Letters to the Editor and liver manifestations. Retinoids are useful in the and pitryiasis rubra pilaris. We describe a four-year- treatment of skin and muscle manifestations.[2] old girl with Down’s syndrome, with segmental and lichen nitidus. Tzanck smear may provide important information in a patient with suspected DCS. A four-year-old female child with Down’s syndrome [Figure 1] presented to us with white patches on the MMuraturat DDurdu,urdu, FahrettinFahrettin AkayAkay1, TTevevÞ k AAlperlper2, right waist and inguinal area for the last one month. SSerkanerkan YaYaşarar ÇÇelikelik3 She also had asymptomatic on the legs for Başkent University Faculty of Medicine, Department of , the same duration. She did not have any systemic Adana Hospital, Adana/Turkey, Department of 1Ophthalmology, complaints. On examination, she had segmental vitiligo 2Internal Medicine, 3Pathology, Diyarbakõr Military Hospital, [Figure 2] on the right waist. The vitiliginous macule Diyarbakõr, Turkey had an irregular border and leucotrichia. She also had AAddressddress forfor correspondencecorrespondence: Dr. Murat Durdu, multiple, discrete, flat, round, smooth, skin-colored- Başkent University Faculty of Medicine, to-slightly pink papules of 1 to 2 mm size, distributed Department of Dermatology, Adana Hospital, 01250, symmetrically over the extensors of both the legs and Adana/Turkey. E-mail: [email protected] thighs [Figure 3]. The rest of the skin, hair, and nails DOI: 10.4103/0378-6323.57737 - PMID: 19915256 were normal. Manifestations of Down’s Syndrome RREFERENCESEFERENCES in our case were hypertelorism, depressed nose, epicanthal folds, high-arched palate, flat occiput, low- 1. Ben Selma Z, Yilmaz S, Schischmanoff PO, Blom A, Ozogul C, set ears, hypotonia, sandle toe, flat feet, clinodactyly, Laroche L, et al. A novel S115G mutation of CGI-58 in a Turkish and delayed milestones. Histopathology of the papules patient with Dorfman-Chanarin syndrome. J Invest Dermatol 2007;127:2273-6. on the leg showed features consistent with lichen 2. Düzovali O, Ikizoğlu G, Turhan AH, Yilgör E. Dorfman- nitidus [Figure 4]. It showed orthokeratosis, focal Chanarin syndrome: a case with hyperlipidemia. Turk J Pediatr 2006;48:263-5. parakeratosis, and thinning of the just below 3. Peña-Penabad C, Almagro M, Martínez W, García-Silva J, Del the parakeratosis. There was no hypergranulosis, Pozo J, Yebra MT, et al. Dorfman-Chanarin syndrome (neutral focal infiltrate of lymphocytes, histiocytes in the lipid storage disease): new clinical features. Br J Dermatol 2001;144:430-2. papilla engulfed by the rete ridges, or perivascular 4. Wollenberg A, Geiger E, Schaller M, Wolff H. Dorfman- lymphocytic infiltration. The rest of the dermis and Chanarin syndrome in a Turkish kindred: conductor diagnosis subcutaneous tissue was normal. Echocardiography, requires analysis of multiple eosinophils. Acta Derm Venereol 2000;80:39-43. ophthalmological evaluation, and the biochemical and 5. Durdu M, Baba M, Seçkin D. The value of Tzanck smear test hematological parameters were within normal limits. in diagnosis of erosive, vesicular, bullous and pustular skin lesions. J Am Acad Dermatol 2008;59:958-64. There are many reports to show the association of vitiligo with Down’s syndrome.[3,4] Our case was associated with segmental vitiligo and lichen nitidus. DDown’sown’s syndromesyndrome withwith lichenlichen In literature, there are only two case reports that show the association of lichen nitidus with Down’s nnitidusitidus aandnd segmentalsegmental vitiligovitiligo syndrome.[5,6] Lichen nitidus was generalized in both the cases; one of these cases[6} was also associated with megacolon. Lichen nitidus,[7] a disorder of unknown Sir, etiology, is composed of multiple, discrete, smooth, Down’s syndrome (trisomy 21) is a multisystem flat, round, flesh-colored to slightly-pink papules. The disorder with a birth incidence of approximately 1 in lesions may occur anywhere over the skin; however, 700 live births, making it the most common autosomal the most frequent sites of predilection are the flexural chromosomal disorder causing mental retardation. surfaces of the arms and wrists, lower abdomen, breast, Down’s Syndrome is also associated with an increased the glans, and shaft of the penis. However, in our case, incidence of cutaneous disorders.[1-4] In particular, the lesions were confined to the legs and thighs only. there seems to be an increased incidence of xerosis, Lichen nitidus is usually asymptomatic; however, , seborrheic dermatitis, cutaneous pruritus is occasionally present. The histopathology of infections, , vitiligo, syringomas, lichen nitidus is usually distinctive. , elastosis perforans, keratoderma palmaris et plantaris, lichen spinulosa, lichen scrofulosorum, and verruca

