DermAtlas: Online Dermatology Image Library dermatology image,ectodermal dysplasia, hypohidrotic,ectodermal dysplasia, hypohidrotic,ectodermal dysplasia, hypohidrotic,ectodermal dysplasia, hypohidrotic,ectodermal dysplasia, hypohidrotic,nail dystrophy,pili torti

© 2001-2010, DermAtlas	*Image Name:*	hypopidrotic_ectoderma_dysplasia_1_070107	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA, HYPOHIDROTIC / ECTODERMAL DYSPLASIA	*Category:*	genodermatosis/genetic disorder / dental/oral disorder
	*Body Site:*	tooth	*Age:*	0
	*Contributors:*	Meg Gerstenblath, MS IV Rachel Nussbaum, MD
	*Description:*	lack of dental enamel and dental caries
	*Comments:*	This 15-year-old boy came to the attention of his pediatrician at 3 years of age because of complete lack of hair growth. He eventually grew sparse brittle hair with a receding hair line. He also developed little body hair. He had no enamel on his teeth and developed multiple dental caries. Although he reported decreased sweating, he was able to participate in sports. Academically, he was number one in his class. On examination he had frontal bossing, a broad nasal root, and prominent (everted) lips. Note the dental changes and brittle sparse receding hair. The ectodermal dysplasias are a large, heterogeneous group of inherited diseases with defects in the development of two or more structures derived from embryonic ectoderm, most commonly the skin, hair, teeth, eccrine glands, and nails. Ectodermal dysplasia is present in over 150 clinically distinct inherited syndromes. These disorders are caused by genetic defects in the ectodysplasin signal transduction pathway, which is used by epithelial cells in the developing tooth, hair follicle, and eccrine sweat gland during morphogenesis; defects in this pathway lead to aplasia, hypoplasia or dysplasia of these structures. The most frequently reported manifestation of ED is hypohidrotic (or anhydrotic) ectodermal dysplasia (HED), also called Christ-Siemens-Touraine syndrome (OMIM #305100). The most common form of HED is X-linked; autosomal dominant and recessive forms are much less common. The X-linked form occurs in about 1 in 100,000 boys (live-born births) and occurs in all racial and ethnic groups. The affected gene is ED1, which codes for ectodysplasin, which is a ligand secreted by epithelial cells. Of note, mutations in the receptor for ectodysplasin, EDAR, are responsible for the autosomal dominant and recessive forms of HED. Newborns may present with a collodion membrane or skin scaling. Scalp hair is sparse or completely absent; it can be wiry and brittle and is usually blond though lusterless. Hair may darken at puberty and secondary sexual hairs may be normal although body hair is usually sparse or absent. Most affected male patients are unable to sweat, which may lead to hyperpyrexia. The skin can be smooth because of absent sweat pores. Primary and secondary teeth may be affected, with no enamel on teeth or teeth that are abnormally shaped (peg-shaped) or absent or reduced in number. Individuals have a typical facies with a broad saddle nose, frontal bossing, and full, everted lips. Other common skin findings are eczema, periorbital wrinkling and hyperpigmentation, and facial sebaceous hyperplasia. Nails are typically unaffected. Some individuals have thick nasal secretions and recurrent upper and lower respiratory tract infections. Intelligence is normal. In the X-linked disorder, female carriers may be affected with patchy hair sparseness, missing or abnormally-shaped teeth, and patchy distribution of sweat glands (along Blaschko’s lines); random X-inactivation accounts for this variability. Treatment includes prevention of hyperpyrexia, dental restoration, and treatment of eczema and other associated conditions. A helpful resource for patients and physicians is the National Foundation for Ectodermal Dysplasias (www.nfed.org). References: • Lamartine J. Towards a new classification of ectodermal dysplasias. Clinical and Experimental Dermatology 2003;28:351-355. • Itin PH and Fistarol SK. Ectodermal Dysplasias. Am J Med Genet 2004;131C:45-51. • Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatology. 2003, Elsevier Limited, Spain. • National Foundation for Ectodermal Dysplasias website: http://www.nfed.org/FAQ.htm • OMIM website: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=305100
	*Related Images:*	All related Images hypopidrotic_ectoderma_dysplasia_5_070107 hypopidrotic_ectoderma_dysplasia_4_070107 hypopidrotic_ectoderma_dysplasia_3_070107 hypopidrotic_ectoderma_dysplasia_2_070107

