Chronic Cutaneous Disorders In Down Syndrome Patients

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Abstract

Background: Down syndrome is an extensively studied chromosomal disorder characterized by mental retardation and distinct physical manifestations, and it is the second most common cause of mental retardation in Iraqi children after idiopathic mental retardation. However, the chronic cutaneous conditions associated with the syndrome have received relatively inadequate attention. The aim of this paper is to determine chronic cutaneous disorders persisting more than six months or reoccurring over six months in Down syndrome patients

Introduction

Down syndrome (Trisomy 21) was first described by Jean-Etienne-Dominique Esquirol in 1838, and later by Edouard Séguin in 1846. However, the disorder was named after John Langdon Down, a British physician who emphasized that the syndrome is a distinct form of mental retardation in 1862. Down syndrome was recognized as a chromosome 21 trisomy by Dr Jérôme Lejeune in 1959, and the condition became known as trisomy 21 [1].

Down syndrome is an extensively studied chromosomal disorder characterized by mental retardation and distinct physical manifestations, and it is the second most common cause of mental retardation in Iraqi children However, the chronic cutaneous conditions associated with the syndrome have received relatively inadequate attention [2, 3]. The aim of this paper is to determine chronic cutaneous disorders persisting more than six months or reoccurring over six month in Down syndrome patients

Patients and methods

Twenty seven patients (17 males and 10 females) with Down syndrome were observed at the Children Teaching Hospital of Baghdad medical City and the Medical Consultation Clinic of Iraq headquarter of Copernicus Scientists international panel in Baghdad during 2018 and 2019. Their ages when they were first seen ranged from 4 months to 30 years. Thirteen patients were seen during infancy and toddlerhood. Four patients were first seen during early childhood before the age of five years. Two patients had atrial septal defect including a female infant and a boy who had secondary tricuspid regurgitation. One female infant had and evidence of hepatic hemangioma on ultrasound. One infant developed transient minor skin rash during infancy and two patients had prominent synophrys including a female infant and a boy. One four-year old boy had undescended testes.

Results

Chronic cutaneous disorders were observed in four patients of the twenty seven patients observed. Three patients including a thirteen-year old girl and two boys had alopecia areata and a man aged thirty years had familial baldness and Tinea corporis of the dorsum of the right hand.

Discussion

The association of alopecia areata which is an autoimmune disorder with Down syndrome has been suggested during the 1960s [4], and confirmed during the 1970s and 1980s [5-10].

Du and Munro (1974) studied 1000 cases (560 males and 440 females) of Down syndrome aged from 5 to 67 years with average age in males of 30 years and in females 33 years). Alopecia areata was found in 60(37 males and 23 females) cases. Du and Munro also reported that only one case of alopecia areata was found amongst 1000 subnormal controls [5]. Carter and Jegasothy (1976) reported that 19 cases of alopecia areata and four cases of vitiligo were found in 214 institutionalized patients with the Down syndrome [6]. Colomb, Vittori, and Zonca reported four Down syndrome patients who developed alopecia areata [7]. Doutre et al (1978) reported two Down syndrome patients who had alopecia areata. One patient also had Hashimoto’s thyroiditis and the second patient had vitiligo [9]. McCulloch, Ince, and Kendall-Taylor (1982) reported a 29-year-old man with Down syndrome who developed alopecia areata and aautoimmune chronic active hepatitis [10]. Hatamochi and Ueki (1984) reported the successful treatment of alopecia areata with dinitrochlorobenzene in a patient with Down syndrome [11]. Scotson (1989) reported a patient with Down syndrome and alopecia who also had mild hypothyroidism [12].

Storm (2000) from Germany reported a 9-year-old girl with Down syndrome, and alopecia areata and developed celiac disease [13]. Dourmishev et al (2000) emphasized the association of Down syndrome with various cutaneous manifestations including atopic dermatitis, alopecia areata, elastosis perforans serpiginosa, syringomas, and skin infections. Dourmishev et al reported an 8-year male patient with Down syndrome who developed alopecia areata [14]. However, a study of alopecia areata in Singapore suggested that the association with Down syndrome [15].

Schepis, Siragusa, and Happle (2017) reported the occurrence of psoriasis and alopecia areata in a Down syndrome patient [16].

Tinea corporis is also called ringworm, is a skin infection that may occur on any part of the body and typically causes a red, itchy, scaly, circular lesion. The condition is caused by various species of dermatophytes including Trichophyton and Microsporum [17]. Tinea corporis which was seen in one patient in this study has not been noticeably highlighted in the previous literature.

Conclusion

This paper highlights the association between Down syndrome and Tinea corporis which has not been noticeably emphasized in the medical literature.

References

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  2. Al-Mosawi AJ. The etiology of mental retardation in Iraqi children. SunKrist Journal of Neonatology and Pediatrics.2019; 1(1):1-9.
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  4. Wunderlich C, Braun-falco O. Mongolism and alopecia areata. Med Welt 1965 Mar 6; 10:477-81. PMID: 14276234[Article in German].
  5. Du Vivier A, Munro DD. Alopecia areata and mongolism. Proc R Soc Med. 1974 Jun; 67(7):596-7. PMID: 4277355.
  6. Carter DM, Jegasothy BV. Alopecia areata and Down syndrome. Arch Dermatol 1976 Oct; 112(10):1397-9. PMID: 134671.
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  9. Doutre MS, Ortonne JP, Floret D, Thivolet J. Alopecia areata and trisomy 21. About 2 cases. Ann Dermatol Venereol 1978 Jun-Jul; 105(6-7):587-90. PMID: 153723 [Article in French].
  10. McCulloch AJ, Ince PG, Kendall-Taylor P. Autoimmune chronic active hepatitis in Down’s syndrome. J Med Genet. 1982 Jun; 19(3):232-4. PMID: 6213775.
  11. Hatamochi A, Ueki H. Successful treatment of alopecia areata with dinitrochlorobenzene in a patient with Down’s syndrome. J Dermatol. 1984 Apr; 11(2):191-3.
  12. Scotson J. A patient with Down’s syndrome, mild hypothyroidism and alopecia. Practitioner. 1989 Feb 8; 233(1462):121.
  13. Storm W. Celiac disease and alopecia areata in a child with Down’s syndrome. J Intellect Disabil Res 2000 Oct; 44 (Pt 5):621-3. PMID: 11079358. PMID: 11138359.
  14. Dourmishev A, Miteva L, Mitev V, Pramatarov K, Schwartz RA. Cutaneous aspects of Down syndrome. Cutis 2000 Dec; 66(6):420-4.
  15. Tan E, Tay YK, Giam YC. A clinical study of childhood alopecia areata in Singapore. Pediatr Dermatol 2002 Jul-Aug; 19(4):298-301. PMID: 12220271.
  16. Schepis C, Siragusa M, Happle R. Psoriasis and alopecia areata in a Down syndrome patient: a Renbök phenomenon. Eur J Dermatol 2017 Jun 1; 27(3):300-301. PMID: 28251896.
  17. Antuori A, Fernández G, Fernández A, Alcaide M, Boada A, Bielsa MI, Romaní N, Matas L. Epidemiology of dermatophytic infections between 2008 and 2017 in Barcelona, Spain. Enferm Infecc Microbiol Clin. 2019 Dec; 37(10):642-647. PMID: 31076176.

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