|Year : 2020 | Volume
| Issue : 2 | Page : 110-115
Mucocutaneous findings in patients with cerebral palsy: A cross-sectional observational study
Bhabani STP Singh1, Tapaswini Tripathy1, Manish Kumar Sahu1, Sweta2, Bikash Ranjan Kar1
1 Department of Skin and VD, IMS and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Paediatrics, IMS and SUM Hospital, Bhubaneswar, Odisha, India
|Date of Submission||18-Oct-2019|
|Date of Acceptance||28-Dec-2019|
|Date of Web Publication||01-Apr-2020|
Department of Skin and VD, IMS and SUM Hospital, Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
Introduction: Cerebral palsy (CP) is a condition characterized by abnormal muscle tone or posture and coordination. It is caused due to injury to the immature brain usually at or before birth. Speech, cognition, visual, and other neurological problems have been studied in this disorder. However, dermatological and mucosal changes seen in CP have rarely been studied. Materials and Methods: Mucocutaneous findings of CP patients visiting a rehabilitation center on two different visits were analyzed by two dermatologists. Results: Trauma, motor incoordination and deformities, drug-related side effects, and cognitive and behavioral impairment are the major causes of various cutaneous and mucosal changes seen in CP patients. Conclusion: Our study finds majority of dermatoses are secondary to cerebral palsy and they are preventable.
Keywords: Cerebral palsy, cutaneous, children, mucosal
|How to cite this article:|
Singh BS, Tripathy T, Sahu MK, Sweta, Kar BR. Mucocutaneous findings in patients with cerebral palsy: A cross-sectional observational study. Indian J Paediatr Dermatol 2020;21:110-5
|How to cite this URL:|
Singh BS, Tripathy T, Sahu MK, Sweta, Kar BR. Mucocutaneous findings in patients with cerebral palsy: A cross-sectional observational study. Indian J Paediatr Dermatol [serial online] 2020 [cited 2021 Jan 21];21:110-5. Available from: https://www.ijpd.in/text.asp?2020/21/2/110/281721
| Introduction|| |
Cerebral palsy (CP) is a clinical syndrome primarily affecting motor coordination and muscle tone. It results from various injuries to the developing brain. The prevalence of CP ranges from 1.5 to more than 4 per 1000 live births. Intellectual impairment, visual disturbances, and orthopedic and urinary disorders have been described as various manifestations of this disease. Impaired muscle tone and coordination, lack of alertness for self-care, behavioral problems, use of medications, and supportive appliances put these patients at risk for various dermatoses. There is a paucity of literature about this aspect.
Aims and objectives
This study was conducted to document and analyze mucocutaneous findings in CP children.
| Material and Methods|| |
A cross-sectional observational study was carried out at a rehabilitation center over 2 months, March–April 2019, on two separate visits. Informed consent was obtained from the parents or guardians of the patients. All the patients were examined in natural daylight, and photographs were obtained from appropriate sites. Wood's lamp examination was done whenever required. However, biopsy of any patient could not be done. All abnormal findings were noted and analyzed. The clinical findings were independently confirmed by two different dermatologists. Institutional ethics committee approval was obtained prior to initiation of the study.
All cases with a documented diagnosis of CP who attended the rehabilitation center for physiotherapy/speech therapy/occupational therapy.
- Patients uncooperative for examination
- Patients without any mucocutaneous findings
- Age of more than 18 years.
The Statistical Package for the Social Sciences version 23 (IBM Corp., SPSS Statistics for Windows, Armonk, NY: USA) was used for statistical analysis.
| Results|| |
A total of 50 consecutive CP patients were examined at a rehabilitation center on two separate visits. Three patients were above 18 years of age and five patients were uncooperative to be examined. Three children had no cutaneous or mucosal findings. Hence, 39 CP children were included in the study population.
Males outnumbered females in the ratio of 2.24:1. The mean age of presentation of the patients was 9.6 ± 4.9 years. The youngest was 8 months of age. Seventeen children had been diagnosed with spastic syndromes, 10 had dyskinetic CP, and 12 had hypotonic symptoms.
The cutaneous and mucosal findings in the participants were classified into dermatoses secondary to CP and primary dermatoses [Table 1] and [Table 2]. CP-related changes were categorized according to their etiologies such as trauma associated, due to deformities, findings secondary to motor incoordination, drug-related side effects, and cutaneous changes due to cognitive impairment.
|Table 1: Primary dermatoses among cerebral palsy children unrelated to disease condition|
Click here to view
| Discussion|| |
Our study showed a male preponderance similar to observation made by Parra.
