Nevus sebaceus Q82.5

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

Nevus sebaceus of Jada; Nevus sebaceus syndrome; organoid nevus; sebaceous epitheliomatous nevus; sebaceous nevus; Sebaceous nevus

History
This section has been translated automatically.

Jadassohn, 1895; Robinson, 1932

Definition
This section has been translated automatically.

Organoid hemartoma of the skin (organoid epidermal nevus), which mainly affects the surface epithelium, hair follicles and the sebaceous and sweat glands.

Occurrence/Epidemiology
This section has been translated automatically.

In a larger study (Yang CC et al.), in 265 children (age 1-12 years) with capillitium neoplasms the sebaceous nevus was represented by 68%, followed by melanocytic nevi (6.2%) and juvenile xanthogranuloma (6.2%). Infant hemangiomas were found in 41% of this patient group.

Etiopathogenesis
This section has been translated automatically.

This is based on postzygotic HRAS mutations (95%) and KRAS mutations (5%) (see RAS below), regulatory genes that code for the Ras protein. This protein is a central regulator of various signal transduction pathways that regulate growth and differentiation processes (see also RASopathies)

Manifestation
This section has been translated automatically.

  • Congenital
  • Mainly sporadic occurrence. (Very rare) familial occurrence has been reported (Benedetto L et al. 1990; Sahl WJ Jr 1990).
  • No gender bias.

Localization
This section has been translated automatically.

90% of the malformations affect the capillitium (about 45% the parietal region); less frequently, the face, rertroauricular region, neck, trunk or genitals are affected. An absolute (negligible) rarity is the occurrence in the mucous membrane region.

Clinical features
This section has been translated automatically.

Age-related clinical picture:

  • 1st birth:
  • At this time, depending on the localization, a solitary, completely symptomless, oval to bizarrely configured (elongated with uneven spurs), whitish-yellowish or skin-colored, waxy, always hairless spot or a corresponding, slightly raised, surface-smooth plaque appears. Since the sebaceous nevus occurs most frequently on the capillitium, the main clinical symptom is alopecia. Important here is the clinical finding of"absence of follicles" (Note: the follicular organs are not developed in the area of the lesion, no formation of a hair follicle!)
  • 2nd childhood:
  • No active surface growth during childhood. Size growth analogous to body growth.
  • 3. peripubertal:
  • In the puberty phase and later in younger adults a growth in thickness of the usually completely symptom-free skin change occurs. Development of a still hairless, beet-like, warty plaque of now yellow-red or yellow-brown (growth of sebaceous glands during puberty) colour and spongy, firm consistency.
  • 4. late adulthood:
  • Increasingly verrucous surface formation of the lesion, occasionally skin-coloured or blue-black coloured nodules (see complications below)
  • Arrangement: Mostly bizarrely configured, also linear arrangement.
  • The Schimmelpenning-Feuerstein-Mims Syndrome is a neuroectodermal symptom complex with extensive sebaceous nevus (also multiple), malformations and dysplasia of the eyes, skin, brain, skeleton and heart.
  • The SCALP syndrome describes a syndrome with the constellation: "Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome (melanocytic giant nevus)".
  • Didymosis aplasticosebacea is the very rare concordant encounter of aplasia cutis circumscripta with a sebaceous nevus.

Histology
This section has been translated automatically.

Convolute malformed hair follicles with mostly numerous, mature sebaceous gland lobules, which are grouped like grapes around the usually dilated excretory duct. Frequent proliferation of abortive, apocrine or eccrine differentiated glands.
  • Prepubertal: The surface epithelium is slightly acanthotic, in the upper dermis immature sebaceous gland complexes and infundibulum structures are visible. Hair roots and terminal hairs are completely missing.
  • Postpubertal: Epidermal hyperplasia is present as well as large sebaceous gland complexes with differently developed apocrine glands. Hair follicles are missing.

Differential diagnosis
This section has been translated automatically.

The differential diagnosis depends (see clinical picture) on the age of the patient.

