Linear Nevus Research Articles

Solomon's Epidermal Nevus Syndrome (Type: Linear Nevus Sebaceus) and Hypophosphatemic Vitamin D—Resistant Rickets

Epidermal nevus syndrome is very variable in symptoms and associated abnormalities. Synonyms of this syndrome are linear nevus sebaceus syndrome or Schimmelpenning-Feuerstein-Mims syndrome or Solomon syndrome. The combination with vitamin D-resistant rickets is rare and only sporadically described. Less than 10 cases with this combination of symptoms have been described in the literature. We describe a boy suffering from epidermal nevus syndrome (type: nevus sebaceus). This child also presented with severe rickets with hyperphosphaturia, resistant to vitamin D. Our patient was seen in consultation at birth, but after a delay of 4 years we were consulted again for a second opinion and treatment; the vitamin D-resistant rickets was recognized. Treatment with 1,25-dihydroxy vitamin D3 and phosphorus resulted in healing of rickets. Removal of parts of the tumors did not influence the rickets. This is in contrast with a formerly described case. Removal of fibroangiomas led in that case to normalization of the alkaline phosphatase, calcium, and phosphate serum levels. The rickets results from massive phosphate excretion by defective renal tubular reabsorption of phosphate. In all patients described, rickets developed at an early age. Clinical symptoms were marked bone abnormalities, muscle weakness, and bone pain.

Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review.

"Epidermal nevus syndrome" ("ENS") is a neurocutaneous disorder in which epidermal nevi are associated with other abnormalities, most commonly of the skeletal and central nervous systems. We present two cases of epidermal nevus syndrome (ENS) with very different clinical findings. The first case is a newborn with multiple linear epidermal nevi of the trunk and limbs, and several other anomalies, including bony duplications of the lower limbs and hypoplastic left heart syndrome. The second patient, a 6-year-old boy, has a linear nevus sebaceous of the scalp with severe CNS involvement, including generalized seizures, moderate mental retardation, microcephaly, and a left hemiparesis. He also has genitourinary, cardiac, and skeletal defects. These two patients exhibit several abnormalities not previously recognized and illustrate the wide clinical spectrum of "epidermal nevus syndrome." We present a review of the clinical findings in 74 cases of "ENS." Correlation was noted between the presence of skin lesions located on the head and CNS involvement. The wide clinical spectrum of "ENS" as illustrated by these two patients suggests that "ENS" is a causally heterogeneous group of disorders.

Hypophosphatemic vitamin D-resistant rickets and multiple spindle and epithelioid nevi associated with linear nevus sebaceus syndrome

province of Trento (Italy). Am J Dermatopathol 1984; 6(suppl 1):283-8. 9. Doherty VR, MacKie RM. Experienceof a publiceducation program on early detection of cutaneous malignant melanoma. Br Med J 1988;297:388-91. 10. Fixler Zc. Melanoma/skin cancer screeningprogram in a private office setting [Letter]' .T AM ACAD DERMATOL 1985;16:634-5. 11. Arundell FD. Campaigns for the detectionand prevention of melanoma and skin cancers [Editorial]. J AM ACAD DERMATOL 1987;16:406-7. 12. Koh HK, Caruso A, Gage I, et ai. Evaluation of melanoma/skin cancer screening in Massachusetts. Cancer 1990;65:375-9. 13. BologniaJL, BerwickM, Fine.TA. Completefollow-up and evaluationof a skincancer screeningin Connecticut.J AM ACAD DERMATOL 1990;23:1098-106. Brief communications 109

Proteus syndrome: Ultrastructural study of linear verrucous and depigmented nevi

Proteus syndrome is a rare hamartomatous disorder characterized by multifocal overgrowths that can involve any structure of the body. Clinical manifestations include macrodactyly, hemihypertrophy, subcutaneous masses, exostosis, cerebroid thickening of palms and soles, and linear skin lesions. About 50 cases have been described, but the ultrastructural features of the linear skin lesions have not been characterized. We describe the clinical, histologic, and ultrastructural findings for a 30-year-old patient who had a mild form of Proteus syndrome with linear lesions characterized by a mixed pattern of hyperkeratosis and depigmentation. Light microscopy of the linear nevus showed acanthosis and hyperorthokeratosis. Electron microscopy revealed extensive vacuolation at the interface between melanocytes and keratinocytes, with large aggregations of densely packed granules in the intercellular space. Melanocytes showed only slight degenerative changes. An immunohistochemical study of the expression of epidermal growth factor receptors revealed no significant abnormalities.