The most common cutaneous finding observed in our study was posttraumatic ulceration and scarring over the face and extremities [Figure 1] and [Figure 2]. This can be attributed to muscle in coordination and improper gait which makes the patients vulnerable for repeated falls. Three of our patients had dental trauma in the form of broken incisors. Holan et al. in their study found that 57% of CP children had evidence of dental trauma. Parra found scars in 47.34% of cases in his study. All the scars were secondary to surgical procedures. Two surgical scars were observed in our population of the study. They were seen secondary to foot deformity correction surgery. Lichenification and frictional keratosis were observed over areas of friction and toes were seen in 12.8% of children [Figure 3] and [Figure 4]. Toe walking due to fixed deformity caused the lichenified changes. Abnormal posture and movements are the possible causes of frictional keratosis. Parra observed lichenification due to crawling in patients in approximately 10% of his study population.
We observed intertrigo in 7.69% of cases as compared to a 5.65% case by Parra. All the patients in the study by Parra had maceration on the palms; however, intertrigo was seen on the cubital fossa and popliteal fossa in the present study [Figure 5]. This difference in observation is due to the type of CP patients present in the respective studies. Erythema and maceration over flexures can be explained by lack of aeration and sweat accumulation due to fixed contractures. Lack of hygiene and proper care might be the contributing factor.
Drooling of saliva was present in 17.9% of our patients. Previous studies have reported a prevalence of drooling in CP children varying from 12% to as high as 58%., The lowest age of the patient with drooling was 3.5 years, whereas the highest age was 17 years. With age, the severity of drooling decreases. However, in our study, we found drooling present also in higher aged children. Hegde and Pani observed that the prevalence of drooling was highest among children with a mixed type of CP (60%) and spastic quadriplegia (58.5%), whereas it was lowest among children with spastic diplegia (36.8%) and athetosis (22.2%). Drooling of saliva in patients with CP is due to multiple factors such as motor incoordination inability to close mouth properly, and coordinate swallowing. We found irritant contact dermatitis due to saliva in one patient. Parra observed contact dermatitis due to saliva in five patients. A high-arched palate was observed in three of our cases [Figure 6]. This finding was also noted by Rahul et al. in 20% of CP patients. Al Hashmi et al. also found a significantly higher proportion of high-arched palate in children with CP compared to controls. Macroglossia was seen in 59.8% of study population by Al Hashmi et al. In contrast, we found the same in one patient [Figure 7].
Mutilation and lichenified changes were secondary to self-induced trauma by repetitive chewing and nibbling of the digits [Figure 8] and [Figure 9]. Erythematous plaque due to licking was also found in 3.5% and lichenification and hyperkeratosis due to bite were observed in 12.72% of study population in the previous Mexican study.
Ant bite abrasions were present in one patient [Figure 10]. The patient was on sugar-flavored medication, which used to trickle from the angle of mouth. Improper cleaning of mouth by the caregiver as well as drooling from the mouth could be the possible cause for the same. Another patient also had scarring following rat bite on the face [Figure 11]. These children are prone to animal bite due to impaired cognitive ability. No such findings were observed by Parra.
Seborrhea and acneiform eruption were secondary to medications such as phenobarbitone, phenytoin, and valproic acid used for prophylaxis of epilepsy.
Our patients had skin lesions suggestive of cutaneous mosaicism, i.e., segmental vitiligo, lichen striatus, and nevus depigmentosus. There are reports of other mosaicism-associated disorders such as linear and whorled hypomelanosis and incontinentia pigmenti in association with CP., Genetic analysis of CP patients has reported mosaicism as one of the modes of inheritance. One of our patients aged 8 months had corpus callosum agenesis; however, no features suggestive of cutaneous mosaicism were present in this patient. We intend to keep the patient under follow-up for development of any relevant dermatoses.
Many of our cases had acanthosis nigricans over knuckles and toes despite thin body built [Figure 12]. Acanthosis nigricans has been reported in neurocutaneous syndromes such as PTEN hamartoma tumor syndrome, Cowden syndrome, and Costello syndrome. However, no such findings were previously reported in any study.
There has been an increasing prevalence of dermatophytosis among children in the recent past years. There were only three cases of tinea corporis among the 39 cases of total study patients. Possible explanation for the relatively lesser number of dermatophytosis in our study could be due to selective sampling of the study participants from a rehabilitation center where parents receive training about personal hygiene.
Contrary to the common sites of intertrigo in general population, these special children are prone to develop intertrigo over sites of fixed contractures. Hence, educating the caregiver about these facts and special attention of these locations regarding proper wiping and use of antiperspirant powder would be beneficial.
Fissuring and ulceration can occur over the frictional sites. Adaptive footwear can minimize trauma and friction over the feet. Application of bland emollients and keratolytics is suggested by the authors to be used in appropriate circumstances by the caregivers. The use of soft padded protective cushions around the joints would reduce the chances of skin changes associated with friction around joints and pressure points.
Parents should be trained and instructed to clean the oral cavity and perioral skin properly following food intake, particularly sweet food, as ant bite abrasions are a possible outcome of this. Such a case has been observed in our study population.
Papular urticaria or insect bite reactions are as common as in other pediatric age groups in the CP population. Mosquito net and use of full sleeve clothes are advised.
