Letters to the Editor patient with linear scleroderma has been reported so chromosome 3p21. Because of these mutations, far with anonychia. Basically, the loss of plate triacylglycerols accumulate in cytosolic droplets in results from the involvement of the nail apparatus multiple organs.[1] Tzanck smear findings of DCS were due to the sclerotic process. This change will be not reported previously. irreversible. Anonychia is generally described in textbooks as a congenital anomaly; acquired causes A 21-year-old man was admitted in the dermatology are usually trauma and .[6] The present department with generalized present since case shows, for the first time, that linear scleroderma birth. He had no family history of a similar condition. can cause anonychia thus documenting a so far Dermatologic examination revealed generalized scaly unreported cause of acquired anonychia. skin lesions in face, abdomen, arms, and legs [Figure 1]. There was marked involvement of flexures. SSanjayanjay Singh,Singh, SurendraSurendra KumarKumar The individual scales over the trunk were white, Department of Dermatology, Institute of Medical Sciences, Banaras fine, translucent, and semiadherent while those Hindu University, Varanasi - 221 005, India on the face were grey brown scale-crust. Bullous lesions, erosions, and keratosis pilaris were absent. AAddressddress forfor correspondencecorrespondence: Dr. Sanjay Singh, C-9, New Medical Enclave, Banaras Hindu University, Varanasi - 221 005, The palms, soles, teeth, and nails were normal. India. E-mail: [email protected] Ophthalmological examination revealed early DOI: 10.4103/0378-6323.57736 - PMID: 19915255 corticonuclear cataract, hyperopia, and ectropion. Audiological examination revealed bilateral sensory RREFERENCESEFERENCES neural hearing loss. Abdominal and neurologic examinations were normal. 1. Falanga V, Killoran CE. Morphea. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York: McGraw- Laboratory parameters revealed increased levels of Hill; 2008. p. 543-6. aspartate aminotransaminase (75 U/L; normal range: 2. Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, et al. Localized scleroderma in adults and children. Clinical 0–40), alanine aminotransaminase (81 U/L; normal and laboratory investigations on 239 cases. Eur J Dermatol range: 0–40), and creatine phosphokinase (785 U/L; 2003;13:171-6. normal range: 25–175). The complete blood count, 3. Woo TY, Rasmussen JE. Juvenile linear scleroderma associated with serological abnormalities. Arch Dermatol 1985;121:1403- fasting blood sugar, renal function tests, thyroid 5. function tests, lipid levels, albumin, and bilirubin 4. Wollina U, Buslau M, Weyers W. Squamous cell carcinoma were within normal range. Hepatitis markers were in pansclerotic morphea of childhood. Pediatr Dermatol 2002;19:151-4. negative. Giemsa stained peripheral blood smear 5. Wollina U, Buslau M, Heinig B, Petrov I, Unger E, Kyriopoulou of patient showed lipid vacuoles in neutrophils E, et al. Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell consistent with Jordan’s anomaly [Figure 2a]. Chest carcinoma. Int J Low Extrem Wounds 2007;6:291-8. X-ray, electrocardiogram, electromyelography, cranial 6. de Barkers DAR, Baran R, Dawber RPR. Disorders of nails. computerized tomography were normal. Abdominal In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. 7th ed. Oxford: Blackwell; 2004. p. ultrasonography revealed increased echotexture 62.1-62. suggestive of grade II–III steatohepatitis. Liver biopsy was planned, but the patient did not allow.

