Symptoma

Linear Nevus Sebaceus Syndrome

Nevus Sebaceus of Jadassohn

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]

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]

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]

  • Short Stature

    In children, growth rates may be slow, ultimately resulting in short stature. Affected individuals may be prone to fractures. [aaatobabyz.com]

  • Collapse

    No pressure was used in order to avoid collapse of the vessels. Statistical Analysis Descriptive and univariate analysis using the SPSS 20 software were performed. [karger.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]

  • Bone Pain

    A special type of this syndrome is associated with hypophosphatemia and vitamin D-resistant rickets, which manifests with bowing of the legs, bone pain, height loss, and multiple fractures of the ribs and bones. [news-medical.net]

  • 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]

    There is a report on initial presentation as neonatal alopecia [ 5 ]. [omicsonline.org]

  • Myoclonic Jerking

    Patient 1 The patient is an 11-month-old female infant who presented at 1 day of age with myoclonic jerks in both arms. A computed tomographic scan performed at that time showed right hemimegalencephaly. [archivesofpathology.org]

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]

  • Hyponatremia

    PubMed CrossRef Google Scholar Yu TW,Tsau YK, Young C, Chiu HC, Shen YZ (2000) Epidermal nevus syndrome with hypermalanosis and chronic hyponatremia. Pediatr Neurol 22: 151–154. [link.springer.com]

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]

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 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]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

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 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]

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]

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]

We hope that this case report will lead to further investigations in the pathophysiology and genetics of this disorder. [content.iospress.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 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 - 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]

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]

Current medical research has not established a way of preventing a Nevus Sebaceous. [dovemed.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]

For those who do choose to reduce the appearance of their nevus sebaceous for cosmetic purposes or to prevent malignant growths, laser treatment can be an effective option. [healthline.com]