Patient Presentation
An otherwise healthy 20-year-old presented with a raised pebbly plaque on the right occipital area of the scalp, which he had since infancy. This plaque became prominent around puberty. He was concerned about the cosmetic disfigurement and risk of malignancy developing in it. No family history of similar lesions was reported. On physical examination, there was 3 cm by 1.5 cm pebbly, linear, orange-to-yellow-colored plaque on the occipital area. The surface of this plaque had a cobblestone pattern, but no outstanding masses inside this plaque. No lymphadenopathy was noted.
What's Your Diagnosis?
Diagnosis: Nevus Sebaceus of Jadassohn (NS)
Jadassohn initially described this condition in 1895 as a malformation of the skin with an excess or deficiency of one or more of the normal mature constituents: hair, glands, epidermis or connective tissue.1 In 1932, Robinson coined the term nevus sebaceus of Jadassohn (NS).2,3 NS presents as a sharply demarcated, oval or linear, yellow-orange, elevated plaque that is usually devoid of hair. It more frequently occurs on the head and neck of infants in up to 95% of cases but may occur occasionally on the trunk.3,4 It is frequently flat and inconspicuous in early childhood, but during adolescence the plaque becomes verrucous with formation of rubbery nodules. Familial forms of NS, as well as NS in association with convulsions and mental retardation, were reported.5,6 On histology, NS is characterized by a variable degree of hyperkeratosis, hyperplasia of the epidermis and abundance of sebaceous glands. All elements of the skin are represented; there are malformed hair follicles, as well as ectopic apocrine glands. It is thought that NS arises from pleuripotential primary epithelial germ cells, which can dedifferentiate into various epithelial tumors. This may explain the reported consequent development of secondary malignancies and benign adnexal tumors.
Prophylactic Surgical Excision
Long considered the treatment of choice, total excision before adolescence has recently been questioned by certain authors.4,7 We will briefly review some of the controversial opinions in the following paragraph. Basal cell carcinoma (BCC) has been reported to develop in scalp location of NS in children, especially after puberty.4,8,9 After 12 years of age, Alessi et al., found “deficit malformation” of the follicles with hyperplasia of epidermis and sebaceous glands in 90% of cases.9 Occasionally, age-independent squamous cell carcinoma can appear. The incidence of malignant degeneration of NS was reported in 0% to 50% of the cases depending on the series.3,4,7,9 More recently, malignant melanoma arising in NS of the scalp in an adult patient,10 and a BCC of NS of the scalp in a 7-year-old boy, were reported.11 In 2000, Cribier et al.,4 reported a series of 596 cases of NS where they analyzed all the surgically excised NS specimens and concluded that more than 90% of tumors occurring in association with NS were actually benign, and only five BCCs were found in this large series. The authors also reported a list of “benign tumors” arising in NS. These include syringocystadenoma papilliferum, trichoblastoma (which may be difficult to differentiate from BCC microscopically), trichilemmoma, sebaceoma, nevocellular nevus and keratoacanthoma. The latter is considered a form of squamous cell carcinoma by some authors, thus a malignant tumor. Other tumors included seborrheic keratosis and viral warts. In a more recent retrospective study of 757 cases of NS in children aged 16 years or younger, Santibanez-Gallerani et al.,7 found no cases of BCC in NS. These authors question the need for prophylactic surgical removal of the NS. Following the above, and according to authors’ interpretations of the data, two opposing opinions have been established. On the one hand, because the potential for malignant degeneration of such tumors can be up to 50%, excision was suggested as the treatment of choice.12 Weng et al.,8 recommended early removal of these lesions in childhood before they enlarge and mature, and sebaceous glands develop. In favor of the prophylactic excision of NS, some authors cited several reasons:
• high rates of malignant transformation, metastases and subsequent deaths
