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Linear Nevus Sebaceus Syndrome

Nevus Sebaceus of Jadassohn


Presentation

  • Thus, increased androgen sensitivity may be present within this lesional skin at all times during life.[nature.com]
  • The lesion is usually present at birth, slowly enlarges with age, and typically grows most rapidly during the pubertal hormonal surge.[ncbi.nlm.nih.gov]
  • If it changes noticeably, malignancy may be suspected, especially if any of the following are present: a highly irregular border, an uneven (pebbly) surface, or a mixture of colors (especially black, gray, or blue).[medical-dictionary.thefreedictionary.com]
  • Present at birth, the condition initially consists of a single hairless patch of orange-yellow skin. During adolescence, the lesion may become warty, bumpy or scaly.[ozarkderm.com]
  • Epidermal nevi are usually present at birth (congenital), although they might not be identified until later during childhood or after puberty.[aaatobabyz.com]
Short Stature
  • In children, growth rates may be slow, ultimately resulting in short stature. Affected individuals may be prone to fractures.[aaatobabyz.com]
Small Eyes
  • Eye symptoms may include abnormally small eyes, lipodermoid scleral tumors (yellowish-white, fatty, non-cancerous scleral tumors), corneal opacity (cloudiness of the front part of the eye), eye calcifications , and coloboma (a structural defect of the[xpertdox.com]
Leg Pain
  • Individuals with Schimmelpenning syndrome may also develop vitamin D-resistant rickets, a condition characterized by bow deformities of the legs, pain in the legs and progressive softening of the bone structure.[aaatobabyz.com]
  • Individuals with nevus sebaceus syndrome may also develop vitamin D-resistant rickets, a condition characterized by bow deformities of the legs, pain in the legs and progressive softening of the bone structure.[rarediseases.org]
Alopecia
  • Differential Diagnosis (Other conditions with similar appearance) Aplasia cutis congenita Juvenile xanthogranulomas Mastocytomas Congenital triangular alopecia Diagnosis Key Points *Diagnosis based on characteristic appearance of lesions *Biopsy and other[ozarkderm.com]
  • When the scalp is involved, large lesions may be present and patchy areas of hair loss (alopecia) may occur. Sebaceous nevi can be prominent and easily noticeable at birth or be subtle and easily missed.[aaatobabyz.com]
  • […] can only reach the papillary dermis and are therefore not recommended because of the possibility of malignant degeneration of remaining cells in the lower dermis. 6 Nevus sebaceus is a rare congenital abnormality that can lead to cosmetic deformity, alopecia[ncbi.nlm.nih.gov]
  • It may appear as a yellow or tan waxy patch of alopecia at birth. During puberty the lesion becomes raised, thick, and verrucous. It is usually treated with local excision.[medical-dictionary.thefreedictionary.com]
  • Alopecia is frequently associated with scalp lesions. Maternal hormones briefly increase the prominence during infancy.[dermatologyadvisor.com]
Cutis Laxa
  • Autosomal recessive cutis laxa syndrome revisited. Eur J Hum Genet. 2009 Sep. 17(9):1099-110. [Medline]. [Full Text]. Mitchell AL, Schwarze U, Jennings JF, Byers PH. Molecular mechanisms of classical Ehlers-Danlos syndrome (EDS).[medscape.com]

Workup

  • However, the patient refused additional biopsy and further workup to determine histologic subtype of the nevus.[idoj.in]
  • Evaluation Nevus sebaceus is a clinical diagnosis; additional workup is typically unnecessary.[ncbi.nlm.nih.gov]

Treatment

  • To the authors' knowledge, this report represents the largest surgical series and suggests a reliable approach to the treatment of the cutaneous manifestations of this syndrome.[ncbi.nlm.nih.gov]
  • Treatment *Monitoring lesions for development of tumors is imperative *As long as nevi do not change, treatment is typically not required *Typical treatment consists of removal Nevus sebaceous is typically not treated, as they do not generally cause issues[ozarkderm.com]
  • Lenalidomide for the treatment of resistant discoid lupus erythematosus. Arch Dermatol. 2009 Mar. 145(3):303-6. [Medline]. [Full Text]. Panjwani S. Early diagnosis and treatment of discoid lupus erythematosus.[medscape.com]
  • Treatment Options The surgical option of choice is excision. Physical treatment with photodynamic therapy (PDT), CO2 laser, and dermabrasion has been reported, but these are not preferred treatment modalities.[dermatologyadvisor.com]
  • All patients were treated with expansion of the adjacent texture, color, and adnexal matched skin as the primary modality of treatment.[asps.confex.com]

