A m e r i c a n Journal of Medical Genetics 44:664-667 (1992)
Brief Clinical Report
Familial Systematized Epidermal Nevus Syndrome J a m e s F. Meschia, Edward Junkins, and Karen J. Hofman The Center for Medical Genetics ( J P . M , K.J.H.),Department of Pediatrics (E.J., K.J.H.), The Johns Hopkins University School of Medicine, Baltimore, Maryland
Epidermal nevi are typically congenital but rarely familial. We report on a family in which 3 relatives have systematized epidermal nevi. The propositus also has evidence of a hemangioma and a hemangioendothelioma. Peripheral blood and skin fibroblast karyotypes of the propositus did not show evidence of mosaicism. Epidermal nevi have been associated with nondermatologic pathology, involving the nervous, vascular, and skeletal systems in sporadic cases. This report demonstrates that nondermatologic pathology can be also be associated with systematized epidermal nevi in a familial setting. The apparent skipping of generations may be explained by autosomal dominant inheritance with decreased penetrance. 0 1992 Wiley-Liss, Inc. KEY WORDS: epidermal nevus, Blaschko’s lines, ichthyosis hystrix, hemangioma, hemangioendothelioma, SchimmelpenningFeuerstein-Mims syndrome, nevus unius lateris, nevus sebaceous of Jadassohn INTRODUCTION Epidermal nevi arise in the basal layer of the dermis and show a slight propensity for undergoing neoplastic transformation to such lesions as basal cell epitheliomas [Jones and Heyl, 1970; Mehregan and Pinkus, 1965; Swint and Klaus, 19701.Disorders defined by the distribution and type of epidermal nevi include ichthyosis hystrix, nevus unius lateris, and nevus sebaceous of Jadassohn. Evidence for genetic transmission of epidermal nevi is scant. We report on a family in which both the propositus and a maternal female relative have biopsy confirmed epidermal nevi. An epidermal nevus was found on clinical examination of the maternal grandfather. Received for publication January 14, 1992; revision received April 17, 1992. Address reprint requests to Karen J. Hofman, M.D., Center for Medical Genetics, The Johns Hopkins Hospital, Blalock 1008, 600 N. Wolfe Street, Baltimore, MD 21205.
0 1992 Wiley-Liss, Inc.
CLINICAL REPORT History The propositus, a 4-month-old male, presented with failure to thrive, “marble cake” skin hyperpigmentation, and a left chest mass. He was the 2.9 kg product of a spontaneous vaginal delivery following a 42 week gestation, born to a 17-year-old mother and a 23-year-old father, both of African American origin. There was no known consanguinity. Patchy hyperpigmentation was noted at birth. At age 3 days, a dermatologist noted lesions involving the chest, right shoulder, axilla, buttocks, and right leg. Subcutaneous nodules with overlying erythema were noted on chest and back. At 4 days a superficial abdominal hemangioma was excised. At 3.5 months, a chest CT showed a homogeneous, noncalcified 4.5 x 3.5 x 2.0 cm left lateral chest wall mass deep to the musculature, displacing but not deforming ribs. By 4 months, a chest radiograph showed deformity of ribs underlying the mass. A maternal first cousin, currently aged 37 years (Fig. 1, 111-21, has had right-sided hyperpigmented, hyperkeratotic, verrucous plaques with a linear configuration involving the neck, antecubital fossa, thigh, popliteal fossa and foot since birth. Histopathology of the lesions at age 9 indicated a diagnosis of ichthyosis hystrix. She has had no medical problems to date. The maternal grandfather (Fig. 1,ll-3) of the propositus has a 15 x 12 cm longitudinally arrayed patch of nevi on his right lateral thigh. He refused biopsy. Examination of the skin of the propositus’ mother (Fig.1, 111-7)including a Wood‘s lamp examination did not show any evidence of hyperpigmentation. Physical Examination Height and weight were less than the 5th centile for age, and head circumference was on the 10th centile. Mild right lingual hemihypertrophy was present. A hard, fixed chest mass was present over the left anterior axillary line, and a right scrota1mass was palpable. The neurological examination was nonfocal. Verrucous patchy hyperpigmentation was present on the face and arms and the right anterior trunk (Fig. 2). Lesions of the face and trunk stopped abruptly at the anterior midline. The back was symmetrically involved with the stripes resembling inverted chevrons. Nevi were also present on the right lower limb with relative sparing of the left lower limb. Limb lesions were arrayed in longitudinal stripes (Fig. 2).
