|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 186-188
Hypomelanosis of Ito with brain abscess: An incidental association or consequence of underlying structural defect?
Abhijit Saha1, Joly Seth2, Samipa Mukherjee3, Suprit Basu4
1 Pediatric Dermatology Unit, Rita Skin Foundation, Salt Lake, Kolkata, India
2 Department of Dermatology, STD and Leprosy, Burdwan Medical College, Burdwan, West Bengal, India
3 Department of Dermatology, Banglore Medical College and Research Institute, Bengaluru, Karnataka, India
4 Department of Pediatrics, Dr. B. C. Roy Post Graduate Institute of Pediatric Sciences, Kolkata, West Bengal, India
|Date of Web Publication||10-Jul-2015|
Pediatric Dermatology Unit, Rita Skin Foundation, Salt Lake, Kolkata
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Saha A, Seth J, Mukherjee S, Basu S. Hypomelanosis of Ito with brain abscess: An incidental association or consequence of underlying structural defect?. Indian J Paediatr Dermatol 2015;16:186-8
|How to cite this URL:|
Saha A, Seth J, Mukherjee S, Basu S. Hypomelanosis of Ito with brain abscess: An incidental association or consequence of underlying structural defect?. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Jul 7];16:186-8. Available from: http://www.ijpd.in/text.asp?2015/16/3/186/160656
Hypomelanosis of Ito (HI), also known as incontinentia pigmenti (IP) achromians, is a rare neurocutaneous syndrome characterized by hypopigmented macules arranged in whorl, streak, patch or linear configuration along the line of Blaschko. HI, a classical example of cutaneous mosaicism often has multisystem involvement; central nervous system, musculoskeletal and eye being most frequent.
A 1-year-old female child born of nonconsanguineous marriage was brought to the pediatrics department with high-grade fever and repeated episodes of convulsions for last 15 days. Parents also complained of multiple asymptomatic hypopigmented lesions distributed bilaterally symmetrically over trunk and extremities for last 6 months. Lesions were gradually increasing in size with the growth of the baby. Lesions were not preceded by any redness or blister formation. The baby was born of an uneventful pregnancy. Her other siblings were normal. Parents also narrated some degree of developmental delay in the form of delayed monosyllable speech and absence of the pincer grasp and sitting with support.
On examination, baby was drowsy with 102°F axillary temperature. Her pulse rate was 140/min, regular and respiratory rate 52/min. She had mild pallor with positive neck rigidity. Anthropometric measurements were within normal limits. Auscultation revealed no abnormalities. Her abdomen was soft with just palpable liver and spleen below the costal margin; considered to be physiological. Babinsky sign was elicited positive and deep tendon reflexes were exaggerated. Her fontanelle was felt tensed and bulged. She had frequent episodes of vomiting and generalized tonic-clonic seizures during the hospital stay. Deteriorating condition of the patient forced us to shift her to Intensive Care Unit. She was referred to the dermatology department for evaluation of skin lesions.
Cutaneous examination revealed multiple, nonscaly, hypopigmented macules distributed bilaterally symmetrically over trunk and extremities along the line of Blaschko [Figure 1]. Hypochromic lesions over trunk arranged in whorled or streak like configuration whereas those over the limbs were along the length of the same [Figure 1] and [Figure 2]. Accentuation of the lesions underwood lamp examinations was worth-mentioning. Dermatological features were consistent with HI.
|Figure 1: Bilaterally symmetrical hypopigmented lesions over trunk arranged in whorled or streak like configuration whereas those over the limbs were along the length of the same|
Click here to view
Routine blood investigations explored low haemoglobin (8.7 g%), markedly raised total leukocyte count (25,300) with 70% neutrophil and positive C-reactive protein (0.8 mg/L). Tests for malarial parasites, Widal test, liver function test, renal function test, and electrolyte assessment did not reveal any abnormality. Her chest X-ray, echocardiography, and ultrasound found reports were normal. Computer tomography (CT) scan of the brain showed dilated ventricular system, debris and enhancement of ependyma suggestive of ventriculitis [Figure 3]. Most important finding was multiple hypo dense lesions with enhancing margins and intense peripheral edema over right temporal and occipital lobes suggestive of brain abscess [Figure 4]. We planned brain magnetic resonance imaging (MRI) to delineate structural anomaly if any. But grave general condition of the patient and financial restraint of the parents forced us to postpone MRI. Parents did not allow us for skin biopsy.
|Figure 3: Dilated ventricular system in computer tomography scan of the brain|
Click here to view
|Figure 4: Multiple hypo dense lesions with enhancing margins and intense peripheral edema over right temporal and occipital lobes|
Click here to view
Hypomelanosis of Ito is a rare neurocutaneous disorder with slight female preponderance but without any racial predilection. Epidemiological data regarding this syndrome are very limited. Though theoretically third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis; actual estimated prevalence is too low. Disease manifests at birth or early in childhood. The entity was first described by Ito as a negative image of IP. But HI is preferred designation as histopathology finding does not fit with that of the IP; rather findings are just reverse, increased number of melanocytes and melanosomes in the basal layer of the epidermis.
Multisystem extracutaneous involvement is seen in a good number of cases mostly of central nervous s and musculoskeletal system though data vary according to different studies., Epilepsy other than mental retardation is the most common neurological presentation in HI. The seizure types reported include generalized tonic-clonic, partial seizures, myoclonic seizures, and infantile spasms. Mental retardation, developmental delay, and autistic behavior have been reported to be common associations of HI. Although there have been no documentation of constant central nervous system anomalies, more than 50% patients had specific detectable white matter anomalies as found on MRI and CT scan. The anomalies revealed include localized or generalized cerebral, brain stem, ventricular asymmetry including hemimegalencephaly and hemiatrophy. Cerebellar aplasia, dysgenesis of the corpus callosum, heterotopia, vascular malformation, medulloblastoma, choroid plexus papilloma, and periventricular white matter changes are the other reported abnormalities.
Our case showed dilated ventricular system as detected by CT scan with signs suggestive of multiple abscesses in the brain. This evokes a thought that brain abscesses in a child with HI may not be just an incidental finding; underlying subtle malformations in the brain could have acted as a portal of entry for microorganisms leading to abscess formation thereby leading to the clinical manifestation of drowsiness and seizures. Our observation again reinforces the need of systemic evaluation in any suspected case of HI.
| References|| |
Sybert VP. Hypomelanosis of Ito. Pediatr Dermatol 1990;7:74-6.
Pascual-Castroviejo I, López-Rodriguez L, de la Cruz Medina M, Salamanca-Maesso C, Roche Herrero C. Hypomelanosis of Ito. Neurological complications in 34 cases. Can J Neurol Sci 1988;15:124-9.
Ito M. A singular case of naevus depigmentosus systematicus bilateralis. Jpn J Dermatol 1951;61:31.
Ruggieri M, Pavone L. Hypomelanosis of Ito: Clinical syndrome or just Phenotypez. J Child Neurol 2000;15:635-44.
Muhammed K, Mathew J. Coexistence of two neurocutaneous syndromes: Tuberous sclerosis and hypomelanosis of Ito. Indian J Dermatol Venereol Leprol 2007;73:43-5.
Grover S, Dash K, Singh G, Venkateshwar V, Rodrigues P. Hypomelanosis of Ito with rare neurological association. Indian J Dermatol Venereol Leprol 1997;63:65-6.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]