et al. Aggressive digital papillary adenocarcinoma: a review. Clin Exp Dermatol 2010; 35: 113–9. 2 Duke WH, Sherrod TT, Lupton GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). Am J Surg Pathol 2000; 24: 775–84. 3 Rulon DB, Helwig EB. Papillary eccrine adenoma. Arch Dermatol 1977; 113: 596–8.

Alitretinoin: the Nottingham experience doi: 10.1111/ced.12242 We share our experience of using alitretinoin (9-cis-retinoic acid). Alitretinoin has been available in the UK for hand dermatitis since 2008. It has been a welcome addition to the limited treatment options for management of this chronic and challenging condition.1 Morbidity from hand dermatitis is considerable, and can create an economic burden. Topical treatments are difficult to incorporate into everyday life, and phototherapy requires time and transport. Systemic treatments (unlicensed) can be effective, but their side effects limit use. Since 2010, we have treated 66 patients (37 women, 29 men; mean  SD age 55  16.75 years (range 18–

101). The majority of these patients had longstanding disease, with 6 of the 66 patients having a shorter duration (< 12 months). Our data were collected via a review of clinic letters and a pharmacy database. Details of the diagnoses treated are presented in Table 1. We appreciate that alitretinoin is not licensed for use in psoriasis or mycosis fungoides; however, on humanitarian grounds, we decided to trial it in these patients, as they had not responded to other treatments. A Dermatology Life Quality Index (DLQI) score of >15 was documented in 33 patients (50%). The remaining patients had no DLQI documented in the sources of information used. Patch testing was performed in all patients, with positive reactions in 24 patients (36%). Allergen avoidance can take months to be effective, thus alitretinoin was initiated because of the disease severity. Prior to the alitretinoin therapy, 18 patients (27%) had received phototherapy (psoralen ultraviolet A for 16, and TL01 for 2), and 12 (18%) patients had received systemic immunosuppressive medication. All patients had unsatisfactory responses to potent topical steroids. The durations of treatment with alitretinoin are shown in Table 2. Of the 58 patients with different forms of hand eczema, 40 (69%) had an improvement in their disease, 8 (13.7%) had no response, and 10 (17.2%) had an unknown response or stopped treatment due to side effects. On a mechanistic point, 4 of 5 vesicular hand eczemas (80%) and 22 of 26 hyperkeratotic hand eczemas (84.6%) responded to treatment. Of the seven patients with psoriasis we treated, three (49%) had an improvement in disease condition, two had no response and one had an undocumented response. The single patient with treatment-resistant, hyperkeratotic mycosis fungoides had a good response. Relapse of disease upon discontinuation of alitretinoin was documented in 24 patients (36.4%): 7 patients (10.6%) relapsed within 2 weeks of cessation, a further 7 (10.6%) within 2–4 weeks from cessation, a further 7 (10.6%) within 1–3 months from stopping treatment, and the final 3 (4.5%) within 3–6 months from cessation. Table 2 Duration of treatment needed with alitretinoin.

Table 1 Frequency of each diagnosis treated with alitretinoin. Diagnosis

Patients, n

Hyperkeratotic hand and foot eczema Allergic contact dermatitis Chronic hand dermatitis Chronic irritant contact dermatitis Discoid eczema Endogenous eczema Keratoderma climactericum Fingertip dermatitis Hand and foot psoriasis Palmoplantar pustular psoriasis Mycosis fungoides

26 7 5 4 2 8 2 4 4 3 1

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Length of treatment

Patients, n

< 4 weeks < 8 weeks < 12 weeks 12 weeks 12–16 weeks 21–24 weeks 35–36 weeks > 9 months Ongoing No data

4 5 4 20 5 6 4 4 7 7

Range of duration: < 4 weeks to ongoing treatment; mode: 12 weeks; median: 12 weeks.

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Correspondence

There were no relapse data for the remaining 63.6% of our patients. Our data confirm the usefulness of alitretinoin in the management of hand dermatitis. It is a much needed addition to the armentarium of treatments for this condition. However, there was a significant number of patients within the cohort who relapsed quickly upon cessation of treatment, and for these patients, continuous treatment may be necessary.1,2 A. N. Patel, C. I. Wootton, P. Babakinejad, and J. S. C. English Department of Dermatology, Nottingham University Hospitals NHS Trust, Derby Road, Nottingham NG7 2UH, UK E-mail: [email protected] Conflict of interest: JSCE has received payment from Stiefel for chairing scientific symposia. None of the other authors has any conflict of interest to declare. Accepted for publication 5 August 2013

References 1 Lynde C, Cambazard F, Ruzicka T, Sebastian M, Brown TC, Maares J. Extended treatment with oral alitretinoin for patients with chronic hand eczema not fully responding to initial treatment. Clin Exp Dermatol 2012; 37: 712–7. 2 National Institute for Health and Clinical Excellence. Alitretinoin for the treatment of severe chronic hand

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eczema. NICE technology appraisal guidance no. 177. Available at: http://www.nice.org.uk/nicemedia/pdf/ TA177Guidance.pdf (accessed 8 June 2013).

Two cases of Marie Unna hereditary hypotrichosis: clinical features and mutation analysis of the U2HR and EPS8L3 genes doi: 10.1111/ced.12245 Marie Unna hereditary hypotrichosis (MUHH; OMIM 146550) is a rare autosomal dominant hair disorder. This disease was originally described in 1925 in a large German family.1 However, the pathogenic mutation in the U2HR gene was not identified for another 85 years.2 In 2009, Wen et al.3 discovered that MUHH was caused by a loss-of-function mutation in an inhibitory upstream open reading frame in the human hairless (HR) gene. Recently, we identified a missense mutation in the gene EPS8L3 on 1p21.1–1q21.3 in a large Chinese family with MUHH.4 In this paper, we report on two further cases of MUHH from China. The first case was that of a 13-year-old girl, who presented with a scarcity of scalp hair, which had been present since the age of 3 years. Her parents did not have any similar condition. On physical examination, both the

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Figure 1 (a–i) Clinical and scanning electronic microscopy (SEM) features. (a–d) The first patient had extremely sparse, coarse and wiry scalp hair on her frontal and vertex areas, as well as slightly sparse eyebrows and eyelashes, associated with keratosis pilaris and plantar keratoderma. (e) SEM showed an irregularly twisted hair shaft with normal cuticles. (f–h) Patients in the second family had almost complete absence of scalp hair on their frontal and temporal areas, and of their eyelashes, and also had sparse eyebrows. (i) SEM showed that the hair shaft had a normal shape with mild peeling of the cuticles. Original magnification (e) 9 400; (i) 9 1000.

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Alitretinoin: the Nottingham experience.

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