Three of our patients had pediculosis capitis. Basarsian and Yilmaz showed that demodex folliculorum density was more in CP children compared to age-matched controls. This was due to decreased immunity secondary to malnutrition favoring the proliferation of parasite. However, Parra had not observed any parasitic infestation in his study population.
Parra and Turrentine et al. observed dermatitis neglecta in this group of population., No such finding was found in our study.
Cases of myiasis have also been observed by few authors., We did not find any such cases.
Several findings observed in the Mexican study were not seen in our study, possibly because of smaller study population.
Limitations of our study
- Sample size was not estimated before the study
- Photographs could not be obtained in some uncooperative patients.
| Conclusion|| |
Dermatoses found in CP children are mostly secondary to deformities, fixed contractures, and cognitive impairment. Cutaneous conditions unrelated to the neurodegenerative conditions are also seen in this group of patients. Most of the acquired dermatoses are preventable. We propose that specific training should be provided to the caregivers of these patients regarding proper skin and mucosal care. This will ensure improvement in the quality of life of these special children.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Stavsky M, Mor O, Mastrolia SA, Greenbaum S, Than NG, Erez O. Cerebral palsy-trends in epidemiology and recent development in prenatal mechanisms of disease, treatment, and prevention. Front Pediatr 2017;5:21.
Parra IV. Skin findings in patients with cerebral palsy and their classification. Dermatol Rev Mex 2017;61:10-27.
Holan G, Peretz B, Efrat J, Shapira Y. Traumatic injuries to the teeth in young individuals with cerebral palsy. Dent Traumatol 2005;21:65-9.
Budhraja SN, Smiles SR, Perianayagam WJ. Surgical management of dribbling saliva in cerebral palsy. Indian J Surg 1973;35:283-7.
Van De Heyning PH, Marquet JF, Creten WL. Drooling in children with cerebral palsy. Acta Otorhinolaryngol Belg 1980;34:691-705.
Hegde AM, Pani SC. Drooling of saliva in children with cerebral palsy-etiology, prevalence, and relationship to salivary flow rate in an Indian population. Spec Care Dentist 2009;29:163-8.
Ray SA, Bundy AC, Nelson DL. Decreasing drooling through techniques to facilitate mouth closure. Am J Occup Ther 1983;37:749-53.
Rahul VK, Mathew C, Jose S, Thomas G, Noushad MC, Feroz TP. Oral manifestation in mentally challenged children. J Int Oral Health 2015;7:37-41.
Al Hashmi H, Kowash M, Hassan A, Al Halabi M. Oral health status among children with cerebral palsy in Dubai, United Arab Emirates. J Int Soc Prev Community Dent 2017;7:S149-54.
Du-Thanh A, Kluger N, Bensalleh H, Guillot B. Drug-induced acneiform eruption. Am J Clin Dermatol 2011;12:233-45.
Deb S, Sarkar R, Samanta AB. A brief review of nevus depigmentosus. Pigment Int 2014;1:56-8. [Full text]
Schepis C, Alberti A, Siragusa M, Romano C. Progressive cribriform and zosteriform hyperpigmentation: The late-onset feature of linear and whorled nevoid hypermelanosis associated with congenital neurological, skeletal and cutaneous anomalies. Dermatology 1999;199:72-3.
Shah SN, Gibbs S, Upton CJ, Pickworth FE, Garioch JJ. Incontinentia pigmenti associated with cerebral palsy and cerebral leukomalacia: A case report and literature review. Pediatr Dermatol 2003;20:491-4.
Matthews AM, Blydt-Hansen I, Al-Jabri B, Andersen J, Tarailo-Graovac M, Price M, et al
. Atypical cerebral palsy: Genomics analysis enables precision medicine. Genet Med 2019;21:1621-8.
Toll A, Real FX. Somatic oncogenic mutations, benign skin lesions and cancer progression: Where to look next? Cell Cycle 2008;7:2674-81.
Mishra N, Rastogi MK, Gahalaut P, Yadav S, Srivastava N, Aggarwal A. Clinicomycological study of dermatophytoses in children: Presenting at a tertiary care center. Indian J Paediatr Dermatol 2018;19:326-30. [Full text]
Halpert E, Borrero E, Ibañez-Pinilla M, Chaparro P, Molina J, Torres M, et al
. Prevalence of papular urticaria caused by flea bites and associated factors in children 1-6 years of age in Bogotá, D.C. World Allergy Organ J 2017;10:36.
Basarsian F, Yilmaz C. Children with cerebral palsy have greater demodex density than age matched control group. Health MED j 2002;56:73-6.
Turrentine JE, Blalock TW, Davis LS. Unusually severe case of dermatosis neglecta. Skinmed 2012;10:46-7.
Verma P. Cutaneous myiasis in an infant with cerebral palsy. Indian J Dermatol 2014;59:310-1.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]
[Table 1], [Table 2]