Prognosis

  • Prognosis The prognosis depends on the severity of the clinical manifestations and the extent of the multisystem involvement. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Prognosis - Nevus sebaceus of Jadassohn Not supplied. Treatment - Nevus sebaceus of Jadassohn Not supplied. Resources - Nevus sebaceus of Jadassohn Not supplied.[checkorphan.org]
  • (Outcomes/Resolutions) The prognosis of Nevus Sebaceous is excellent on its complete excision and removal However, if it turns into a cancerous tumor, then the prognosis mostly depends upon the stage of the tumor and health status of the individual Additional[dovemed.com]
  • Prognosis The prognosis of nevus sebaceus depends on whether it is benign or malignant at the time of diagnosis.[ncbi.nlm.nih.gov]
  • Nevus Sebaceous Prognosis The prognosis of this disorder is good, however, approxiamtely 1% of the patients experience malignant transformation, generally in adulthood or adolescence.[patienthelp.org]

Etiology

  • Etiology LNSS is a sporadic disease. It is hypothesized that the epidermal nevus syndromes are the result of genetic mosaicism involving a dominant gene.[orpha.net]
  • Etiology Schimmelpenning syndrome is caused by sporadic (not inherited) somatic mutations in a cell belonging to the ectodermal germline in the embryo. This is a post-zygotic mutation, and usually affects the HRAS, KRAS and NRAS genes.[news-medical.net]
  • Etiology Pathophysiology The cause of this condition is unknown, but it is possibly linked to postzygotic somatic gene mutation involving PTCH gene. Maternal human papillomavirus (HPV) may be a risk factor.[dermatologyadvisor.com]
  • Other investigations for the etiology of hemiplegia (hematological, immunological and infectious) were normal.[indianpediatrics.net]
  • Etiology Cutaneous lesions represent postzygotic somatic cell genetic mosaicism in most instances. Some well-defined syndromes have been characterized by known gene defects. Pathophysiology Pathophysiology for most syndromes is unknown.[dermatologyadvisor.com]

Epidemiology

  • Summary Epidemiology The incidence of sebaceous nevi at birth is estimated at 1 in 1000 but the prevalence of these lesions in association with other anomalies, as in the case of LNSS, varies between case reports to around 1:10,000.[orpha.net]
  • EPIDEMIOLOGY Epidermal nevi occur in approximately 1 to 3 per 1000 live births; males and females tend to be equally affected [ 2 ].[uptodate.com]
  • Image from the Dermatology Atlas Epidemiology Estimated prevalence is 0.3% of all newborns worldwide, with an equal sex distribution [ 2 ].[patient.info]
  • Epidemiology Nevus sebaceous occurs in approximately 0.3% of all newborn infants [1]. Both sexes are equally affected [5]. There is no racial and or ethnic predominance [2].[austinpublishinggroup.com]
Sex distribution
Age distribution

Pathophysiology

  • Etiology Pathophysiology The cause of this condition is unknown, but it is possibly linked to postzygotic somatic gene mutation involving PTCH gene. Maternal human papillomavirus (HPV) may be a risk factor.[dermatologyadvisor.com]
  • Keloids: pathophysiology and management. Dermatol Online J. 2007 Jul 13. 13(3):9. [Medline]. van Leeuwen MC, van der Wal MB, Bulstra AE, et al. Intralesional cryotherapy for treatment of keloid scars: a prospective study.[medscape.com]
  • Pathophysiology Pathophysiology for most syndromes is unknown. However genetic defects for some well-recognized conditions have recently been discovered.[dermatologyadvisor.com]
  • We hope that this case report will lead to further investigations in the pathophysiology and genetics of this disorder.[content.iospress.com]
  • Pathophysiology In nevus sebaceus, postzygotic somatic mutations may result in various clinical expressions of mosaicism. Mutations in pluripotential cells may give rise to hamartomas with multiple cell lines.[emedicine.medscape.com]

Prevention

  • Prevention - Nevus sebaceus of Jadassohn Not supplied. Diagnosis - Nevus sebaceus of Jadassohn Not supplied. Prognosis - Nevus sebaceus of Jadassohn Not supplied. Treatment - Nevus sebaceus of Jadassohn Not supplied.[checkorphan.org]
  • Current medical research has not established a way of preventing a Nevus Sebaceous.[dovemed.com]
  • OP onset is often prevented using a tetravalent human papillomavirus (HPV) vaccine. However, since LEN is not an infectious pathology, there is no preventive treatment ever described [ 6 ].[hindawi.com]
  • Previous studies reported as much as a 10% risk of developing BCCs in nevus sebaceous, leading to the recommendation of excision to prevent “skin cancer”. However, it is now thought that less than 1% of lesions actually develop BCCs.[healio.com]
  • Thus prevention is not yet an option. Excision of the lesion is the mainstay of treatment.[ijdpdd.com]

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