Familial Systematized Epidermal Nevi
665
1
rl 5
IV 1
7
3
4
Fig. 1. Pedigree.
Laboratory Studies and Hospital Course On admission the propositus’ peripheral leukocyte count was 10,200/mm3, hematocrit was 34.3%, and platelet count was 108,000/mm3.The prothrombin and activated partial thromboplastin times were both 1.1 x control. Skin biopsies were taken of hyperpigmented and normal skin on the right and left back, respectively. Routine histopathology showed mild acanthosis in the skin specimen from the grossly hyperpigmented area and no abnormality of the normal skin specimen. Karyotypes from peripheral blood leukocytes and cultured fibroblasts of the normal and pigmented skin were 46,XY at the 800 band stage. The propositus became progressively thrombocytopenic, with a nadir of 28,000/mm3on day 9 of admission. Hypofibrinogenemia was also noted, with a fibrinogen level of 115 mg/dl measured on day 13. A bone marrow aspirate showed normal cellularity including megakaryocytes. The hematological studies were considered to be consistent with Kasabach-Merritt syndrome. A skeletal survey and renal ultrasound were normal. Tes-
ticular ultrasonography showed a 1.8 x 1.8 x 1.2 cm nonhyperemic right extratesticular mass. Histopathology of the surgically removed left chest wall mass showed a hemangioendothelioma. Two weeks following surgery, the platelet count had risen to 347,000/mm3.
DISCUSSION The incidence of congenital sporadic epidermal nevi is approximately 1:1,000,with no gender predilection [Solomon and Esterly, 19751. The term epidermal nevus syndrome (ENS) is used when vascular, neurologic, and osseous abnormalities have been associated with epidermal nevi [Rogers et al., 1989; Solomon et al., 1968; Solomon and Esterly, 19751. The propositus is the first documented case of familial ENS with associated nondermatolop pathology. In one series of 60 patients with sporadic ENS, 22 had cutaneous hemangiomas other than a nuchal nevus flammeus [Solomon and Esterly, 19751. Vascular lesions of higher morbidity have included a leptomeningeal hemangioma and other forms of cerebrovascular dysplasia [Mollica et al., 1974; Dobyns and
Fig, 2. (A) Anterior trunk and (B) back of propositus. The protruding left lateral chest wall mass is a hemangioendothelioma.
666
Meschia et al. TABLE I. Previously Reported Familial Cases of Epidermal Nevi
Reference Pack et al. [19411
Pedigree number 1
2 3 4
5
6 Solomon e t al. [19751
7
Monk et al. [19751
8 9 10
Benedetto e t al. 119901
11
Sahl [19901
12
Affected members
Associated findings
Female propositus Daughter Female propositus Daughter Female propositus Mother Female propositus Brother Grandmother Male propositus Sister A Sister B Female twin A Female twin B Sib A Sib B Father to son Father t o son Propositus Daughter Propositus Maternal half brother Propositus Daughter Granddaughter
None None None None None None None None Malignant degeneration of nevus None None None None None None None None None None None None None Basal cell Ca arising from nevus None None
Garg, 19911. Neurologic manifestations such as seizures and mental retardation have prompted some observers to regard the ENS as a neurocutaneous disorder, alternatively known as the Schimmelpenning-FeuersteinMims syndrome (McKusick #165630) [Baker et al., 1987; Lantis et al., 1986; Marden and Venters, 1966; Feuerstein and Mims, 19621.Recently, a discrete neurological variant of the ENS has been proposed consisting of facial epidermal nevus, ipsilateral hemimegalencephaly with gyral malformation, mental retardation, seizures, and variable facial hemihypertrophy [Pavone et al., 19911. Neurologic signs in other patients with ENS may be secondary to vascular anomalies [Dobyns and Garg, 19911. "he nevi of the propositus strikingly conform to the pathological body topography first described in 1901 known as Blaschko's lines [Jackson, 19761. Pigmentary anomalies following Blaschko's lines have been seen in the heterozygous state of X-linked disorders like Menke syndrome (McKusick #309400) and incontinentia pigmenti (McKusick #308300). Happle [1985] has proposed that in such cases the pattern of Blaschko is the result of functional X-chromosomal mosaicism. He further proposes that mosaicism in as yet undiscovered lethal genes causes the sporadic occurrence of syndromes like ENS which are collectivelycharacterized by patchy skin lesions, variably associated congenital anomalies, and an equal gender distribution [Happle, 19871. Patients with ENS have consistently been shown to lack mosaicism detectable a t a chromosomal level. In 8 previously reported cases of sporadic ENS as well as this familial one, the karyotypes were normal [Holden and Dekaban, 1972; Mollica et al., 1974; Solomon, 19751. Aside from the propositus, in only one other case was a
fibroblast karyotype performed; this too was normal [Holden and Dekaban, 19721. It is rare t o uncover a positive family history for epidermal nevi. In a prospective series of 131 sequential unselected cases presenting to dermatologists with epidermal nevi, a family history was sought, but none was found [Fbgers et al., 19891. A total of 12 pedigrees with epidermal nevi have been documented (Table 1). X-linked inheritance is unlikely since men and women seem affected in equal numbers. The pattern of inheritance in this case is consistent with autosomal dominance with variable expressivity, including nonpenetrance. In conclusion, patients with epidermal nevi are at increased risk of associated abnormalities, and warrant careful assessment and followup. Furthermore, when a patient with ENS is encountered a family history of this disorder should be sought.