© 2001-2010, DermAtlas	*Image Name:*	hypopidrotic_ectoderma_dysplasia_2_070107	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA, HYPOHIDROTIC / ECTODERMAL DYSPLASIA	*Category:*	genodermatosis/genetic disorder / dental/oral disorder
	*Body Site:*	face / hair	*Age:*	0
	*Contributors:*	Meg Gerstenblath, MS IV Rachel Nussbaum, MD
	*Description:*	frontal bossing, a broad nasal root, and prominent (everted) lips
	*Comments:*	This 15-year-old boy came to the attention of his pediatrician at 3 years of age because of complete lack of hair growth. He eventually grew sparse brittle hair with a receding hair line. He also developed little body hair. He had no enamel on his teeth and developed multiple dental caries. Although he reported decreased sweating, he was able to participate in sports. Academically, he was number one in his class. On examination he had frontal bossing, a broad nasal root, and prominent (everted) lips. Note the dental changes and brittle sparse receding hair. The ectodermal dysplasias are a large, heterogeneous group of inherited diseases with defects in the development of two or more structures derived from embryonic ectoderm, most commonly the skin, hair, teeth, eccrine glands, and nails. Ectodermal dysplasia is present in over 150 clinically distinct inherited syndromes. These disorders are caused by genetic defects in the ectodysplasin signal transduction pathway, which is used by epithelial cells in the developing tooth, hair follicle, and eccrine sweat gland during morphogenesis; defects in this pathway lead to aplasia, hypoplasia or dysplasia of these structures. The most frequently reported manifestation of ED is hypohidrotic (or anhydrotic) ectodermal dysplasia (HED), also called Christ-Siemens-Touraine syndrome (OMIM #305100). The most common form of HED is X-linked; autosomal dominant and recessive forms are much less common. The X-linked form occurs in about 1 in 100,000 boys (live-born births) and occurs in all racial and ethnic groups. The affected gene is ED1, which codes for ectodysplasin, which is a ligand secreted by epithelial cells. Of note, mutations in the receptor for ectodysplasin, EDAR, are responsible for the autosomal dominant and recessive forms of HED. Newborns may present with a collodion membrane or skin scaling. Scalp hair is sparse or completely absent; it can be wiry and brittle and is usually blond though lusterless. Hair may darken at puberty and secondary sexual hairs may be normal although body hair is usually sparse or absent. Most affected male patients are unable to sweat, which may lead to hyperpyrexia. The skin can be smooth because of absent sweat pores. Primary and secondary teeth may be affected, with no enamel on teeth or teeth that are abnormally shaped (peg-shaped) or absent or reduced in number. Individuals have a typical facies with a broad saddle nose, frontal bossing, and full, everted lips. Other common skin findings are eczema, periorbital wrinkling and hyperpigmentation, and facial sebaceous hyperplasia. Nails are typically unaffected. Some individuals have thick nasal secretions and recurrent upper and lower respiratory tract infections. Intelligence is normal. In the X-linked disorder, female carriers may be affected with patchy hair sparseness, missing or abnormally-shaped teeth, and patchy distribution of sweat glands (along Blaschko’s lines); random X-inactivation accounts for this variability. Treatment includes prevention of hyperpyrexia, dental restoration, and treatment of eczema and other associated conditions. A helpful resource for patients and physicians is the National Foundation for Ectodermal Dysplasias (www.nfed.org). References: • Lamartine J. Towards a new classification of ectodermal dysplasias. Clinical and Experimental Dermatology 2003;28:351-355. • Itin PH and Fistarol SK. Ectodermal Dysplasias. Am J Med Genet 2004;131C:45-51. • Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatology. 2003, Elsevier Limited, Spain. • National Foundation for Ectodermal Dysplasias website: http://www.nfed.org/FAQ.htm • OMIM website: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=305100
	*Related Images:*	All related Images hypopidrotic_ectoderma_dysplasia_5_070107 hypopidrotic_ectoderma_dysplasia_4_070107 hypopidrotic_ectoderma_dysplasia_3_070107 hypopidrotic_ectoderma_dysplasia_1_070107