• a significant number of adults develop a benign neoplasm within the NS
• cosmetic and/or symptomatic sequelae, particularly during adolescence
• a defect in the PTCH gene within NS has recently been reported.12
On the other hand, others suggested that in the absence of clinical signs suggestive of malignant transformation, prophylactic surgery of NS in children should not be systematically performed.4,7 Management of NS Until a consensus on the best method of management of NS is found, prophylactic surgery is being frequently performed because of the development of various carcinomas on NS. Reconstruction of postsurgical defects is often challenging. If surgery is to be performed, the primary excision of NS should be carried out with a minimum of 2 mm to 3 mm margins. This is underscored by the malignant potential of the underlying nevus and reflects margins needed to excise 95% of basal cell carcinomas. The excision should be full thickness down through the skin, epidermis, dermis, subcutaneous tissue and underlying supporting fat. The excision should stop at the level of the temporoparietal fascia to preserve any branches of the temporal branch of the facial nerve. The exact size that can be closed primarily is dependent on tissue laxity and how far back from the hairline the lesion is located. We believe the limit of primary closure is a defect that is 1 cm to 1.5 cm in diameter. Care must be taken to avoid placing resorbable sutures around hair follicles. The resultant inflammation from these sutures can lead to alopecia. Because of the favored temporal scalp location of NS, reconstructions must take into account the preservation of hair-bearing skin and the hairline. Davison (personal communication) proposed a standardization of the reconstruction using the temporal rotation flap for NS of the lateral temporal hairline. This flap closes the defect while preserving hair on the scalp and producing an aesthetically pleasing result. This option further obviates any need for skin grafting. If surgery is to be performed, the current accepted approach is full-thickness, complete excision with 2 mm to 3 mm margins, followed by primary reconstruction.8,13,14 Dermaplaning, which has been suggested for superficial NS in the scalp area, leaves residual lesion because NS extends deep to the level of the subcutaneous tissue and involves associated adnexal structures.15
Patient Presentation
An otherwise healthy 20-year-old presented with a raised pebbly plaque on the right occipital area of the scalp, which he had since infancy. This plaque became prominent around puberty. He was concerned about the cosmetic disfigurement and risk of malignancy developing in it. No family history of similar lesions was reported. On physical examination, there was 3 cm by 1.5 cm pebbly, linear, orange-to-yellow-colored plaque on the occipital area. The surface of this plaque had a cobblestone pattern, but no outstanding masses inside this plaque. No lymphadenopathy was noted.
What's Your Diagnosis?
Diagnosis: Nevus Sebaceus of Jadassohn (NS)
Jadassohn initially described this condition in 1895 as a malformation of the skin with an excess or deficiency of one or more of the normal mature constituents: hair, glands, epidermis or connective tissue.1 In 1932, Robinson coined the term nevus sebaceus of Jadassohn (NS).2,3 NS presents as a sharply demarcated, oval or linear, yellow-orange, elevated plaque that is usually devoid of hair. It more frequently occurs on the head and neck of infants in up to 95% of cases but may occur occasionally on the trunk.3,4 It is frequently flat and inconspicuous in early childhood, but during adolescence the plaque becomes verrucous with formation of rubbery nodules. Familial forms of NS, as well as NS in association with convulsions and mental retardation, were reported.5,6 On histology, NS is characterized by a variable degree of hyperkeratosis, hyperplasia of the epidermis and abundance of sebaceous glands. All elements of the skin are represented; there are malformed hair follicles, as well as ectopic apocrine glands. It is thought that NS arises from pleuripotential primary epithelial germ cells, which can dedifferentiate into various epithelial tumors. This may explain the reported consequent development of secondary malignancies and benign adnexal tumors.