REFERENCES Baker RS, Ross PA, Baumann FLJ (1987):Neurologic complications of the epidermal nevus syndrome. Arch Neurol 44:227-232. Benedetto L, Sood U, Blumenthal N, Madjar D, Sturman S, Hashimoto K (1990): Familial nevus sebaceus J Am Acad Dermatol 23: 130-132. Dobyns WB, Garg BP (1991):Vascular abnormalities in the epidermal nevus syndrome. Neurology 41:276-278. Feuerstein RC, Mims LC (1962):Linear nevus sebaceous with convulsions and mental retardation. Am J Dis Child 104:675-679. Happle R (1985):Lyonization and the lines of Blaschko. Hum Genet 70200-206. Happle R (1987):Lethal genes surviving by mosaicism: a possible explanation for sporatic birth defects involving the skin. J Am Acad Dermatol 16:899-906. Holden KR, Dekaban AS (1972):Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology 22:879-887.
Familial Systematized Epidermal Nevi Jackson R (1976):The lines of Blaschko: A review and reconsideration. Br J Dermatol 95:349-360. Jones EW, Hey1 T (1970): Nevus sebaceus: A report of 140 cases with special regard to the development of secondary m a l i p a n t tumors, Br J Dermatol 82:99-117. Lantis S, Leyden J, Thew M, Heaton C (1968): Nevus sebaceus of Jadassohn. Part of a new neurocutaneous syndrome? Arch Dermatol 98:117-123. Marden PM, Venters HD Jr (1966):A new neurocutaneous syndrome. Am J Dis Child 112:79-81. Mehregan AH, Pinkus H (1965): Life history of organoid nevi. Special reference to nevus sebaceus of Jadassohn. Arch Dermatol 91: 574-588. Mollica F, Pavone L, Nuciforo G (1974): Linear sebaceus nevus syndrome in a newborn. Am J Dis Child 1285368-871. Monk BE, Vollum DI (1982): Familial nevus sebaceus. J R SOCMed 75:660-661. Pack GT, Sunderland DA (1941): Naevus unius lateris. Arch Surg 43:341-375.
667
Pavone L, Curatolo P, R i m R, Micali G, Incorpora G, Garg BP, Dunn DW, Dobyns WB (1991): Epidermal Nevus Syndrome: a neurological variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy. Neurology 41:266-27 1. Rogers M, McCrossin I, Commens C (1989): Epidermal nevi and the epidermal nevus syndrome. J Am Acad Dermatol20:476-488. Sahl WJ, Jr. (1990):Familial nevus sebaceus of Jadassohn: occurrence in three generations. J Am Acad Dermatol 222353454. Solomon LM (1975): Epidermal Nevus Syndrome. Mod Rob1 Paediatr 17:27-30. Solomon LM, Esterly NB (1975): Epidermal and other organoid nevi. Curr Probl Pediat 6:l-55. Solomon LM, Fretzin DF, Dewald RL (1968): The epidermal nevus syndrome. Arch Dermatol97:273-285. Swint RB, Klaus SN (1970): Malignant degeneration of an epithelial nevus. Arch Dermatol 101:56-58.
