Congenital Localized Alopecia - Assignment Example

Congenital localized alopecia: A review Hair and the Newborn Clinical inspection of the hair and scalp of a newborn infant can tell us much. It is a method of early identification of genetic, metabolic, and neurologic disorders. There are a number of autosomal positive and autosomal negative gene disorders that are first manifested by disorders of the scalp and hair. When patterns of hair growth are not normal, it may signal underlying brain or central nervous system disorders. The initial assessment of any newborn should include a good evaluation of scalp, hair color, quantity of hair, the direction the hair grows, what the hairline looks like and whether there are whorls. Thos infants that are found should have a focused reassessment, a thorough family history, and continued examination of the hair (Furdon & Clark, 2003). The clinician must also have a good understanding of the embryological development and cycles of hair growth, as well as an understanding of abnormal conditions. This paper will discuss a few of those conditions that might be seen in the newborn and young child. Aplasia Cutis Aplasia Cutis is a rare condition in which there is a congenital absence of the skin, usually presenting on the scalp (Beckman, Griot, Niessen et.al., 2008). It can occur anywhere in the body but most often occurs at or near the scalp vertex and appears to be a single or sometimes multiple non-inflammatory patch of absent skin. It can vary in shape and size and how deep it is (Huang, Llang, Hu,et.al., 2009). In about 20% of reported cases, part of the skull is also missing. The area may be pink or red and oval or round and may have a smooth, atrophic or papyraceus surface (Savoia, Giacomini, Aprile, et.al., 2008). If the child is seen for the first time later, there may be hypertropic scarring where any healing takes place. There are two different clinical presentations of this particular disease. Membranous which is believed to be a mild form of tubal closure defect and non-membranous which is irregular. In both cases this is a sporadic disorder with a rare familial distribution (Huang, et.al., 2008). The average child is Caucasian of Italian origin. Aplasia Cutis may appear isolated or with other anomalies and frequently appears when there is also a nervous system defect. It sometimes appears with epidermolysis bullosa and deformed nails and occasionally with congenital heart lesions. In the case of membranous, there is possibility that it represents a mild neural tube defect. The difference in membranous and non-membranous may be the difference in pathogenic effects in the formation of the scalp. It may appear with Adams-Oliver syndrome in which there is a combination of congenital aplasia cutis and anomalies of the extremities and vessels , especially in the skull (Beckman, et.al., 2008) The severity of the disease when the child is born will determine what needs to be done next. In some cases the lesions will have already scarred over, in others tissue expansion will need to be done. Full thickness skin grafts can be used in those cases where necessary. If the defect is minor, most heal readily. It is recommended that topical antiseptic or topical antibiotics be used however to prevent infection while healing. There may need to be later treatment of hair transplant and there may be a need for some plastic surgery later in life based on the scaring that takes place. When using a non surgical approach there are recommendations for Omniderm with absorbent fine mesh gauze impregnated with 3% bismuth tribromophenateridin for cranial defects and the use of permeable polyurethne film in others. Certainly a full work up and follow up on these children, other than the skin lesions is necessary to assure that there are not other problems to be cared for or stabilized over their lifetime. Those can most often include neurological disorders as well as cardiac disorders. Nevus Sebaceous Nevus Sebaceous is difficult to differentiate from aplasia cutis. Nevus Sebaceous is pink-yellow or yellow orange, usually round or oval and has a velvety surface and at the beginning may be difficult to see the difference between the two. It becomes more evident as diagnosis is made. It is one of the two most common of the alopecia diseases seen in newborns, the other being aplasia cutis. The differentiation can be important, however, for treatment as well as long term follow up Nevus Sebaceous is often seen with several other problems. There is often a syndrome called Epidermal nevus syndrome in which there may be seizures and or mental retardation. There may also be extracutaneous abnormalities involving organs derived from neuroectoderm Nevus Sebaceous may need a full work up to determine that this is nevus sebaceous and to be sure of whether it is just the nevus or if there is another part of the syndrome involved (Miller, 2007). Dermatoscopy may be important to use in this particular diagnosis. It is increasingly used tool whether or not the nevus is melanocytic or nonmelanocytic. There are syndromes related to the nevus sebaceous that may be noted by using this method. Most often, this syndrome is characterized by a focal or diffuse defect of the skin. It is