Prophylactic Surgical Excision
Long considered the treatment of choice, total excision before adolescence has recently been questioned by certain authors.4,7 We will briefly review some of the controversial opinions in the following paragraph. Basal cell carcinoma (BCC) has been reported to develop in scalp location of NS in children, especially after puberty.4,8,9 After 12 years of age, Alessi et al., found “deficit malformation” of the follicles with hyperplasia of epidermis and sebaceous glands in 90% of cases.9 Occasionally, age-independent squamous cell carcinoma can appear. The incidence of malignant degeneration of NS was reported in 0% to 50% of the cases depending on the series.3,4,7,9 More recently, malignant melanoma arising in NS of the scalp in an adult patient,10 and a BCC of NS of the scalp in a 7-year-old boy, were reported.11 In 2000, Cribier et al.,4 reported a series of 596 cases of NS where they analyzed all the surgically excised NS specimens and concluded that more than 90% of tumors occurring in association with NS were actually benign, and only five BCCs were found in this large series. The authors also reported a list of “benign tumors” arising in NS. These include syringocystadenoma papilliferum, trichoblastoma (which may be difficult to differentiate from BCC microscopically), trichilemmoma, sebaceoma, nevocellular nevus and keratoacanthoma. The latter is considered a form of squamous cell carcinoma by some authors, thus a malignant tumor. Other tumors included seborrheic keratosis and viral warts. In a more recent retrospective study of 757 cases of NS in children aged 16 years or younger, Santibanez-Gallerani et al.,7 found no cases of BCC in NS. These authors question the need for prophylactic surgical removal of the NS. Following the above, and according to authors’ interpretations of the data, two opposing opinions have been established. On the one hand, because the potential for malignant degeneration of such tumors can be up to 50%, excision was suggested as the treatment of choice.12 Weng et al.,8 recommended early removal of these lesions in childhood before they enlarge and mature, and sebaceous glands develop. In favor of the prophylactic excision of NS, some authors cited several reasons:
• high rates of malignant transformation, metastases and subsequent deaths
• a significant number of adults develop a benign neoplasm within the NS
• cosmetic and/or symptomatic sequelae, particularly during adolescence
• a defect in the PTCH gene within NS has recently been reported.12
On the other hand, others suggested that in the absence of clinical signs suggestive of malignant transformation, prophylactic surgery of NS in children should not be systematically performed.4,7 Management of NS Until a consensus on the best method of management of NS is found, prophylactic surgery is being frequently performed because of the development of various carcinomas on NS. Reconstruction of postsurgical defects is often challenging. If surgery is to be performed, the primary excision of NS should be carried out with a minimum of 2 mm to 3 mm margins. This is underscored by the malignant potential of the underlying nevus and reflects margins needed to excise 95% of basal cell carcinomas. The excision should be full thickness down through the skin, epidermis, dermis, subcutaneous tissue and underlying supporting fat. The excision should stop at the level of the temporoparietal fascia to preserve any branches of the temporal branch of the facial nerve. The exact size that can be closed primarily is dependent on tissue laxity and how far back from the hairline the lesion is located. We believe the limit of primary closure is a defect that is 1 cm to 1.5 cm in diameter. Care must be taken to avoid placing resorbable sutures around hair follicles. The resultant inflammation from these sutures can lead to alopecia. Because of the favored temporal scalp location of NS, reconstructions must take into account the preservation of hair-bearing skin and the hairline. Davison (personal communication) proposed a standardization of the reconstruction using the temporal rotation flap for NS of the lateral temporal hairline. This flap closes the defect while preserving hair on the scalp and producing an aesthetically pleasing result. This option further obviates any need for skin grafting. If surgery is to be performed, the current accepted approach is full-thickness, complete excision with 2 mm to 3 mm margins, followed by primary reconstruction.8,13,14 Dermaplaning, which has been suggested for superficial NS in the scalp area, leaves residual lesion because NS extends deep to the level of the subcutaneous tissue and involves associated adnexal structures.15
Patient Presentation
An otherwise healthy 20-year-old presented with a raised pebbly plaque on the right occipital area of the scalp, which he had since infancy. This plaque became prominent around puberty. He was concerned about the cosmetic disfigurement and risk of malignancy developing in it. No family history of similar lesions was reported. On physical examination, there was 3 cm by 1.5 cm pebbly, linear, orange-to-yellow-colored plaque on the occipital area. The surface of this plaque had a cobblestone pattern, but no outstanding masses inside this plaque. No lymphadenopathy was noted.
What's Your Diagnosis?
Diagnosis: Nevus Sebaceus of Jadassohn (NS)
Jadassohn initially described this condition in 1895 as a malformation of the skin with an excess or deficiency of one or more of the normal mature constituents: hair, glands, epidermis or connective tissue.1 In 1932, Robinson coined the term nevus sebaceus of Jadassohn (NS).2,3 NS presents as a sharply demarcated, oval or linear, yellow-orange, elevated plaque that is usually devoid of hair. It more frequently occurs on the head and neck of infants in up to 95% of cases but may occur occasionally on the trunk.3,4 It is frequently flat and inconspicuous in early childhood, but during adolescence the plaque becomes verrucous with formation of rubbery nodules. Familial forms of NS, as well as NS in association with convulsions and mental retardation, were reported.5,6 On histology, NS is characterized by a variable degree of hyperkeratosis, hyperplasia of the epidermis and abundance of sebaceous glands. All elements of the skin are represented; there are malformed hair follicles, as well as ectopic apocrine glands. It is thought that NS arises from pleuripotential primary epithelial germ cells, which can dedifferentiate into various epithelial tumors. This may explain the reported consequent development of secondary malignancies and benign adnexal tumors.
