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Home , Acanthosis nigricans, Crouzon syndrome, Piebaldism

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case of female. Ambras syndrome is a type of universal Generalized hypertrichosis affecting the vellus hair, where there is uniform overgrowth of hair over the and external hypertrichosis ear with or without dysmorphic facies.[3] Patients with Gingival fi bromaatosis also have generalized hypertrichosis Sir, especially on the face.[4] Congenital hypertrichosis can A 4-year-old girl born out of non-consanguinous marriage occur due to fetal alcohol syndrome and fetal hydentoin presented with generalized increase in body hair noticed syndrome.[5] Prepubertal hypertrichosis is seen in otherwise since birth. None of the other family members were healthy infants and children. There is involvement of affected. Hair was pigmented and soft suggesting vellus hair. face back and extremities Distribution of hair shows an There was generalized increase in body hair predominantly inverted fi r-tree pattern on the back. More commonly seen affecting the back of trunk and legs [Figures 1 and 2]. in Mediterranean and South Asian descendants.[6] There is Face was relatively spared except for fore head. Palms and soles were spared. Scalp hair was normal. Teeth and nail usually no hormonal alterations. Various genodermatosis were normal. There was no gingival hypertrophy. No other associated with hypertrichosis as the main or secondary skeletal or systemic abnormalities were detected clinically. diagnostic symptom are: Routine blood investigations were normal. Hormonal  Lipoatrophy (Lawrernce Seip syndrome) study was within normal limit for her age. With this  Cornelia de Lange syndrome clinical picture of generalized hypertrichosis with no other  Craniofacial associated anomalies a diagnosis of universal hypertrichosis  Winchester syndrome was made.  Rubinstein–Taybi syndrome  Mucopolysaccharidoses Hypertrichosis is the growth of hair of an excessive amount  Dystrophic epidermolysis bullosa and thickness on any part of the body.[1] Generalized  Porphyrias hypertrichosis may be congenital or acquired. Congenital  Ostheochondrodysplasia generalized affecting the lanugo hair is a rare  Gingival fi bromatosis autosomal dominant disease, where soft nonpigmented  Globoid leukodystrophy(Krabbe disease) nonmedullated lanugo hair covers the whole body except  palms and soles and is not replaced by terminal hair after  birth.[2] Lanugo hair can grow up to 10 cm in length.  Hammerschlag-Telfer syndrome In some cases, it may acquire terminal characteristics.[2]  Dystrichiasis-lymphedema syndrome Associated dental anomalies are reported. Generalized  Oliver-MacFarlane syndrome hypertrichosis of vellus or terminal hair can occur as sole  achromians of Ito feature in universal hypertrichosis.[1] in which normally hairy areas like , back, and limbs show increase in Ambika Hariharasubramony, Sujatha Chankramath thickness and density of hair. Some people do not consider Department of and Paediatrics, M.V.J. Medical College it as pathological. But it is psychogically disturbing in and Research Hospital, Hoskote, Bangalore, Karnataka, India

Figure 1: Hypertrichosis of arms Figure 2: Back showing inverted fi r-tree pattern

Indian Journal of Endocrinology and Metabolism / Nov-Dec 2012 / Vol 16 | Issue 6 1057 Letters to the Editor

Corresponding Author: Dr. Ambika H, Department of Dermatology, MVJMC and RH Hoskote, Bangalore, Karnataka, India. E-mail: [email protected] REFERENCES

1. Wendelin DS, Pope DN, Mallory SB. Hypertrichosis. J Am Acad Dermatol 2003;48:161-79; quiz 180-1. 2. Demikova NS, Blinnikova OE, Udler EE. [A case of congenital generalized hypertrichosis]. Klin Med (Mosk) 1986;64:125-6. 3. Belengeanu V, Rozsnyai K, Gug C, Bănăţeanu M, M, Farcaş S, Belengeanu A. Ambras syndrome: Report on two affected siblings with no prior family history. Clin Dysmorphol 2004;13:265-7. 4. Stoll C, Audeoud F, Gaugler C, Bernardin A, Messer J. Multiple congenital malformations including generalized hypertrichosis with gum hypertrophy in a child exposed to valproic acid in utero. Genet Couns 2003;14:289-98. 5. Guevara-Sanginés E, Villalobos A, Vega-Memije ME, Figure 1: seen on Mosqueda-Taylor A, Canún-Serrano S, Lacy-Niebla RM. Congenital generalized terminal hypertrichosis with gingival hyperplasia. Pediatr Dermatol 2002;19:114-8. . The common sites involved are posterior and lateral 6. Trüeb RM, Borelli S, Gloor M, Wüthrich B. [Prepubertal [1] hypertrichosis]. Schweiz Med Wochenschr 1994;124:595-600. folds of , , umbilicus, and groin. Forehead, oral mucosa, and eyelids are rarely involved. It may be mistaken

Access this article online with giant melanocytic nevus, atrophic seborrheic dermatitis, Quick Response Code: hemochromatosis, or atopic dermatitis. AN is associated with Website: resistance states like type 2 , polycystic ovarian www.ijem.in syndrome, and . Crouzon’s syndrome and Costello’s syndrome are also associated with AN. Severe DOI: with developmental delays and AN (SADDAN) have been 10.4103/2230-8210.103046 documented.[2] As in our case, achondroplasia without the presence of developmental delays and AN have also been reported. AN is a paraneoplastic manifestation of internal malignancies of stomach, gut, ovary, prostate, and Atypical presentation of others. AN is a clinical diagnosis only rarely requiring a skin biopsy. However, a meticulous search for underlying disease acanthosis nigricans and malignancy is required. Treatment of AN involves treatment of the underlying disease or tumor, avoidance of Sir, precipitating drugs like , steroids, protease inhibitors, We document one case of acanthosis nigricans (AN) in and use of topical keratinolytics, oral isotretinoin, metformin, an atypical site. and ultimately, cosmetic surgery.[3]

The patient was a 48-year-old achondroplastic, diabetic Nirmalya Roy, Tanima Das1, Arup K. Kundu, male, school teacher by profession with a body mass index Anupam Maity1 2 (BMI) of 26 kg/m . His clinical examination revealed AN Department of Medicine, KPC Medical College, over the nape of the neck, axilla, and forehead [Figure 1]. 1R.G. Kar Medical College, Kolkata, West Bengal, India He had normal developmental milestones, and other systemic examinations were essentially within normal Corresponding Author: Dr. Nirmalya Roy, 3/H Dover Terrace, Kolkata-700 019, India. limits. Laboratory investigations revealed glycosylated E-mail: [email protected] hemoglobin of 8.6% and dyslipidemia (total cholesterol level of 375 mg/dl, low density lipoprotein level of REFERENCES 195 mg/dl, triglyceride level of 180 mg/dl, and high density lipoprotein level of 36 mg/dl). There was no 1. Kundu AK. Acanthosis nigricans revisited. J Assoc Physicians India evidence of internal malignancy in chest radiography, 2001;49:1003-6. abdominal ultrasound, upper gastrointestinal , 2. Vajo Z, Francomano CA, Wilkin DJ. The molecular and genetic basis of fibroblast growth factor 3 disorders: The achondroplasia and colonoscopy. family of skeletal dysplasias, Muenke , and with acanthosis nigricans. Endocr Rev 2000;21:23-39. AN is a brown-to-black velvety of 3. Walling HW, Messingham M, Myers LM, Mason CL, Strauss JS.

1058 Indian Journal of Endocrinology and Metabolism / Nov-Dec 2012 / Vol 16 | Issue 6 Copyright of Indian Journal of Endocrinology & Metabolism is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.