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Figure 3: Discrete papules on the legs and thighs

Figure 1: Facies of the child with Down’s Syndrome

Figure 4: Circumscribed lymphocytic and histiocytic infi ltrate in the papillary dermis engulfed by rete ridges situated directly beneath the thinned epidermis with central parakeratosis, loss Figure 2: Depigmented macules (segmental vitiligo) on the right of granular layer, (H and E, x10) waist and inguinal area plana can be considered in the differential diagnosis of with Down’s syndrome to be associated with other lichen nitidus. Keratosis pilaris and lichen spinulosa dermatological disorders. usually exhibit more keratotic and scaling lesions, unlike the smoother surfaced papules of lichen SSaurabhaurabh Agarwal,Agarwal, VishalVishal GuglaniGuglani1, BBinayinay KKumarumar2 nitidus. Verruca plana are more variable in size, have Departments of Dermatology & Venereology, 1Pediatrics, 2Pathology, a more verrucous surface, and are less likely to involve Uttarakhand Forest Hospital Trust Medical College, Haldwani, Nainital - 263 169, Uttarakhand, India multiple sites. The lesions in lichen scrofulosorum are perifollicular, grouped, keratotic papules, which occur AAddressddress forfor correspondencecorrespondence: Dr. Saurabh Agarwal, in young patients with tuberculosis. Department of Dermatology & Venereology UFHT Medical College, Haldwani, Nainital - 263 139, Uttarakhand, India. Our patient was unique, in that she showed the E-mail: [email protected] association of segmental vitiligo and lichen nitidus DOI: 10.4103/0378-6323.57738 - PMID: 19915257 with Down’s syndrome. While it is not unusual to RREFERENCESEFERENCES encounter Down’s syndrome with vitiligo, to the best of our knowledge, this is the third case of lichen 1. Dourmishev A, Miteva L, Mitev V, Pramatarov K, Schwartz RA. nitidus seen in the patient of Down’s syndrome. Cutaneous aspects of Down syndrome. Cutis 2000;66:420-4. 2. Barankin B, Guenther L. Dermatological manifestations of However, such a combination is not surprising in view Down’s syndrome. J Cutan Med Surg 2001;5:289-93. of the well-known tendency of the skin of individuals 3. Brown AC, Olkowski ZL, McLaren JR, Kutner MH. Alopecia

