Introduction: Schimmelpenning syndrome may encompass abnormalities of the cardiovascular, skeletal, ophthalmologic and urogenital systems. Nevus sebaceous is a hallmark finding and ophthalmologic findings are seen in 59% of the cases which include colobomas and choristomas. Case Summary: A 1-month-old female presented with a verrucous plaque over the scalp and right zygomatic area upon birth. Physical examination reveals a linear yellowish alopecic verrucous plaque over the right frontal region, yellowish alopecic verrucous plaque topped with a skin colored papule over the right zygomatic region, conjunctival mass over the right eye and an atrophic patch with areas of circular erosion over the right occipital region. Ballard score and reflexes were appropriate for gestational age. Newborn screening was normal and otoacoustic-emission-test revealed no hearing loss. She was referred to an ophthalmologist and was assessed to have a lipodermoid, right upper eyelid and optic nerve coloboma. Cranial CT scan is unremarkable. Histopathology showed an increase in number of sebaceous glands with malformed hair units. She was managed holistically and does not have seizures and no secondary development of tumors in the nevus sebaceous. Conclusion Schimmelpenning syndrome is usually associated with the clinical triad of nevus sebaceous, mental retardation and seizures. In this case, seizures were absent, however, there is an associated lipodermoid, right upper eyelid and right optic nerve coloboma. In addition, she also presented with aplasia cutis congenita. Hence, it is important to look for other manifestations when patients present with nevus sebaceous because management requires collaboration with different specialties.
Nevus, Sebaceous of Jadassohn

No abstract available.
Animals ; Horns* ; Nevus, Sebaceous of Jadassohn*

A 5-year-old female presented a 4-year history of generalized well-demarcated asymptomatic brown to dark brown thin verrucous plaques with a Blaschkoid distribution. Histopathology was consistent with an epidermal nevus. Patient was diagnosed to have systematized verrucous epidermal nevus. Due to the extent of the lesions, surgical management was not feasible. Hence acitretin was given which showed partial decrease in the thickness of the lesions.

Inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon variant of verrucous epidermal nevus, clinically characterized by erythematous and hyperkeratotic verrucous papules with a typical linear arrangement. Most reported cases present as solitary lesions, but systematized ILVEN is rare. Epidermal nevus syndrome is a congenital disorder in which epidermal nevi are associated with abnormalities in other organ systems. However, ILVEN has been reported rarely in association with such abnormalities, including those of the skeleton. Here we report a rare case of systematized ILVEN presenting with concomitant mandibular hypoplasia.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Nevus ; Nevus, Sebaceous of Jadassohn* ; Skeleton

Nevus sebaceus of Jadassohn is organoid nevus that tends to develop secondary tumors on the lesion in adulthood. We report a case of verruca vulgaris associated with nevus sebaceus in a 8-year-old girl. Histologic findings showed typical findings of verruca vulgaris such as papillomatosis, hyperkeratosis, and acanthosis with vaculoated cells as well as the findings of nevus sebaceus. To our knowledge, this is the first case report of verruca vulgaris associated with nevus sebaceus in the Korean dermatologic literatures.
Child ; Female ; Humans ; Nevus* ; Nevus, Sebaceous of Jadassohn* ; Organoids ; Papilloma ; Warts*

No abstract available.
Carcinoma, Basal Cell* ; Hamartoma ; Nevus* ; Nevus, Sebaceous of Jadassohn

We report a case of nevus sebaceus of Jadassohn in a 31-year-old female associated with various epidermal appendage tumors, such as syringocystadenoma papilliferum, sebaceus adenoma and eccrine hidrocystoma. We supposed that the multiple lesions seem to support the concept of nevus sebaceus arising from the pluripotential primary epithelial germ.
Adenoma ; Adult ; Female ; Hidrocystoma ; Humans ; Nevus* ; Nevus, Sebaceous of Jadassohn

We observed a case of sebaceous epithelioma associated with a nevus sebaceus in a 25-year-old male. The tumor was bean-sized, dome-shaped nodule on the slightly yellowish plaque of nevus sebaceus. Histopathological finding shows undifferentiated cells which are arranged in a palisade fashion at the periphery of a cell mass and a fairly large number of transitional cells and groups of mature sebaeeous cells.
Adult ; Carcinoma* ; Humans ; Male ; Nevus* ; Nevus, Sebaceous of Jadassohn*

Proliferating trichilemmal cyst(PTC) is a rare benign neoplasm that is thought to be derived from the outer root sheath of anagen hair follicles differentiating toward the infundibular and matrical segments, sebaceous glands, apocrine glands, or acrosyringium. Clinically, it presents as a solitary elevated, lobulated mass on the scalp of elderly women. Histologically, it shows well demarcated multiple lobules of squamous epithelium. The center of the lobule undergoes abrupt keratinization without formation of keratohyalin and foci of calcification are often present in the areas of amorphous keratin. PTC can be associated with sebaceous nevus of Jadassohn, ectopic apocrine sweat glands, spindle cell carcinoma, etc. We report an unusual case of proliferating trichilemmal cyst with trichoepitheliomatous change in a young woman.
Aged ; Apocrine Glands ; Epithelium ; Female ; Hair Follicle ; Humans ; Nevus, Sebaceous of Jadassohn ; Scalp ; Sebaceous Glands ; Sweat Glands

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 70-year-old woman with six neoplastic proliferations including a syringocystadenoma papilliferum, pigmented trichoblastoma, tubular apocrine adenoma, sebaceoma, tumors of follicular infundibulum and superficial epithelioma with sebaceus differentiation arising in a long standing nevus sebaceus on the scalp. Our case is extraordinary because a single nevus sebaceus contained six neoplastic proliferations with differentiation toward the folliculosebaceous-apocrine unit.
Adenoma ; Aged ; Carcinoma ; Female ; Hamartoma ; Humans ; Nevus* ; Nevus, Sebaceous of Jadassohn ; Scalp ; Skin