Brief Clinical Report
Familial Systematized Epidermal Nevus Syndrome J a m e s F. Meschia, Edward Junkins, and Karen J. Hofman The Center for Medical Genetics ( J P . M , K.J.H.),Department of Pediatrics (E.J., K.J.H.), The Johns Hopkins University School of Medicine, Baltimore, Maryland
Epidermal nevi are typically congenital but rarely familial. We report on a family in which 3 relatives have systematized epidermal nevi. The propositus also has evidence of a hemangioma and a hemangioendothelioma. Peripheral blood and skin fibroblast karyotypes of the propositus did not show evidence of mosaicism. Epidermal nevi have been associated with nondermatologic pathology, involving the nervous, vascular, and skeletal systems in sporadic cases. This report demonstrates that nondermatologic pathology can be also be associated with systematized epidermal nevi in a familial setting. The apparent skipping of generations may be explained by autosomal dominant inheritance with decreased penetrance. 0 1992 Wiley-Liss, Inc. KEY WORDS: epidermal nevus, Blaschko’s lines, ichthyosis hystrix, hemangioma, hemangioendothelioma, SchimmelpenningFeuerstein-Mims syndrome, nevus unius lateris, nevus sebaceous of Jadassohn INTRODUCTION Epidermal nevi arise in the basal layer of the dermis and show a slight propensity for undergoing neoplastic transformation to such lesions as basal cell epitheliomas [Jones and Heyl, 1970; Mehregan and Pinkus, 1965; Swint and Klaus, 19701.Disorders defined by the distribution and type of epidermal nevi include ichthyosis hystrix, nevus unius lateris, and nevus sebaceous of Jadassohn. Evidence for genetic transmission of epidermal nevi is scant. We report on a family in which both the propositus and a maternal female relative have biopsy confirmed epidermal nevi. An epidermal nevus was found on clinical examination of the maternal grandfather. Received for publication January 14, 1992; revision received April 17, 1992. Address reprint requests to Karen J. Hofman, M.D., Center for Medical Genetics, The Johns Hopkins Hospital, Blalock 1008, 600 N. Wolfe Street, Baltimore, MD 21205.
0 1992 Wiley-Liss, Inc.
CLINICAL REPORT History The propositus, a 4-month-old male, presented with failure to thrive, “marble cake” skin hyperpigmentation, and a left chest mass. He was the 2.9 kg product of a spontaneous vaginal delivery following a 42 week gestation, born to a 17-year-old mother and a 23-year-old father, both of African American origin. There was no known consanguinity. Patchy hyperpigmentation was noted at birth. At age 3 days, a dermatologist noted lesions involving the chest, right shoulder, axilla, buttocks, and right leg. Subcutaneous nodules with overlying erythema were noted on chest and back. At 4 days a superficial abdominal hemangioma was excised. At 3.5 months, a chest CT showed a homogeneous, noncalcified 4.5 x 3.5 x 2.0 cm left lateral chest wall mass deep to the musculature, displacing but not deforming ribs. By 4 months, a chest radiograph showed deformity of ribs underlying the mass. A maternal first cousin, currently aged 37 years (Fig. 1, 111-21, has had right-sided hyperpigmented, hyperkeratotic, verrucous plaques with a linear configuration involving the neck, antecubital fossa, thigh, popliteal fossa and foot since birth. Histopathology of the lesions at age 9 indicated a diagnosis of ichthyosis hystrix. She has had no medical problems to date. The maternal grandfather (Fig. 1,ll-3) of the propositus has a 15 x 12 cm longitudinally arrayed patch of nevi on his right lateral thigh. He refused biopsy. Examination of the skin of the propositus’ mother (Fig.1, 111-7)including a Wood‘s lamp examination did not show any evidence of hyperpigmentation. Physical Examination Height and weight were less than the 5th centile for age, and head circumference was on the 10th centile. Mild right lingual hemihypertrophy was present. A hard, fixed chest mass was present over the left anterior axillary line, and a right scrota1mass was palpable. The neurological examination was nonfocal. Verrucous patchy hyperpigmentation was present on the face and arms and the right anterior trunk (Fig. 2). Lesions of the face and trunk stopped abruptly at the anterior midline. The back was symmetrically involved with the stripes resembling inverted chevrons. Nevi were also present on the right lower limb with relative sparing of the left lower limb. Limb lesions were arrayed in longitudinal stripes (Fig. 2).