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areata and vitiligo associated with Down’s syndrome. Arch to normal, platelets gradually improved and vital Dermatol 1977;113:1296. parameters settled. She was maintained on prednisolone 4. Hazini AR, Rongioletti F, Rebora A. rubra pilaris and vitiligo in Down’s syndrome. Clin Exp Dermatol 1988;13:334-5. 1 mg/kg/day after which she stopped developing new 5. Patrizi A, Di Lernia V, Pauluzzi P. Lichen nitidus generalize, vesicles. On review after one month, prednisolone was trisomie 21 et megacolon congenital. Ann Dermatol Venereol 1991;118:725. tapered to 30 mg and the patient is doing well. 6. Laxmisha C, Thappa DM. Generalized lichen nitidus with Down syndrome. J Eur Acad Dermatol Venereol 2006;20:1156-7. Syndrome of inappropriate antidiuretic hormone 7. Daoud MS, Pittelkow MR. Lichen nitidus. In: Fitzpatric TB, Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, (SIADH) is characterized by hyponatremia because Katz SI, editors. Dermatology in General Medicine 6th ed. New of dilution of body fluids by an excess of water York; McGraw-Hill: 2003. p. 478-81. relative to total solute.[1] If SIADH develops rapidly, it is accompanied by symptoms of water intoxication which include nausea, vomiting, headache, confusion CCyclophosphamide-inducedyclophosphamide-induced and coma.[1] These occur as osmotic fluid shifts result in cerebral edema and increased intracranial pressure. ssyndromeyndrome ofof iinappropriatenappropriate Inappropriate secretion of the antidiuretic hormone aantidiureticntidiuretic hormonehormone secretionsecretion (ADH), also known as vasopressin, due to any cause interferes with renal excretion of water and results in production of concentrated urine and hyponatremia. Sir, Pulse therapy with dexamethasone and Antidiuretic hormone (ADH) is secreted by the posterior cyclophosphamide (DCP) is effective in the pituitary gland. The key action of ADH in the kidney treatment of . The adverse effects of is the insertion of water channels like aquaporin-2 and cyclophosphamide include hemorrhagic cystitis, -3 into the principal cells of the collecting duct, thus bone marrow suppression, gastrointestinal symptoms increasing the permeability of water. These channels and gonadal suppression. We report a 26-year-old allow free water to be reabsorbed from the collecting female who developed the Syndrome of Inappropriate duct within the hypertonic renal medulla. In SIADH, Antidiuretic Hormone (SIADH) secretion following the inappropriately elevated level of vasopressin DCP and subsequently also developed features of enhances the reabsorption of water, thereby leading to disseminated intravascular coagulation (DIC) and production of concentrated urine, inability to excrete secondary septicemia. water and consequently hyponatremia.[2] The proposed mechanism by which a drug interferes with the normal A 26-year-old unmarried female with uncontrolled secretion and action of ADH depends on the drug.[3] Drugs was administered dexamethasone that stimulate the release of ADH from the posterior pulse for three days. Oral steroids were continued pituitary gland include nicotine, phenothiazines and since we wanted to avoid antimetabolites, but she tricyclics. Some drugs increase or potentiate the renal continued to develop new vesicles. Eighteen days action of ADH. They include desmopressin, oxytocin following admission we administered DCP pulse. One and prostaglandin synthesis inhibitors. Drugs that cause day following DCP she developed malaise, headache, SIADH by means of mixed or uncertain mechanism nausea, anorexia and excessive thirst. On evaluation, of action include chlorpropamide, carbamazepine, the patient had severe hyponatremia (117 mEq) with cyclophosphamide and vincristine.[3] elevated urinary spot sodium and urine osmolality (280 mmol/kg) suggestive of SIADH. She was treated Prior to establishing a diagnosis of SIADH, a detailed with 3% sodium chloride; however, circulatory volume history should be obtained in order to exclude the was not compromised. Simultaneously, the patient numerous disorders capable of causing hyponatremia. developed features of DIC, such as thrombocytopenia, These include congestive heart failure, hepatic d-Dimer positive and low-normal serum fibrinogen dysfunction, adrenal insufficiency, renal failure and levels that gradually progressed to septicemia and thyroid diseases.[2] Type I respiratory failure. She required critical care management and was supported with noninvasive On physical examination, evaluation of the volume ventilator and administered four units of platelets, four status is important as SIADH is characterized by units of fresh frozen plasma along with 3% NaC1 for euvolemic hyponatremia. Edema in a hyponatremic hyponatremia. Five days later, serum sodium reverted patient should lead to the consideration of conditions

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