Letters to the Editor

AAddressddress forfor correspondence:correspondence: Dr. Sanjiv V. Choudhary, of developmental milestones, associated with feeble 28, Modern Nagpur Society, Chhatrapati Nagar, vocalization, difficulty in walking and dystonic Nagpur - 15 (M.S), India E-mail: [email protected] posturing, all of which apparently developed after the age of about one and half years. Child also had DDOI:OI: 10.4103/0378-6323.53152 - PPMID:*****MID: of the left eye since birth, which did not show RREFERENCESEFERENCES any diurnal variation. There was no definite history of seizures. The patient’s parents noticed hypopigmented 1. Prose NS, Abson KG, Scher RK. Disorders of the nails and hair associated with human immunodeficiency virus infection. and hyperpigmented patches initially over both Int J Dermatol 1992;31:453-7. hands, followed by involvement of foot and face since 2. Sehgal VN, Join S. - clinical perspective. around the age of one year. There was no history of Int J Dermatol 2000;39:241-9. 3. Cribier B, Mena ML, Rey D, Partisani M, Fabien V, Lang JM, any tendency to itch or scratch and there was no et al. Nail changes in patients infected with human history suggestive of photo-exacerbation. There was immunodeficiency virus. A prospective controlled study. Arch no history of any other significant skin or mucosal Dermatol 1998;134:1216-20. 4. Midgley G, Moore MK, Cook C, Phan QG. Mycology of nail lesion or of any hair, nail and teeth abnormalities. disorders. J Am Acad Dermatol 1994;31:S68-74. There was no significant family history of any skin or 5. Tosti A, Piraccini BM, Lorenzi S. Onychomycosis caused by neurological disorders. nondermatophytic molds: Clinical features and response to treatment of 59 cases. J Am Acad Dermatol 2000;42:217-24. 6. Goettmann-Bonvallot S. Clinical types of onychomycosis. Ann On examination, the patient had a dysmorphic Dermatol Venereol 2003;130:1237-43. facies with long philtrum, and thin upper lip. 7. Surjushe A, Kamath R, Oberai C, Saple D, Thakre M, Dharmshale S, et al. A clinical and mycological study of Dermatological examination revealed hypopigmented onychomycosis in HIV infection. Indian J Dermatol Venereol and hyperpigmented macules grouped over the dorsum Leprol 2007;73:397-401. 8. Gianni C, Romano C. Clinical and histological aspects of of hands and feet and over the face [Figures 1 and 2]. toenail onychomycosis caused by Aspergillus spp. Dermatology There was no evidence of leuko-trichia over the 2004;209:104-10. hypopigmented lesions. The skin did not reveal atrophy 9. Mahmoudabadi AZ, Zarrin M. Onychomycosis with Aspergillus flavus: A case report from Iran. Pak J Med Sci 2005;21:497-8. or telangiectasia. No other significant skin or mucosal 10. Hilimoglu-Polat S, Metin DY, Inci R, Dereli T, Kilinc I, Tumbay E. lesions were seen. Hair, teeth and nails were normal. Nondermatophyte molds as agents of onychomycosis in Turkey: A prospective study. Mycopathologica 2005;160:125-8. 11. Kaplan MH, Sadick N, McNutt NS, Meltzer M, Sarngadharan MG, Significant findings on neurological examination Pahwa S. Dermatologic findings and manifestations of AIDS. included - left partial ptosis with Marcus Gunn J Am Acad Dermatol 1987;16:485-506. phenomena. Dystonic posturing of right upper 12. Conant MA. The AIDS epidemic. J Am Acad Dermatol 1994;31:47-50. extremity was seen with spasticity of left lower limb. The patient also had an awkward gait with short steps and narrow base and also had a prominent knock-knee and bilateral pes plano-valgus. There were no other AAcralcral ddyschromatosisyschromatosis withwith significant skeletal system abnormalities. ddevelopmentalevelopmental regressionregression Laboratory investigations, including blood work-up, aandnd dystoniadystonia inin a sseven-even- screening for metabolic disorders and karyotyping were normal. MRI of the brain was normal except for yyear-oldear-old child:child: DyschromatosisDyschromatosis symmetrical hyperintensities in bilateral putamen. ssymmetricaymmetrica hhereditariaereditaria variantvariant oror EEG was essentially normal. Skin biopsy from the hypopigmented areas showed a marked absence a nnewew ssyndrome?yndrome? of basal melanin while the hyperpigmented lesion showed increased focal melanin pigmentation [Figures 3 and 4]. Based on the clinical features and Sir, the investigation findings, a diagnosis of symmetrical A 7-year-old girl, born to non-consanguineous parents acral dyschromatosis (to rule out dyschromatosis was referred to us from the Pediatric Neurology symmetrica hereditaria) was considered. department for evaluation of pigmentary changes over the face and extremities. The patient was being Dyschromatosis symmetrica hereditaria (DSH) or evaluated under neurology for progressive regression reticulate acropigmentation of Dohi is characterized by