© 2001-2010, DermAtlas	*Image Name:*	hypopidrotic_ectoderma_dysplasia_3_070107	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA, HYPOHIDROTIC / ECTODERMAL DYSPLASIA	*Category:*	genodermatosis/genetic disorder / dental/oral disorder
	*Body Site:*	face / scalp hair	*Age:*	0
	*Contributors:*	Meg Gerstenblath, MS IV Rachel Nussbaum, MD
	*Description:*	sparse brittle hair, frontal bossing, a broad nasal saddle, and prominent (everted) lips
	*Comments:*	This 15-year-old boy came to the attention of his pediatrician at 3 years of age because of complete lack of hair growth. He eventually grew sparse brittle hair with a receding hair line. He also developed little body hair. He had no enamel on his teeth and developed multiple dental caries. Although he reported decreased sweating, he was able to participate in sports. Academically, he was number one in his class. On examination he had frontal bossing, a broad nasal root, and prominent (everted) lips. Note the dental changes and brittle sparse receding hair. The ectodermal dysplasias are a large, heterogeneous group of inherited diseases with defects in the development of two or more structures derived from embryonic ectoderm, most commonly the skin, hair, teeth, eccrine glands, and nails. Ectodermal dysplasia is present in over 150 clinically distinct inherited syndromes. These disorders are caused by genetic defects in the ectodysplasin signal transduction pathway, which is used by epithelial cells in the developing tooth, hair follicle, and eccrine sweat gland during morphogenesis; defects in this pathway lead to aplasia, hypoplasia or dysplasia of these structures. The most frequently reported manifestation of ED is hypohidrotic (or anhydrotic) ectodermal dysplasia (HED), also called Christ-Siemens-Touraine syndrome (OMIM #305100). The most common form of HED is X-linked; autosomal dominant and recessive forms are much less common. The X-linked form occurs in about 1 in 100,000 boys (live-born births) and occurs in all racial and ethnic groups. The affected gene is ED1, which codes for ectodysplasin, which is a ligand secreted by epithelial cells. Of note, mutations in the receptor for ectodysplasin, EDAR, are responsible for the autosomal dominant and recessive forms of HED. Newborns may present with a collodion membrane or skin scaling. Scalp hair is sparse or completely absent; it can be wiry and brittle and is usually blond though lusterless. Hair may darken at puberty and secondary sexual hairs may be normal although body hair is usually sparse or absent. Most affected male patients are unable to sweat, which may lead to hyperpyrexia. The skin can be smooth because of absent sweat pores. Primary and secondary teeth may be affected, with no enamel on teeth or teeth that are abnormally shaped (peg-shaped) or absent or reduced in number. Individuals have a typical facies with a broad saddle nose, frontal bossing, and full, everted lips. Other common skin findings are eczema, periorbital wrinkling and hyperpigmentation, and facial sebaceous hyperplasia. Nails are typically unaffected. Some individuals have thick nasal secretions and recurrent upper and lower respiratory tract infections. Intelligence is normal. In the X-linked disorder, female carriers may be affected with patchy hair sparseness, missing or abnormally-shaped teeth, and patchy distribution of sweat glands (along Blaschko’s lines); random X-inactivation accounts for this variability. Treatment includes prevention of hyperpyrexia, dental restoration, and treatment of eczema and other associated conditions. A helpful resource for patients and physicians is the National Foundation for Ectodermal Dysplasias (www.nfed.org). References: • Lamartine J. Towards a new classification of ectodermal dysplasias. Clinical and Experimental Dermatology 2003;28:351-355. • Itin PH and Fistarol SK. Ectodermal Dysplasias. Am J Med Genet 2004;131C:45-51. • Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatology. 2003, Elsevier Limited, Spain. • National Foundation for Ectodermal Dysplasias website: http://www.nfed.org/FAQ.htm • OMIM website: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=305100
	*Related Images:*	All related Images hypopidrotic_ectoderma_dysplasia_5_070107 hypopidrotic_ectoderma_dysplasia_4_070107 hypopidrotic_ectoderma_dysplasia_2_070107 hypopidrotic_ectoderma_dysplasia_1_070107

© 2001-2010, DermAtlas	*Image Name:*	hypopidrotic_ectoderma_dysplasia_4_070107	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA, HYPOHIDROTIC / ECTODERMAL DYSPLASIA	*Category:*	genodermatosis/genetic disorder / dental/oral disorder / hair disorders
