|Year : 2016 | Volume
| Issue : 3 | Page : 226-228
Michelin tire baby syndrome
Rita V Vora, Abhishek P Pilani, Nilofar G Diwan, S RahulKrishna
Department of Dermatology, Shree Krishna Hospital, Pramukh Swami Medical College, Karamsad, Anand, Gujarat, India
|Date of Web Publication||5-Jul-2016|
Rita V Vora
Shree Krishna Hospital, Pramukh Swami Medical College, Karamsad, Anand, Gujarat
Source of Support: None, Conflict of Interest: None
Michelin tire baby syndrome (MTBS), is a rare genodermatosis, characterized by generalized folding of excess skin and may be associated with various phenotypic abnormalities. The pathogenesis of this condition is unclear. Various congenital anomalies can be associated with it. It may be a clinical finding associated with various syndromes. Diagnosis is mainly clinical, and skin folds gradually diminish and disappear with age without any intervention. Here, we report a case of MTBS associated with convergent squint and hydrocephaly.
Keywords: Convergent squint, genodermatosis, skin folds
|How to cite this article:|
Vora RV, Pilani AP, Diwan NG, RahulKrishna S. Michelin tire baby syndrome. Indian J Paediatr Dermatol 2016;17:226-8
| Introduction|| |
Michelin tire baby syndrome (MTBS) is a rare genodermatosis. Ross first described about this condition and coined the term MTBS in 1969 because of the physical resemblance of these patients to the mascot of a French tire manufacturer. It is a rare syndrome and can be inherited in an autosomal dominant pattern, this syndrome is also known as “generalized folded skin.” At birth, as the name implies, the skin is thrown into folds, but it is not lax and feels rather thick. With age, the skin folds spontaneously improve and in childhood, these are mainly confined to the extremities. Hence, it is also termed as “symmetrical ringed creases of the extremities.” It may be associated with various phenotypic abnormalities, and it may be a part of various syndromes. Keeping this in mind, the clinician should be careful in excluding the syndromes and identifying phenotypic abnormalities. Here, we report a case of MTBS in a 2-year-old female.
| Case Report|| |
A 2-year-old female child presented to the Department of Dermatology with asymptomatic skin folds over both the upper and lower limbs since birth, intense pruritus since 1 week. There was a gradual and progressive increase in the number and size of skin folds with time. Her body weight was 11 kg, clinical age was 2 years while her developmental age was 6 months with delayed milestones. Head circumference exceeded normal limit for her age. Other anthropometric measurements were in normal limits.
On cutaneous examination, she was having multiple, symmetric, deep, and gyrate skin folds involving the extremities [Figure 1] and [Figure 2] resembling mascot of a French tire manufacturer. There were no skin lesions over the trunk. The skin folds were present since birth which increased in the thickness with age. Depressed nasal bridge was noticed. Hair, nail, and mucosa were normal. Clinically, diagnosis of MTBS was made. For obvious reasons, skin biopsy was not done.
|Figure 1: Multiple symmetric deep gyrate skin folds involving both upper limbs|
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|Figure 2: Multiple symmetric deep gyrate skin folds involving both the lower limbs|
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She had convergent squint [Figure 3], hydrocephalus, hypotonia, and hyper-extensive joints. Ventriculo peritoneal shunt was carried out to treat hydrocephalus, which was incurative. The patient had an occasional history of seizures. All other siblings were normal. None of her family members had similar complaints. There was a history of consanguineous marriage in the parents.
The pruritus improved with emollients. The parents were counseled, and they were explained about the self-limiting course of this disorder. Regular follow-up was advised but unfortunately, we lost the patient in follow-up.
| Discussion|| |
MTBS is characterized by generalized folding of excess skin although it may be associated with additional phenotypic abnormalities. Most common site of involvement is extremities, but it can also involve trunk, palms, and soles. The pathogenesis is yet unclear. The condition may be familial. Autosomal dominant mode of inheritance has been reported in the familial cases. Paracentric inversion of the long arm of chromosome 11 and deletion of short arm of chromosome 7 has been reported in association with MTBS. Hypertrichosis may be observed in the involved area.
MTBS may be associated with various other congenital anomalies as well as other syndromes. Associated congenital anomalies are craniofacial anomalies, cleft palate, hypoplastic scrotum and hernias (inguinal and umbilical), left sided hemihypertrophy, hemiplegia and microcephaly, psychomotor retardation, epilepsy, joint hypermobility, stellate scarring, developmental delay, and smooth muscle hamartoma which may be diffuse. A case of it being associated with panhypopituitarism is also reported.
It may be a clinical finding associated with other disorders such as Beare–Stevenson cutis gyrate syndrome associated with dermatomegaly localized to scalp, forehead, face, and neck, hearing impairment, undescended testis, circumferential skin creases, and mental handicap syndrome, and multiple congenital anomalies/mental retardation syndrome syndrome. Skin folds may be a part of amniotic band sequence in association with multiple congenital skin creases.
Diagnosis of this syndrome is mainly clinical, and its exact pathogenesis remains unknown. The most common histologic features reported are of nevus lipomatosus and smooth muscle hamartoma (with or without overlying hypertrichosis). MTBS is also known as congenital diffuse lipomatosis  as the dermatomegaly may be due to a diffuse lipomatous nevus in the deeper dermis.
Ainhum, which presents as a constricting ring at the digitoplantar fold of the fifth toe, can be considered as the closest differential diagnosis. It usually progresses to spontaneous amputation of the digit in adulthood, a feature not seen in MTBS.
The skin folds gradually diminish and disappear with age without any intervention. In familial cases, older family members may show few remnants of deep skin folds.
Sardana et al. have reported a case of spontaneously improving MTBS. Palit and Inamadar have reported a second case of MTBS from India. Metta et al. have reported the first case of familial MTBS from India of mother and a male child. To the best of our knowledge, this case should be considered as the fifth case of MTBS from India.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]