412 Indian J Dermatol Venereol Leprol | July-August 2009 | Vol 75 | Issue 4 Letters to the Editor

Figure 1: Hyper and hypo-pigmented lesions on the face Figure 2: Acral dyschromatosis

Figure 3: Histopathology - hyperpigmented area showing focal Figure 4: Histopathology - hypopigmented area showing relative clumping of melanin in the basal layer (H and E, ×400) absence of basal melanin (H and E, ×400) hyperpigmented and hypopigmented macules on the hereditaria (DUH), which is characterized by more face and dorsal aspects of the extremities that appear extensive lesions including the non-acral/unexposed in infancy or early childhood. DSH has been more areas of the skin. A segmental variant called unilateral commonly reported in Japanese and Chinese patients and dermatomal pigmentary dermatosis (UDPD) has also is inherited in an autosomal-dominant pattern. Various been described.[6] Some of the differential diagnosis mutations of the RNA-specific adenosine-deaminase of dyschromatosis which needs to be generally gene (DSRAD) also known as adenosine deaminase, considered includes xeroderma pigmentosum, RNA-specific (ADAR), have been reported in DSH.[1-3] dyschromic and exposure to chemicals such as diphenylcyclopropenone and monobenzyl There have been very few significant non-cutaneous ether of hydroquinone.[7] associations reported with DSH. Significantly though there have been two reports of associated dystonia. In our case, there was symmetrical dyschromatosis Tojo et al. reported DSH in a family associated with in an acral distribution associated with regression dystonia and mental deterioration.[4] Patrizi et al. of milestones and progressive dystonia. However, reported a case associated with idiopathic torsion unlike typical DSH, the lesions (both hypo and dystonia.[5] hyperpigmented) were larger in size. We present this case as a possible variant of DSH or a newer syndrome Dyschromatosis symmetrica hereditaria needs to with acral dyschromatosis and neurological features like be differentiated from dyschromatosis universalis regression of developmental milestones and dystonia.

Indian J Dermatol Venereol Leprol | July-August 2009 | Vol 75 | Issue 4 413 Letters to the Editor

FFerozeeroze Kaliyadan,Kaliyadan, K.K. P.P. VVinayaninayan1, face was not sweating as much as the right side. On BBindiyaindiya Fernandes,Fernandes, M.M. GG.. JJayasreeayasree 2 the trunk, there were patchy areas of increased and Departments of Dermatology, 1Pediatric Neurology, and 2Pathology, decreased sweating. The patient had not applied any Amrita Institute of Medical Sciences, Kochi, Kerala - 682 028, India topical medication to any part of the body. There was