	*Body Site:*	hair / scalp	*Age:*	0
	*Contributors:*	Meg Gerstenblath, MS IV Rachel Nussbaum, MD
	*Description:*	sparse brittle hair
	*Comments:*	This 15-year-old boy came to the attention of his pediatrician at 3 years of age because of complete lack of hair growth. He eventually grew sparse brittle hair with a receding hair line. He also developed little body hair. He had no enamel on his teeth and developed multiple dental caries. Although he reported decreased sweating, he was able to participate in sports. Academically, he was number one in his class. On examination he had frontal bossing, a broad nasal root, and prominent (everted) lips. Note the dental changes and brittle sparse receding hair. The ectodermal dysplasias are a large, heterogeneous group of inherited diseases with defects in the development of two or more structures derived from embryonic ectoderm, most commonly the skin, hair, teeth, eccrine glands, and nails. Ectodermal dysplasia is present in over 150 clinically distinct inherited syndromes. These disorders are caused by genetic defects in the ectodysplasin signal transduction pathway, which is used by epithelial cells in the developing tooth, hair follicle, and eccrine sweat gland during morphogenesis; defects in this pathway lead to aplasia, hypoplasia or dysplasia of these structures. The most frequently reported manifestation of ED is hypohidrotic (or anhydrotic) ectodermal dysplasia (HED), also called Christ-Siemens-Touraine syndrome (OMIM #305100). The most common form of HED is X-linked; autosomal dominant and recessive forms are much less common. The X-linked form occurs in about 1 in 100,000 boys (live-born births) and occurs in all racial and ethnic groups. The affected gene is ED1, which codes for ectodysplasin, which is a ligand secreted by epithelial cells. Of note, mutations in the receptor for ectodysplasin, EDAR, are responsible for the autosomal dominant and recessive forms of HED. Newborns may present with a collodion membrane or skin scaling. Scalp hair is sparse or completely absent; it can be wiry and brittle and is usually blond though lusterless. Hair may darken at puberty and secondary sexual hairs may be normal although body hair is usually sparse or absent. Most affected male patients are unable to sweat, which may lead to hyperpyrexia. The skin can be smooth because of absent sweat pores. Primary and secondary teeth may be affected, with no enamel on teeth or teeth that are abnormally shaped (peg-shaped) or absent or reduced in number. Individuals have a typical facies with a broad saddle nose, frontal bossing, and full, everted lips. Other common skin findings are eczema, periorbital wrinkling and hyperpigmentation, and facial sebaceous hyperplasia. Nails are typically unaffected. Some individuals have thick nasal secretions and recurrent upper and lower respiratory tract infections. Intelligence is normal. In the X-linked disorder, female carriers may be affected with patchy hair sparseness, missing or abnormally-shaped teeth, and patchy distribution of sweat glands (along Blaschko’s lines); random X-inactivation accounts for this variability. Treatment includes prevention of hyperpyrexia, dental restoration, and treatment of eczema and other associated conditions. A helpful resource for patients and physicians is the National Foundation for Ectodermal Dysplasias (www.nfed.org). References: • Lamartine J. Towards a new classification of ectodermal dysplasias. Clinical and Experimental Dermatology 2003;28:351-355. • Itin PH and Fistarol SK. Ectodermal Dysplasias. Am J Med Genet 2004;131C:45-51. • Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatology. 2003, Elsevier Limited, Spain. • National Foundation for Ectodermal Dysplasias website: http://www.nfed.org/FAQ.htm • OMIM website: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=305100
	*Related Images:*	All related Images hypopidrotic_ectoderma_dysplasia_5_070107 hypopidrotic_ectoderma_dysplasia_3_070107 hypopidrotic_ectoderma_dysplasia_2_070107 hypopidrotic_ectoderma_dysplasia_1_070107

© 2001-2010, DermAtlas	*Image Name:*	hypopidrotic_ectoderma_dysplasia_5_070107	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA, HYPOHIDROTIC / ECTODERMAL DYSPLASIA	*Category:*	genodermatosis/genetic disorder / dental/oral disorder
	*Body Site:*	tooth	*Age:*	0
	*Contributors:*	Meg Gerstenblath, MS IV Rachel Nussbaum, MD
	*Description:*	lack of dental enamel and dental caries