After cleaning with alcohol, the skin of the face was grasped between the thumb and forefinger of TTzanckzanck ssmearmear Þ nndingding ofof the left hand. A superficial incision was made with DDorfman-Chanarinorfman-Chanarin syndromesyndrome a blade (no. 15) and the tissue was scraped through the incision. The cellular materials were then spread as a thin layer onto two microscopic slides and the Sir, air-dried specimens were stained by Giemsa. Tzanck Dorfman-Chanarin syndrome (DCS), also referred as smear examination showed vacuoles in the cytoplasm neutral lipid storage disease with ichthyosis, is an of keratinocytes [Figure 2b]. Histopathologic autosomal recessive inherited disorder characterized examination of skin biopsy confirmed lipid vacuoles by lipid vacuoles in peripheral leukocytes and several in keratinocytes [Figure 3]. tissues. Genetic studies have identified a causative mutation in hydrolase CGI-58 gene on human Based on the clinical, laboratory, and histological

Indian J Dermatol Venereol Leprol | November-December 2009 | Vol 75 | Issue 6 625 Letters to the Editor

In DCS, skin, liver, muscle, eye, ear, neurologic leucocytes involvement are frequent.[2] Clinical manifestations of this syndrome are ichthyosis (100%), hepatosteatosis (63.6%), myopathy (60.6%), sensorineural hearing loss (30.3%), growth retardation (21.2%), mental retardation (24.2%), cataract (42%), ectropion, strabismus (12.1%), nystagmus (9.1%), myopia (9.1%), and neurological symptoms. Lipid vacuoles in the leucocytes (Jordan’s anomaly) are important diagnostic criteria of this syndrome and can be identified in 100% of leucocytes.[3] In our patient, who had hepatosteatosis, sensorineural hearing loss, cataract, hyperopia, ectropion, Jordan’s anomaly, and raised levels of serum muscle enzyme, hyperopia has Figure 1: Clinical appearance of the patient not been previously reported.

The most common cutaneous finding of DCS is congenital nonbullous ichthyosiform erythroderma. Ichthyosis is often present at birth. Collodion baby, nonspecific ichthyosiform dermatosis, sparing of the face, generalized scaly erythematous plaques, erythematous scaly migrating plaques, hyperkeratosis on the palms and soles, cicatricial or diffuse alopecia are other dermatological manifestations. Nail involvement is absent in most of the cases, but transverse leuconychia, yellow-black discoloration, dystrophic or thickened nails, pitting and onychoschizia can be seen occasionally.[3]

Figure 2: Peripheral blood smear showing lipid droplets (arrows) in polymorphonuclear leukocytes and monocytes (2a), Histopathologically, examination of skin biopsy Tzanck smear showing lipid droplets (arrows) in cytoplasm of demonstrates hyperkeratosis, focal parakeratosis, keratinocytes (2b) (Giemsa stain, x1000) moderate acanthosis, elongation of the rete ridges, lipid droplets in keratinocytes, prominently in the epidermal basal layer and in appendageal epithelia. [1] Furthermore, electron microscopic examination reveals cytoplasmic lipid vacuoles in many dermal cells, such as fibroblasts, pericytes, mast cells, and epidermal Langerhans’ cells.[4]

Tzanck smear test is an inexpensive, useful, and rapid diagnostic tool for certain skin diseases. It has been used in the diagnosis of various erosive-vesiculobullous, nodular and tumoral skin lesions during the following six decades. But, practice of cytodiagnosis in dermatologic diseases has been limited to herpetic infections, pemphigus, and cutaneous leishmaniasis. [5] Figure 3: Lipid droplets in cytoplasm of keratinocytes (H and E, In this case report, we demonstrated vacuoles in x1000) keratinocytes by cytologic examination. findings, a diagnosis of DCS was made. He was started There is no effective treatment of DCS, but a diet low on emollients and responded only minimally. in long-chain fatty acids is reported to decrease skin

626 Indian J Dermatol Venereol Leprol | November-December 2009 | Vol 75 | Issue 6 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 vitiligo 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 papules on the legs for Başkent University Faculty of Medicine, Department of Dermatology, 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 epidermis 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, atopic dermatitis, seborrheic dermatitis, cutaneous pruritus is occasionally present. The histopathology of infections, , vitiligo, syringomas, lichen nitidus is usually distinctive. Keratosis pilaris, elastosis perforans, keratoderma palmaris et plantaris, lichen spinulosa, lichen scrofulosorum, and verruca

Indian J Dermatol Venereol Leprol | November-December 2009 | Vol 75 | Issue 6 627