Prophylactic Surgical Excision
Long considered the treatment of choice, total excision before adolescence has recently been questioned by certain authors.4,7 We will briefly review some of the controversial opinions in the following paragraph. Basal cell carcinoma (BCC) has been reported to develop in scalp location of NS in children, especially after puberty.4,8,9 After 12 years of age, Alessi et al., found “deficit malformation” of the follicles with hyperplasia of epidermis and sebaceous glands in 90% of cases.9 Occasionally, age-independent squamous cell carcinoma can appear. The incidence of malignant degeneration of NS was reported in 0% to 50% of the cases depending on the series.3,4,7,9 More recently, malignant melanoma arising in NS of the scalp in an adult patient,10 and a BCC of NS of the scalp in a 7-year-old boy, were reported.11 In 2000, Cribier et al.,4 reported a series of 596 cases of NS where they analyzed all the surgically excised NS specimens and concluded that more than 90% of tumors occurring in association with NS were actually benign, and only five BCCs were found in this large series. The authors also reported a list of “benign tumors” arising in NS. These include syringocystadenoma papilliferum, trichoblastoma (which may be difficult to differentiate from BCC microscopically), trichilemmoma, sebaceoma, nevocellular nevus and keratoacanthoma. The latter is considered a form of squamous cell carcinoma by some authors, thus a malignant tumor. Other tumors included seborrheic keratosis and viral warts. In a more recent retrospective study of 757 cases of NS in children aged 16 years or younger, Santibanez-Gallerani et al.,7 found no cases of BCC in NS. These authors question the need for prophylactic surgical removal of the NS. Following the above, and according to authors’ interpretations of the data, two opposing opinions have been established. On the one hand, because the potential for malignant degeneration of such tumors can be up to 50%, excision was suggested as the treatment of choice.12 Weng et al.,8 recommended early removal of these lesions in childhood before they enlarge and mature, and sebaceous glands develop. In favor of the prophylactic excision of NS, some authors cited several reasons:
• high rates of malignant transformation, metastases and subsequent deaths
• a significant number of adults develop a benign neoplasm within the NS
• cosmetic and/or symptomatic sequelae, particularly during adolescence
• a defect in the PTCH gene within NS has recently been reported.12
On the other hand, others suggested that in the absence of clinical signs suggestive of malignant transformation, prophylactic surgery of NS in children should not be systematically performed.4,7 Management of NS Until a consensus on the best method of management of NS is found, prophylactic surgery is being frequently performed because of the development of various carcinomas on NS. Reconstruction of postsurgical defects is often challenging. If surgery is to be performed, the primary excision of NS should be carried out with a minimum of 2 mm to 3 mm margins. This is underscored by the malignant potential of the underlying nevus and reflects margins needed to excise 95% of basal cell carcinomas. The excision should be full thickness down through the skin, epidermis, dermis, subcutaneous tissue and underlying supporting fat. The excision should stop at the level of the temporoparietal fascia to preserve any branches of the temporal branch of the facial nerve. The exact size that can be closed primarily is dependent on tissue laxity and how far back from the hairline the lesion is located. We believe the limit of primary closure is a defect that is 1 cm to 1.5 cm in diameter. Care must be taken to avoid placing resorbable sutures around hair follicles. The resultant inflammation from these sutures can lead to alopecia. Because of the favored temporal scalp location of NS, reconstructions must take into account the preservation of hair-bearing skin and the hairline. Davison (personal communication) proposed a standardization of the reconstruction using the temporal rotation flap for NS of the lateral temporal hairline. This flap closes the defect while preserving hair on the scalp and producing an aesthetically pleasing result. This option further obviates any need for skin grafting. If surgery is to be performed, the current accepted approach is full-thickness, complete excision with 2 mm to 3 mm margins, followed by primary reconstruction.8,13,14 Dermaplaning, which has been suggested for superficial NS in the scalp area, leaves residual lesion because NS extends deep to the level of the subcutaneous tissue and involves associated adnexal structures.15