	*Comments:*	This 15-year-old boy came to the attention of his pediatrician at 3 years of age because of complete lack of hair growth. He eventually grew sparse brittle hair with a receding hair line. He also developed little body hair. He had no enamel on his teeth and developed multiple dental caries. Although he reported decreased sweating, he was able to participate in sports. Academically, he was number one in his class. On examination he had frontal bossing, a broad nasal root, and prominent (everted) lips. Note the dental changes and brittle sparse receding hair. The ectodermal dysplasias are a large, heterogeneous group of inherited diseases with defects in the development of two or more structures derived from embryonic ectoderm, most commonly the skin, hair, teeth, eccrine glands, and nails. Ectodermal dysplasia is present in over 150 clinically distinct inherited syndromes. These disorders are caused by genetic defects in the ectodysplasin signal transduction pathway, which is used by epithelial cells in the developing tooth, hair follicle, and eccrine sweat gland during morphogenesis; defects in this pathway lead to aplasia, hypoplasia or dysplasia of these structures. The most frequently reported manifestation of ED is hypohidrotic (or anhydrotic) ectodermal dysplasia (HED), also called Christ-Siemens-Touraine syndrome (OMIM #305100). The most common form of HED is X-linked; autosomal dominant and recessive forms are much less common. The X-linked form occurs in about 1 in 100,000 boys (live-born births) and occurs in all racial and ethnic groups. The affected gene is ED1, which codes for ectodysplasin, which is a ligand secreted by epithelial cells. Of note, mutations in the receptor for ectodysplasin, EDAR, are responsible for the autosomal dominant and recessive forms of HED. Newborns may present with a collodion membrane or skin scaling. Scalp hair is sparse or completely absent; it can be wiry and brittle and is usually blond though lusterless. Hair may darken at puberty and secondary sexual hairs may be normal although body hair is usually sparse or absent. Most affected male patients are unable to sweat, which may lead to hyperpyrexia. The skin can be smooth because of absent sweat pores. Primary and secondary teeth may be affected, with no enamel on teeth or teeth that are abnormally shaped (peg-shaped) or absent or reduced in number. Individuals have a typical facies with a broad saddle nose, frontal bossing, and full, everted lips. Other common skin findings are eczema, periorbital wrinkling and hyperpigmentation, and facial sebaceous hyperplasia. Nails are typically unaffected. Some individuals have thick nasal secretions and recurrent upper and lower respiratory tract infections. Intelligence is normal. In the X-linked disorder, female carriers may be affected with patchy hair sparseness, missing or abnormally-shaped teeth, and patchy distribution of sweat glands (along Blaschko’s lines); random X-inactivation accounts for this variability. Treatment includes prevention of hyperpyrexia, dental restoration, and treatment of eczema and other associated conditions. A helpful resource for patients and physicians is the National Foundation for Ectodermal Dysplasias (www.nfed.org). References: • Lamartine J. Towards a new classification of ectodermal dysplasias. Clinical and Experimental Dermatology 2003;28:351-355. • Itin PH and Fistarol SK. Ectodermal Dysplasias. Am J Med Genet 2004;131C:45-51. • Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatology. 2003, Elsevier Limited, Spain. • National Foundation for Ectodermal Dysplasias website: http://www.nfed.org/FAQ.htm • OMIM website: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=305100
	*Related Images:*	All related Images hypopidrotic_ectoderma_dysplasia_4_070107 hypopidrotic_ectoderma_dysplasia_3_070107 hypopidrotic_ectoderma_dysplasia_2_070107 hypopidrotic_ectoderma_dysplasia_1_070107

© 2001-2010, DermAtlas	*Image Name:*	ectodermal_dysplasia_1_040520	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA / NAIL DYSTROPHY / PILI TORTI	*Category:*	genodermatosis/genetic disorder / hair disorders / nail disorders
	*Body Site:*	nail, foot	*Age:*	5 years
	*Contributor:*	Bernard Cohen, MD
	*Description:*	thin nails with suggestion of koilonychia
	*Comments:*	This healthy 5-year-old boy had fragile spangled hair from birth. Although his growth and development were normal, his eyebrows were also fine and short, and his nails were thin, spooned,and prone to splitting.