usually limited to the dermis and epidermis unlike aplasia cutis (Savoia, Neri, Raone, et.al., 2008). Treatment can range from a small removal of a nevus to biopsy and removal of large tumor area. Most nevus do not become positive for cancer diagnosis until after puberty but there is the possibility that it may happen before then. Treatment of other problems related to this diagnosis may be important and that may be seizures as well as organ problems. These children often have nervous system disorders that need long term care, as well as mental retardation. These children will need to be watched for all of the above problems for a lifetime. They may also need plastic surgery as they progress. Triangular Temporal Alopecia This actually presents in children that are 3-6 years old. It is not uncommon to misdiagnose this as alopecia areata, especially if it appears someplace other than the usually fronto-termporal hairline (Iouzzo, Pazzaglia, Staarpee et.al., 2008). It is a stable form of nonscarring alopecia. There does not seem to be a sexual preference for this disorder but has not been found in African Americans at this time. It is usually characterized by an absence of inflammation and a normal number of hair follicles that can be seen under vidodermoscopy. The differential diagnosis includes such things as alopecia areata, tricholellomaia, traction alopecia and in some cases focal scarring disorder such as aplasia cutis cogenita. It is a common disorder but the correct diagnosis needs to be made to prevent the patient from needing biopsy or other unnecessary intervention. It can sometimes be noted to occur and associated with Down's Syndrome. Once diagnosis is correct there is no specific treatment needed here. Follow up is the same as any other child. Treatment can occur at the discretion of the patient and his family that wishes to have restoration surgery using follicular unit transplants. Hair punch graft transplantation has been shown to be effective and Wu, (2009) reports a case where he shows the effectiveness during study. In this case, complete excision and hair restoration surgery was offered to the patient and family and that was the treatment chosen. Hair growth was cosmetically effective within 16 months. Birth Trauma (electrodes, vacuum assisted devices) Birth injuries are usually related to mechanical forces that cause of hemorrhage or edema in the tissues involved. Many things can cause this including long delivery time, forceful delivery, fetal position and size. Birth trauma injury can cause several different things but often is seen the halo scalp ring which is usually a moderate degree of scalp molding with a halo of contused skin on the left parietooccipital area. It is usually about 1 cm wide and contused (Neal, Merk, Norins, 1984). There may or may not be respiratory distress in the infant and it may resolve quickly or it may not. Alopecia, itself may be related to birth injury but it is unusual. Birth associated fetal head injuries though can be quite complicated and are most often classified as extracranial, cranial, and intracranial. The cosmetic problems from extracranial injuries usually clear up quickly and go away. Alopecia may occur, however. It is usually associated with caput succedaneum but may also be associated with cephalhematoma (Lykoudis, Spyropoulou, Lavasidis, et.al., 2007). Neal, et.al. (1984) relays the fact that the band may not go away and skull radiographs should be done to assure that there are not fractures. Any hematoma that appears should resolve itself within six days. This child should be watched for damage to the hair follicles which usually cause of a band that may be a persistent scalp lesion and may cause hair loss. Based on the trauma that occurred would determine the immediate care given. Respiratory arrest can occur here which certainly would mean not only resuscitating the child but all of the diagnostic testing and follow up needed here. This child may have permanent damage that needs follow up that can include respiratory and cardiac issues. Vascular lesions (hemangioma of infancy) There are several kinds of hemangiomas of infancy. They can be disfiguring but also life threatening. PHACE or large facial angiomas can be especially disfiguring and often times is also very dangerous. The hemangiomas might be posterior fossa malformations, hemangiomas of the large facial variety, arterial malformations, cardiac anomalies, eye anomalies, subglottic hemangiomas and hemangiomas of the liver. CT of the abdomen is recommended on these infants to check for hemangiomas and neuroimaging is recommended to check for brain malformations. Laryngoscopy should be done to detect hemangioma of the airway and 10-20% can lead to endangering complications requiring immediate treatment. 