Familial Systematized Epidermal Nevi
665
1
rl 5
IV 1
7
3
4
Fig. 1. Pedigree.
Laboratory Studies and Hospital Course On admission the propositus’ peripheral leukocyte count was 10,200/mm3, hematocrit was 34.3%, and platelet count was 108,000/mm3.The prothrombin and activated partial thromboplastin times were both 1.1 x control. Skin biopsies were taken of hyperpigmented and normal skin on the right and left back, respectively. Routine histopathology showed mild acanthosis in the skin specimen from the grossly hyperpigmented area and no abnormality of the normal skin specimen. Karyotypes from peripheral blood leukocytes and cultured fibroblasts of the normal and pigmented skin were 46,XY at the 800 band stage. The propositus became progressively thrombocytopenic, with a nadir of 28,000/mm3on day 9 of admission. Hypofibrinogenemia was also noted, with a fibrinogen level of 115 mg/dl measured on day 13. A bone marrow aspirate showed normal cellularity including megakaryocytes. The hematological studies were considered to be consistent with Kasabach-Merritt syndrome. A skeletal survey and renal ultrasound were normal. Tes-
ticular ultrasonography showed a 1.8 x 1.8 x 1.2 cm nonhyperemic right extratesticular mass. Histopathology of the surgically removed left chest wall mass showed a hemangioendothelioma. Two weeks following surgery, the platelet count had risen to 347,000/mm3.
DISCUSSION The incidence of congenital sporadic epidermal nevi is approximately 1:1,000,with no gender predilection [Solomon and Esterly, 19751. The term epidermal nevus syndrome (ENS) is used when vascular, neurologic, and osseous abnormalities have been associated with epidermal nevi [Rogers et al., 1989; Solomon et al., 1968; Solomon and Esterly, 19751. The propositus is the first documented case of familial ENS with associated nondermatolop pathology. In one series of 60 patients with sporadic ENS, 22 had cutaneous hemangiomas other than a nuchal nevus flammeus [Solomon and Esterly, 19751. Vascular lesions of higher morbidity have included a leptomeningeal hemangioma and other forms of cerebrovascular dysplasia [Mollica et al., 1974; Dobyns and
Fig, 2. (A) Anterior trunk and (B) back of propositus. The protruding left lateral chest wall mass is a hemangioendothelioma.
666
Meschia et al. TABLE I. Previously Reported Familial Cases of Epidermal Nevi
Reference Pack et al. [19411
Pedigree number 1
2 3 4
5
6 Solomon e t al. [19751
7
Monk et al. [19751
8 9 10
Benedetto e t al. 119901
11
Sahl [19901
12
Affected members
Associated findings
Female propositus Daughter Female propositus Daughter Female propositus Mother Female propositus Brother Grandmother Male propositus Sister A Sister B Female twin A Female twin B Sib A Sib B Father to son Father t o son Propositus Daughter Propositus Maternal half brother Propositus Daughter Granddaughter
None None None None None None None None Malignant degeneration of nevus None None None None None None None None None None None None None Basal cell Ca arising from nevus None None
Garg, 19911. Neurologic manifestations such as seizures and mental retardation have prompted some observers to regard the ENS as a neurocutaneous disorder, alternatively known as the Schimmelpenning-FeuersteinMims syndrome (McKusick #165630) [Baker et al., 1987; Lantis et al., 1986; Marden and Venters, 1966; Feuerstein and Mims, 19621.Recently, a discrete neurological variant of the ENS has been proposed consisting of facial epidermal nevus, ipsilateral hemimegalencephaly with gyral malformation, mental retardation, seizures, and variable facial hemihypertrophy [Pavone et al., 19911. Neurologic signs in other patients with ENS may be secondary to vascular anomalies [Dobyns and Garg, 19911. "he nevi of the propositus strikingly conform to the pathological body topography first described in 1901 known as Blaschko's lines [Jackson, 19761. Pigmentary anomalies following Blaschko's lines have been seen in the heterozygous state of X-linked disorders like Menke syndrome (McKusick #309400) and incontinentia pigmenti (McKusick #308300). Happle [1985] has proposed that in such cases the pattern of Blaschko is the result of functional X-chromosomal mosaicism. He further proposes that mosaicism in as yet undiscovered lethal genes causes the sporadic occurrence of syndromes like ENS which are collectivelycharacterized by patchy skin lesions, variably associated congenital anomalies, and an equal gender distribution [Happle, 19871. Patients with ENS have consistently been shown to lack mosaicism detectable a t a chromosomal level. In 8 previously reported cases of sporadic ENS as well as this familial one, the karyotypes were normal [Holden and Dekaban, 1972; Mollica et al., 1974; Solomon, 19751. Aside from the propositus, in only one other case was a
fibroblast karyotype performed; this too was normal [Holden and Dekaban, 19721. It is rare t o uncover a positive family history for epidermal nevi. In a prospective series of 131 sequential unselected cases presenting to dermatologists with epidermal nevi, a family history was sought, but none was found [Fbgers et al., 19891. A total of 12 pedigrees with epidermal nevi have been documented (Table 1). X-linked inheritance is unlikely since men and women seem affected in equal numbers. The pattern of inheritance in this case is consistent with autosomal dominance with variable expressivity, including nonpenetrance. In conclusion, patients with epidermal nevi are at increased risk of associated abnormalities, and warrant careful assessment and followup. Furthermore, when a patient with ENS is encountered a family history of this disorder should be sought.