	*Related Images:*	All related Images ectodermal_dysplasia_5_040520 ectodermal_dysplasia_4_040520 ectodermal_dysplasia_3_040520 ectodermal_dysplasia_2_040520

© 2001-2010, DermAtlas	*Image Name:*	ectodermal_dysplasia_2_040520	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA / NAIL DYSTROPHY / PILI TORTI	*Category:*	genodermatosis/genetic disorder / hair disorders / nail disorders
	*Body Site:*	nail, foot	*Age:*	5 years
	*Contributor:*	Bernard Cohen, MD
	*Description:*	thin nails with suggestion of koilonychia
	*Comments:*	This healthy 5-year-old boy had fragile spangled hair from birth. Although his growth and development were normal, his eyebrows were also fine and short, and his nails were thin, spooned,and prone to splitting.
	*Related Images:*	All related Images ectodermal_dysplasia_5_040520 ectodermal_dysplasia_4_040520 ectodermal_dysplasia_3_040520 ectodermal_dysplasia_1_040520

© 2001-2010, DermAtlas	*Image Name:*	ectodermal_dysplasia_3_040520	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA / NAIL DYSTROPHY / PILI TORTI	*Category:*	genodermatosis/genetic disorder / hair disorders / nail disorders
	*Body Site:*	nail, hand	*Age:*	5 years
	*Contributor:*	Bernard Cohen, MD
	*Description:*	thin nails with suggestion of koilonychia
	*Comments:*	This healthy 5-year-old boy had fragile spangled hair from birth. Although his growth and development were normal, his eyebrows were also fine and short, and his nails were thin, spooned,and prone to splitting.
	*Related Images:*	All related Images ectodermal_dysplasia_5_040520 ectodermal_dysplasia_4_040520 ectodermal_dysplasia_2_040520 ectodermal_dysplasia_1_040520

© 2001-2010, DermAtlas	*Image Name:*	ectodermal_dysplasia_4_040520	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA / NAIL DYSTROPHY / PILI TORTI	*Category:*	genodermatosis/genetic disorder / hair disorders / nail disorders
	*Body Site:*	scalp	*Age:*	5 years
	*Contributor:*	Bernard Cohen, MD
	*Description:*	short, fragile, spangled hair
	*Comments:*	This healthy 5-year-old boy had fragile spangled hair from birth. Although his growth and development were normal, his eyebrows were also fine and short, and his nails were thin, spooned,and prone to splitting.
	*Related Images:*	All related Images ectodermal_dysplasia_5_040520 ectodermal_dysplasia_3_040520 ectodermal_dysplasia_2_040520 ectodermal_dysplasia_1_040520

© 2001-2010, DermAtlas	*Image Name:*	ectodermal_dysplasia_5_040520	*File Type:*	jpg
	*Diagnosis:*	ECTODERMAL DYSPLASIA / NAIL DYSTROPHY / PILI TORTI	*Category:*	genodermatosis/genetic disorder / hair disorders / nail disorders
	*Body Site:*	scalp	*Age:*	5 years
	*Contributor:*	Bernard Cohen, MD
	*Description:*	short, fragile, spangled hair
	*Comments:*	This healthy 5-year-old boy had fragile spangled hair from birth. Although his growth and development were normal, his eyebrows were also fine and short, and his nails were thin, spooned,and prone to splitting.
	*Related Images:*	All related Images ectodermal_dysplasia_4_040520 ectodermal_dysplasia_3_040520 ectodermal_dysplasia_2_040520 ectodermal_dysplasia_1_040520