1% are usually life threatening. Success in treatment usually lies in system corticosteroids. This creates more follow up issues as there are long term side effects of corticosteroids including Cushings disease and diabetes. Antiangiogenic drugs can also be used as well as surgery though surgery is almost never first line. Pulse dyed laser can be used and has been used successfully on those that are surface oriented. Hemangiomas may ulcerate and bleed and when this happens should be treated with a topical antibiotic. Occipital Alopecia in newborns Occipital alopecia of newborns is often caused by friction and amounts to an area where pressure or friction is felt often or the occipital region of the head. It is very common, especially in young black babies and children with curly hair. It is seldom permanent but may be if the hair follicles have been damaged. As children get older they may cause the problem with what is called pony tail alopecia or alopecia caused from wearing a ponytail often. There is no treatment and it is not a condition to be concerned about. Rare Syndromes "Focal facial dermal dysplasia is a rare ectodermal disorder (also called Setleis Syndrome) is characterized by congenital bitemporal scar-like depressions resembling forceps marks and variable additional facial manifestations"(Neumann, Stieler, Peytavi, 2008). No gene defects are known to date. There may be distichiasis of the upper eyelids, and a facial appearance that seems rather coarse. There have been autosomal dominant and autosomal recessive inheritance. Gomez-Lopez-Hernandez syndrome is a very rare genetic disorder with a distinct phenotype. There have only been seven documented cases at this point. The study child by Apacik, Cohen, Vojta et.al. (2007). Has lambdoid suture, partial scalp alopecia, corneal opacity, mental retardation, and striking phenotypic features. Other signs are asymmetrical skull, corneal opacity, corneal and facial anesthesia, and ataxia. These things are consistent with Gomez-Lopex-Hernandez syndrome. These clinical signs are also noted with aggressive behavior, seizures and raging periods which this child has. MRI shows rhombencephalosynapsis which a rate fusion of the cerebellar hemispheres. In conclusion, hair characteristics are inherited and both typical and atypical findings may be familial in nature. The clinician must be aware of all the wide variations. Abnormal hair characteristics are not isolated, they often occur along with several other findings. The clinician must systmatically assess the infant from head o toe with special attention paid to skin, hair, nails, or other embryological structures that arise from neuroectoderm. Documentation and follow up on all findings is very important. Resources Apacik, C., Cohen, Vojta, S., Wagner, B. (2007). Gomez-Lopez-Hernandez syndrome: Description of an additional case and review of the literature. 167. 123-126. DOI: 10.1007/s00431-007-0478-z. Beekmans, S., Griot, J., Niessen, F., Mulder, J. (2008). Tissue expansion for correction of Baldness in apasia cutis congenital. Eur J. Peiatr. 168: 541-544. DOI 10.1007/s00431-008 -0784-0. Furdan, S., & Clark, D., (2003). Scalp hair characteristics in newborn infants. Focus on the Physical. 3(6). 286-296. DOI:10.1016/j.adnc.2003.09.005. Hill, S., Rademaker, M. (2009). A collection of rare anomalies: multiple digital glomuvenous Malformations, epidermal naevus, temporal alopecia, heterochromia and abdominal l Lipoblastoma. Clinical Dermatology. 34. 862-864. DOI: 10.1111/j.1365- 2230.2009.03616.x. Huang, J., Llang, C., Hu, S., Chieng, C. (2009). Hypertrophic scarring is the usual outcome of Non-membranous aplasia cutis of the scalp. Pediatric Dermatology. 26(3). Iouzzo, M., Pazzaglia, M., Staarace, M., Meletello, G., Tosti, A. (2008). Videodermoscopy: a useful tool for diagnosing congenital triangular alopecia. 25(6). 652-654. Kenner, J., Sperling, L. (1998). Pathological case of the month. Pediatric Adolescent Medicine. 152. Lykoudis, E., Spyropoulou, G., Lavasidis, L., Pashopoulos, M., et.al, (2007). Alopecia associated With birth injury. The American College of Obstetrics. 110(2). McGaughran, J., Aftimos, S. (2002). Setleis Syndrome: three new cases and a review of Literature. American Journal of Medical Genetics. 111. 376-380. Miller, C. Ioffreda, M., Billingsby, E.,(2007). Sebaceous carcinoma, basel cell carcinoma, Trichoadenoma, trichoblastoma within nevus sebaceous. Dematologic Surgery. 30(12). 1546-1549. Neri, I., Savoia, F., Giacomini, F., Raone, B., et.al. (2008). Usefulness of dermatoscopy for the early diagnosis of sebaceous naevus and differentiation from aplasia cutis congenital. Clinical Dermatology. 34. DOI: 10.1111/j.1365-2230.2008.03189.x. Neal, P., Merk, P., Norins, A. (1984). Halo scalp ring: A form of localized scalp injury Associated with caput succedaneum. Pediatric Dermatology. 2(1). 52-54. Neumann-Graul, L., Stieler, K., Peytavi, U., (2008). Autosomal dominant inheritance in a large Family with focal facial dermal dysplasia. American Journal of Medical Genetics. 149. 746-750. Wen-Yu, W., Otberg, N., Kang, H., et.al. (2009). Successful treatment of temporal triangular Alopecia by hair restoration surgery using follicular unit transplantation. American Society for Dermatologic Surgery, Inc. 35. 1307-1310. DOI: 10.1111/j.1524- 4725.2009.01233.x Wu, W., Otberg, N., Kang, H., Zanet, L., Shapiro, J. (2009). Successful treatment of temporal Triangular alopecia by hair restoration surgery using follicular unit transplantation. Department of Dermatology. DOI: 10.1111/j.1524-4725.2009.01233.x Wu, W., Newmann, G. (2005). Management of infants with ulcerated hemangiomas. Journal of Wound and Continence Nursing. 32(1). 61-63. Read More