AAddressddress forfor correspondence:correspondence: Dr. Feroze Kaliyadan, no history of trauma to the spine, syncopal attacks or Assistant Professor, Department of Dermatology, Amrita Institute of any other chronic febrile illness. Medical Sciences, Kochi, Kerala - 682 028, India E-mail: [email protected] General clinical examination was normal. Blood DDOI:OI: 10.4103/0378-6323.53154 - PPMID:MID: ***** pressure readings both on supine and prone positions RREFERENCESEFERENCES were normal. On cutaneous examination of the left side of the face over the area of the cheek, there was 1. Miyamura Y, Suzuki T, Kono M, Inagaki K, Ito S, Suzuki N, no sweating and the hair in the beard and moustache et al. Mutations of the RNA-specific adenosine deaminase area were comparatively sparse. There was increased gene (DSRAD) are involved in dyschromatosis symmetrica hereditaria. Am J Hum Genet 2003;73:693-9. sweating over areas corresponding to T8 and 2. Oyama M, Shimizu H, Ohata Y, Tajima S, Nishikawa T. T9 dermatomes on the right side and T3 and T4 Dyschromatosis symmetrica hereditaria (reticulate dermatomes on the left side [Figure 1]. The skin over acropigmentation of Dohi): Report of a Japanese family with the condition and a literature review of 185 cases. Br J Dermatol the dermatomes showed uniform hyperpigmentation. 1999;140:491-6. The remaining skin over the trunk showed decreased 3. Kondo T, Suzuki T, Mitsuhashi Y, Ito S, Kono M, Komine M, sweating even after vigorous exercise. Both the upper et al. Six novel mutations of the ADAR1 gene in patients with dyschromatosis symmetrica hereditaria: Histological limbs and the lower limbs including the palms and observation and comparison of genotypes and clinical soles showed decreased sweating. phenotypes. J Dermatol 2008;35:395-406. 4. Tojo K, Sekijima Y, Suzuki T, Suzuki N, Tomita Y, Yoshida K, et al. Dystonia, mental deterioration, and dyschromatosis Pupils of both the eyes were sluggish in their reaction symmetrica hereditaria in a family with ADAR1 mutation. Mov to light and on adding 0.125% pilocarpine drops, there Disord 2006;21:1510-3. was constriction of both the pupils (Holme’s Adie 5. Patrizi A, Manneschi V, Pini A, Baioni E, Ghetti P. Dyschromatosis [2] symmetrica hereditaria associated with idiopathic torsion pupil) [Figure 2]. dystonia. A case report. Acta Derm Venereol 1994;74:135-7. 6. Rai R, Kaur I, Handa S, Kumar B. Dyschromatosis Universalis Ankle reflex, knee reflex, biceps and supinator Hereditaria. Indian J Dermatol Venereol Leprol 2000;66:158-9. reflex of both sides were depressed. Other systemic 7. Urabe K, Hori Y. Dyschromatosis. Semin Cutan Med Surg 1997;16:81-5. examinations including the central nervous system and spine were normal.

Routine investigations like hemogram, urine examination RRossoss ssyndromeyndrome and thyroid profile were within normal limits. Venereal Disease Research Laboratory (VDRL) test was Sir, nonreactive. Chest X-ray and radiographs of the cervical, Ross syndrome is a rare disorder of sweating associated thoracic and lumbosacral spine detected no abnormality. with depressed or absent deep tendon reflexes and tonic Histopathological examination from the anhidrotic area pupil due to selective degeneration of sympathetic showed sparse to absent eccrine sweat glands and the pathways.[1] About 40 cases of Ross syndrome have biopsied skin from the hyperhidrotic area showed been reported so far. We report below a 19-year-old increased number and size of eccrine sweat glands and patient with typical features of Ross syndrome. epidermal hypermelanosis [Figures 3a and b].

A 19-year-old male patient presented with heat Ross syndrome is a rare disorder of sweating comprising intolerance and absence of sweating from the face, of widespread hypohidrosis combined with patchy trunk and extremities in an irregular fashion for compensatory hyperhidrosis associated with areflexia last 5 years. As the patient was working in a bakery, and tonic pupil (Holme’s Adie syndrome).[1] It affects the ambient temperature of which is high, the patient both males and females with age of onset ranging from could not tolerate the atmosphere and used to go 3 to 50 years. Patients with Holme’s Adie syndrome out or pour water on his body frequently. But there often show asymptomatic changes in sweating. The was no history of hospitalization for anhidrosis may be localized or widespread. When at any time. He also noticed that the left half of the anhidrosis is extensive, the remaining areas of the

414 Indian J Dermatol Venereol Leprol | July-August 2009 | Vol 75 | Issue 4