Lelis’ syndrome: treatment with acitretin

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  • International Journal of Dermatology 2008, 47, 000000 2008 The International Society of Dermatology

    1330 Correspondence

    Treatment is not necessary as the hair regrows spontaneously.Therefore, P. pallidula should be kept in mind as a possible

    etiologic agent in all cases of sudden alopecia of the scalpacquired in tropical and subtropical countries.

    Stefano Veraldi, MDLuisa Lunardon, MDClaudia Francia, MDMaria Chiara Persico, MDMauro Barbareschi, MDMilan, Italy

    References1 Radmanesh M, Mousavipour M. Alopecia induced by ants.

    Trans R Soc Trop Med Hyg 1999; 93: 427.2 Shamsadini S. Localized scalp hair shedding caused by

    Pheidole ants and overview of similar case reports. Dermatol Online J 2003; 9: 12.

    3 Aghaei S, Sodaifi M. Circumscribed scalp hair loss following multiple hair-cutter ant invasion. Dermatol Online J 2004; 10: 14.

    4 Mortazavi M, Mansouri P. Ant-induced alopecia: report of 2 cases and review of the literature. Dermatol Online J 2004; 10: 19.

    5 Kapda%li S, Sekin D, Baba M, et al. Localized hair breakage caused by ants. Pediatr Dermatol 2006; 23: 519520.

    6 Marshall J. Alopecia after tick bite. S Afr Med J 1966; 40: 555556.

    7 Heyl T. Tick bite alopecia. Clin Exp Dermatol 1982; 7: 537542.

    8 Sharma AK, Sharma RC, Sharma NL. Diffuse hair loss following multiple honeybee stings. Dermatology 1997; 195: 305.

    Lelis syndrome: treatment with acitretinEctodermal dysplasias (EDs) are inherited disorders charac-terized by abnormalities of the skin derivatives: hair, nails,sweat glands and teeth. There may be additional defects of theinternal organs.1 Lelis reported seven cases of ED associated withacanthosis nigricans;2 Steiner et al.3 and Samdani4 reported onecase each. The patient presented here is the one described bySteiner et al.3 There is no report of treatment of the syndrome.

    A 31-year-old man was referred to the Department ofDermatology for palmoplantar hyperkeratosis with fissures.He had a history of premature loss of the teeth and presentedwith mental retardation, a long narrow face, prognathism, anddivergent squint. He had a strident voice and radial fissures ofthe corners of the mouth, and mentioned suffering fromhypohidrosis. Physical examination also revealed hypotrichosisof the forearms, legs, eyebrows and axillae, hyperconvexity of

    the nails, and papillomatosis nigricans of the neck, axillae, andnose, in addition to hyperkeratosis. The tongue was normal.

    Biopsy of the dorsum of the nose revealed characteristics ofacanthosis nigricans. Other genodermatoses were excluded.3

    Treatment with acitretin, 0.5 mg/kg/day, was started,with marked improvement of the hyperkeratosis (Fig. 1) andacanthosis nigricans, as well as facial comedones (Fig. 2).After 3 months, the dose was reduced to 0.3 mg/kg/day,maintaining good clinical results, with the complete satisfactionof the patient.

    DiscussionAlthough exact incidence data are lacking, it is estimated thatabout seven in 10,000 births are affected with a form of ED.1

    Lelis syndrome is a rare ED in which hypotrichosis,hypohidrosis, and acanthosis nigricans are always present.

    Figure 1 Palm of the right hand of the patient before (a) and after (b) 1 year of low-dose acitretin treatment

    International Journal of Dermatology 2008, 47, 13301331 2008 The International Society of Dermatology

  • 2008 The International Society of Dermatology International Journal of Dermatology 2008, 47, 000000

    1331Correspondence

    Hypodontia, palmoplantar hyperkeratosis, nail dystrophy, andfurrowed tongue occur in more than 50% of cases. Mental retar-dation and perioral radial furrows are present in less than 50%.3

    Acitretin is the drug of choice for the management of severekeratinization disorders. Its main effect is the decrease inscaling and hyperkeratosis. Mashshini-Jason et al.5 reportedthe use of acitretin in a patient who presented with hidrotic ED(with palmoplantar hyperkeratosis, anodontia, and nail and hairdystrophies). The initial dose of 0.5 mg/kg/day was reducedto 0.3 mg/kg/day after the improvement of hyperkeratosis.

    In the case presented here, the symptoms which led thepatient to seek medical attention were markedly reduced ontreatment with acitretin, with an improvement in the patientsquality of life. No abnormalities of blood count, lipid, orhepatic parameters were observed. It is expected that the drugwill be used for a long period in this case; consequently, thebenefits and risks of use must be constantly evaluated. Thedose of medication was reduced to a maintenance dose withthe complete satisfaction of the patient. This is the first reportof the treatment of a patient with Lelis syndrome.

    Andrea Miyuki Yoshimura, MD Paulo Eduardo Neves Ferreira Velho, MD, PhD Renata Ferreira Magalhes, MD Elemir Macedo de Souza, MD, PhDCampinas, SP, Brazil

    References1 Itin PH, Fistarol SK. Ectodermal dysplasias. Am J Med Genet

    2004; 131: 4551.2 Lelis J. Autosomal recessive ectodermal dysplasia. Cutis 1992;

    49: 435437.3 Steiner CE, Cintra ML, Marques-de-Faria AP. Ectodermal

    dysplasia with acanthosis nigricans (Lelis syndrome). Am J Med Genet 2002; 113: 381384.

    4 Samdani AJ. Ectodermal dysplasia with acanthosis nigricans (Lelis syndrome). J Coll Physicians Surg Pak 2004; 14: 626627.

    5 Mashshini-Jason F, Wolff P, Pirard GE, et al. Anodontie et displasie ectodermique hidrotique, hyporactive la chaleur. Effet de l acitrtine. Ann Dermatol Venereol 1994; 121: 120122.

    CASE REPORT

    Acquired unilateral nevoid telangiectasia in a 51-year-old femaleUnilateral nevoid telangiectasia (UNT) is a rare, cutaneousdisorder that was first described by Blaschko in 1899.1 UNTpresents as confluent telangiectases with a linear patternand a unilateral distribution and can be either acquired or con-genital. Some authors propose that elevated estrogen levelscause this disorder, but the exact pathogenesis is unknown.13

    A 51-year-old white female presented with mildly pruriticlesions involving the right forearm, upper arm and upper chest.The lesions began after her first pregnancy 15 years earlier,and slowly expanded. She had no history of liver disease andher medications included quinapril, sertraline, potassium

    citrate and a daily multivitamin. On examination, the patienthad numerous discrete and coalescing, nonblanchable,violaceous, telangiectatic patches with scattered, pinpoint,erythematous, smooth papules (Fig. 1). The differential diagnosisincluded angioma serpiginosum and UNT. Histopathologyrevealed thin, dilated capillaries in the papillary dermischaracteristic of UNT (Fig. 2). Angioma serpiginosum, con-versely, would have revealed dilated capillaries with thickwalls found within the dermal papillae.

    UNT may be congenital or acquired.14 The rare congenitalform occurs more frequently in males, appears during orshortly after the neonatal period, and is inherited in an auto-somal dominant fashion.2 The acquired form occurs almost

    Figure 2 Left malar region of the patient before (a) and after (b) 1 year of low-dose acitretin treatment

    2008 The International Society of Dermatology International Journal of Dermatology 2008, 47, 13311333

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