Infancy and early childhood:

  • Alopecia areata: Previous hairiness. Follicles preserved.
  • Aplasia cutis congenita (ACC): at birth, frequent erosion or ulcer formation with consecutive scarring (this course is known to the parents).
  • Tinea capitis: acute onset; scaling, follicles preserved. Fungus detection.

Postpubertal:

  • At this stage, clinical diagnosis is easy. Differential diagnostic are benign adnexal tumors to consider.

Late adulthood:

Complication(s)
This section has been translated automatically.

In about 10-30% of the cases, development of various benign but also malignant adnexal tumors, such as (the percentages refer to neoplasms in 707 cases of sebaceous nevus, cited in Idriss MH et al:

Therapy
This section has been translated automatically.

Excision should be attempted at an age when children tolerate (possible) local anesthesia. Partial excision possible. For smaller hamartomas excision in toto. Here, the cosmetic aspect must also be taken into account! Controlled waiting" can also be accepted. In case of development of secondary tumours (90% are benign), their isolated excision (e.g. in very large organoid naevi) is also justified.

Progression/forecast
This section has been translated automatically.

Favourable; in a part of the hamartomas benign neoplasms of the adnexa such as trichoblastomas and syringocystadenomas develop. Less frequent is the development of malignant tumours such as basal cell carcinomas (a part of these cases published as basal cell carcinoma is to be addressed as trichoblastoma), ductal (apocrine) adenocarcinomas, porocarcinomas, sebaceous gland carcinomas, spinocellular carcinomas and keratoacanthomas.

Notice! Benign or malignant transformations are not to be expected until middle to high adulthood, not in childhood or adolescence.

Literature
This section has been translated automatically.

  1. Benedetto L et al (1990) Familial nevus sebaceus.J Am Acad Dermatol 23:130-132.
  2. Happle R et al (2001) Didymosis aplasticosebacea: coexistence of aplasia cutis congenita and nevus sebaceus may be explained as a twin spot phenomenon. Dermatology 202:246-248.
  3. Hügel H et al (2003) Ductal carcinoma arising from a syrigocystadenoma papilliferum in a nevus sebaceus of Jadassohn. At J Dermatopathol 25: 490-493
  4. Idriss MH et al (2014) Secondary neoplasms associated with nevus sebaceus of
  5. Jadassohn: a study of 707 cases. J Am Acad Dermatol 70: 332-337
  6. Jaqueti G et al (2000) Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases. At J Dermatopathol 22: 108-118
  7. Jadassohn J (1895) Remarks on the histology of systematized nevi and on "Sebaceous gland nevi? Arch Dermatol Syphil (Berlin) 88: 355-394
  8. Lam J et al (2008) SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutiscongenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity. J Am Acad Dermatol 58:884-888.
  9. Moody BR, Hurt MA (2004) Surgical management of the cutaneous manifestations of linear nevus sebaceus syndrome. Plast Reconstr Surgery 113: 799-800
  10. Munoz-Perez MA et al (2002) Sebaceus naevi: a clinicopathologic study. J Eur Acad Dermatol Venereol 16: 319-324
  11. Sahl WJ Jr (1990) Familial nevus sebaceus of Jadassohn: occurrence in three generations.
  12. J Am Acad Dermatol 22:853-854.
  13. Santibanez-Gallerani A et al (2003) Should Nevus Sebaceus of Jadassohn in Children be Excised? A Study of 757 Cases, and Literature Review. J Craniofac Surgery 14: 658-660
  14. Saphiro M et al (1999) Spiradenoma arising in nevus sebaceus of Jadasson: Case report and literature review. Arch J Dermatopathy 21: 462-467
  15. Yang CC et al (2014) Neoplastic skin lesions of thescalp in children: a retrospective study of 265 cases in Taiwan. Eur J Dermatol24:70-75
  16. Zutt M et al (2003) Mold-penning flint mims syndrome with hypophosphatemic rickets. Dermatology 207: 72-76

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020