REFERENCES Baker RS, Ross PA, Baumann FLJ (1987):Neurologic complications of the epidermal nevus syndrome. Arch Neurol 44:227-232. Benedetto L, Sood U, Blumenthal N, Madjar D, Sturman S, Hashimoto K (1990): Familial nevus sebaceus J Am Acad Dermatol 23: 130-132. Dobyns WB, Garg BP (1991):Vascular abnormalities in the epidermal nevus syndrome. Neurology 41:276-278. Feuerstein RC, Mims LC (1962):Linear nevus sebaceous with convulsions and mental retardation. Am J Dis Child 104:675-679. Happle R (1985):Lyonization and the lines of Blaschko. Hum Genet 70200-206. Happle R (1987):Lethal genes surviving by mosaicism: a possible explanation for sporatic birth defects involving the skin. J Am Acad Dermatol 16:899-906. Holden KR, Dekaban AS (1972):Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology 22:879-887.
Familial Systematized Epidermal Nevi Jackson R (1976):The lines of Blaschko: A review and reconsideration. Br J Dermatol 95:349-360. Jones EW, Hey1 T (1970): Nevus sebaceus: A report of 140 cases with special regard to the development of secondary m a l i p a n t tumors, Br J Dermatol 82:99-117. Lantis S, Leyden J, Thew M, Heaton C (1968): Nevus sebaceus of Jadassohn. Part of a new neurocutaneous syndrome? Arch Dermatol 98:117-123. Marden PM, Venters HD Jr (1966):A new neurocutaneous syndrome. Am J Dis Child 112:79-81. Mehregan AH, Pinkus H (1965): Life history of organoid nevi. Special reference to nevus sebaceus of Jadassohn. Arch Dermatol 91: 574-588. Mollica F, Pavone L, Nuciforo G (1974): Linear sebaceus nevus syndrome in a newborn. Am J Dis Child 1285368-871. Monk BE, Vollum DI (1982): Familial nevus sebaceus. J R SOCMed 75:660-661. Pack GT, Sunderland DA (1941): Naevus unius lateris. Arch Surg 43:341-375.
667
Pavone L, Curatolo P, R i m R, Micali G, Incorpora G, Garg BP, Dunn DW, Dobyns WB (1991): Epidermal Nevus Syndrome: a neurological variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy. Neurology 41:266-27 1. Rogers M, McCrossin I, Commens C (1989): Epidermal nevi and the epidermal nevus syndrome. J Am Acad Dermatol20:476-488. Sahl WJ, Jr. (1990):Familial nevus sebaceus of Jadassohn: occurrence in three generations. J Am Acad Dermatol 222353454. Solomon LM (1975): Epidermal Nevus Syndrome. Mod Rob1 Paediatr 17:27-30. Solomon LM, Esterly NB (1975): Epidermal and other organoid nevi. Curr Probl Pediat 6:l-55. Solomon LM, Fretzin DF, Dewald RL (1968): The epidermal nevus syndrome. Arch Dermatol97:273-285. Swint RB, Klaus SN (1970): Malignant degeneration of an epithelial nevus. Arch Dermatol 101:56-58.
