Letters to Editor keratinocyte differentiation, it is possible that acitretin may Acne is the most common cutaneous association of Apert’s inhibit viral replication and assembly within the affected syndrome. cells.[3] Oral retinoids have been used with remarkable improvement in the management of extensive and An eight-month-old boy born of nonconsanguineous recalcitrant warts without recurrences.[3,4] parents presented with multiple congenital anomalies. The mother’s antenatal period was uneventful, with no history Epidermodysplasia verruciformis, an inherited disorder of any infectious illness, medication(s) exposure or addiction characterized by widespread and persistent infection with during that period. Family history was non-contributory. HPV, pityriasis versicolor-like lesions and reddish plaques, The patient’s anomalies included craniofacial abnormality has shown dramatic improvement with oral acitretin when and of all four limbs. The child had a typical used alone or in combination.[5] Our patient tolerated the facial appearance with a short, wide head (), oral acitretin well with gratifying clinical results without any frontal bossing, retruded (sunken) mid-face, depressed nasal major side effects or recurrences. Hence, oral acitretin can bridge, upward slanting of the eyes, low-set ears and a low be considered as a useful treatment option for extensive posterior hairline [Figure 1]. Examination of the upper and recalcitrant warts. lower limbs showed symmetrical syndactyly leading to the fusion of all the fingers and toes [Figure 2]. The child had DD.. SS.. KKruparupa SShankar,hankar, RRachanaachana ShilpakarShilpakar both growth and mental retardation. Department of Dermatology, Manipal Hospital, Bangalore, India Apert’s syndrome is specifically related to the paternal AAddressddress fforor ccorrespondence:orrespondence: Dr. D. S. Krupa Shankar, Head of Department, Department of Dermatology, Manipal Hospital, 98, age effect having a frequency of one in every 65,000 live Rustom Bagh, Airport Road, Bangalore 560017, India, Email: [email protected]

RREFERENCESEFERENCES

1. Laurent R, Kienzler JL. Epedemilology of HPV infection. Clin Dermatol 1985;3:64-70. 2. Massing AM, Epstein WL. Natural history of warts. A two year study. Arch Dermatol 1963;87:306-10. 3. Choi YL, Lee KJ, Kim WS, Lee DY, Lee JH, Lee ES, et al. Treatment of extensive and recalcitrant viral warts with acitretin. Int J Dermatol 2006;45:480-2. 4. Gelmetti C, Cerri D, Schiuma AA, Menni S. 20 children with extensive warts were given Etretinate 1mg/kg/day for 3months. Pediatr Dermatol 1987;4:254-8. 5. Anadolu R, Oskay T, Erdem C, Boyvat A, Terzi E, Gurgey E. Treatment of epidermodysplasia verruciformis with a Figure 1: Typical facial dysmorphosis combinationof acitretin and interferon alfa-2a. J Am Acad Dermatol 2001;45:296-9.

BBrachycephalyrachycephaly andand syndactyly:syndactyly: AApert’spert’s ssyndromeyndrome

Sir, Apert’s syndrome (acrocephalosyndactyly type 1) is a rare malformation syndrome first described by Wheaton in 1894, and later by Apert in 1906.[1] It is characterized by associated with maxillary hypoplasia, symmetric syndactyly of the hands and feet, and other systemic malformations including mental retardation. Figure 2: Deformities in feet

Indian J Dermatol Venereol Leprol | July-August 2008 | Vol 74 | Issue 4 395 Letters to Editor births; the affected patients generally do not survive AAddressddress fforor ccorrespondence:orrespondence: Prof. Amrinder J. Kanwar, Department [1] of Dermatology, Postgraduate Institute of Medica l Education and beyond infancy. In those patients who reach adolescence, Research, Chandigarh-160 012, India. acne is an important cutaneous manifestation. Most cases E-mail: [email protected] are sporadic, although autosomal dominant inheritance as well as germinal mosaicism have been reported.[2] The RREFERENCESEFERENCES syndrome is caused by nucleotide alterations resulting in amino acid substitutions, leading to a mutation in the 1. Cohen MM Jr, Kreiborg S, Lammer EJ, Cordero JF, fibroblast growth factor receptor-2 (FGFR-2) gene mapped Mastroiakovo P, Ericson JD, et al. Birth prevalence study of to chromosome 10q26.[3] Premature fusion of the bones is the . Am J Med Genet 1992;42:655-9. 2. Allanson JE. Germinal mosaicism in Apert syndrome. Clin responsible for bony abnormalities; premature fusion of Genet 1986;29:429-33. coronal sutures lead to brachycephaly. Other craniofacial 3. Hollway GE, Suthers GK, Haan EA, Thompson E, David DJ, abnormalities include a prominent forehead with skin Gecz J, et al. Mutation detection in FGFR2 craniosynostosis wrinkling, a broad cranium, and a flat occiput. The syndromes. Hum Genet 1997;99:251-5. shortened bony orbit leads to , proptosis 4. Munro CS, Wilkie AO. Epidermal mosaicism producing and strabismus. Additional features include a short broad localized acne: somatic mutation in FGFR2. Lancet nose with a bulbous tip, micrognathia, and a cleft palate. 1998;352:704-5. Intracranial anomalies include megalocephaly, progressive hydrocephalus, hypoplastic white matter, and agenesis of the corpus callosum and limbic structures, leading to HHydroxychloroquineydroxychloroquine versusversus cognitive impairment. Cardiac abnormalities including atrial and ventricular septal defects and renal anomalies such as cchloroquinehloroquine inin polymorphicpolymorphic lightlight hydronephrosis occur in about 10% of these patients. eeruptionruption The cutaneous abnormalities reported are acne, hyperhidrosis and oculocutaneous albinism. Acne is now Sir, known to represent the dermatological hallmark of Apert’s We read the article titled “Comparative study of efficacy and syndrome; it usually appears between the age of nine and safety of hydroxychloroquine and chloroquine in polymorphic 12 years. The acneiform lesions involve not only the face light eruption: A randomized, double-blind, multicentric and upper trunk, but also affect the forearms, buttocks, and study” with great interest. However, we wish to point out thighs. Oily skin is striking by the time of adolescence but the the following incongruities observed in this study. etiology of these acneiform lesions remains controversial. Increases in circulatory androgens or the density of sebaceous The conclusion drawn about superior efficacy and tolerability gland androgen receptors have not been documented. End- of hydroxychloroquine over chloroquine is not corroborated organ androgen metabolism defects, structural malformation by the findings of the study, where only a marginal difference of pilosebaceous apparatus and a role of FGFR-2 in regulating was observed in the efficacy of the two drugs, and in fact, androgen sensitivity of the pilosebaceous unit have been chloroquine fared better in the side effect profile.[1] suggested hypotheses for the acneiform lesions.[4] The skin, eyes and hair may show pigmentary dilution. Other Negative serological tests for light eruption (LE) are occasional cutaneous abnormalities include interrupted pertinent and desirable for the diagnosis of polymorphic eyebrows, forehead wrinkling, paronychial infections, skin light eruption (PLE) patients as PLE and LE may often coexist dimpling over the knuckles, and elbows, as well and PLE precedes LE in a subset of the patients.[2,3] as lateral plantar hyperkeratosis.

The lack of ocular toxicity observed in the hydroxychloroquine There is no definitive treatment for this syndrome. The treatment group after a short course of therapy is prognosis varies from child to child and a multidisciplinary understandable as the same is expected after long-term approach is recommended. use only. The important risk factors for ocular toxicity associated with hydroxychloroquine therapy are: excessive DDipankaripankar De,De, TarunTarun Narang,Narang, AmrinderAmrinder J Kanwar,Kanwar, daily dosage, increasing cumulative dosage, the duration of SSunilunil DDograogra Department of Dermatology, Postgraduate Institute of Medical treatment and the patient’s age as well as coexistent renal Education and Research, Chandigarh-160 012, India or liver disease and concomitant retinal disease.[4,5]

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