Linear nevus sebaceous syndrome: Megalencephaly and heterotopic gray matter

A 3-month-old white male with linear nevus sebaceous syndrome (LNSS), facial deformities, brain malformations, and cortical blindness is presented. Facial deformities included left ear hypertrophy and a left-sided hamartomatous neck mass. Magnetic resonance imaging suggested that a central nervous system (CNS) abnormality, involving gray and white matter, should be linked with LNSS. Magnetic resonance imaging was more useful than computed tomography in demonstrating the full spectrum of CNS anomalies, including unilateral lissencephaly, a paucity of white matter, excessive and heterotopic gray matter, apparent left schizencephaly, and unilateral left colpocephaly (dilation of atrium and posterior horn of lateral ventricle). Postmortem examination revealed left hemisphere megalencephaly with a dilated posterior horn of the lateral ventricle. The impressive gray matter heterotopias likely were the source of the patient's seizures and perhaps the ultimate cause of death. Megalencephaly and other deviations of neuronal migration should be considered as a dramatic component of LNSS.

Linear Epidermal Nevus with Acantholytic Dyskeratosis in an Infant

A 7-month-old boy came to use with flat papules and small erosions on the extensor aspect of his left forearm and a linear arrangement of verrucous papules on the dorsum of his left hand. Histological examination revealed hyperkeratosis, parakeratosis, irregular acanthosis, and marked acantholysis from suprabasal through upper epidermis. This is the first reported case of linear epidermal nevus with acantholytic dyskeratosis in Japan.

Linear nevi, hemihypertrophy, connective tissue hamartomas, and unusual neoplasms in children

The association of cutaneous nevi, hemihypertrophy, and connective tissue overgrowth has been described in many patients. We report long-term follow-up in two boys with such association who in addition developed testicular neoplasms. One patient also had progressive spinal cord compression as a result of stenosis of the spinal canal, and required amputation of a severely deformed limb. The importance of careful, long-term management in such patients is emphasized.

Linear Nevus Sebaceous Syndrome

The linear nevus sebaceous syndrome is a rare disorder first described in 1962 in two cases by Feuerstein and Mims. As originally described, it consisted of the triad of the characteristic midline facial linear nevus sebaceous of Jadassohn, seizures, and mental retardation. The authors have followed a patient with this syndrome from birth to age 8 and although he does demonstrate several neurologic and ocular abnormalities, he enjoys normal intelligence and has never suffered seizures. The features of this syndrome and several new ocular findings demonstrated in this case are described. Based on our longterm follow-up of this patient and our review of the reported cases to date, the authors suggest that the triad for this disorder should be changed to include the midline facial linear nevus sebaceous of Jadassohn, neurologic abnormalities which may, but not necessarily, include seizures and mental retardation, and ophthalmologic abnormalities.

A Case of Linear Nevus Sebaceous Syndrome Associated with Early-Infantile Epileptic Encephalopathy with Suppression Burst EIEE

A Case of Linear Nevus Sebaceous Syndrome Associated with Early-Infantile Epileptic Encephalopathy with Suppression Burst EIEE

Linear Nevus Sebaceous Syndrome: Report of a Case and a Review of the Literature

Linear Nevus Sebaceous Syndrome: Report of a Case and a Review of the Literature

Gingival manifestation in linear nevus sebaceous syndrome

Gingival involvement was observed in an 11-year-old female patient with linear nevus sebaceous syndrome. Clinical, histological and scanning-electronmicroscopic findings are described. 4 years after surgical removal, the oral lesion recurred; clinical and histological features are presented. Problems in diagnosis, differential diagnosis and validity of oral involvement in the linear nevus sebaceous syndrome are discussed.

Nevos sebáceo linear de Jadassohn: registro de um caso

É relatado um caso de nevos sebáceo linear de Jadassohn, com sua forma clínica, apresentando a clássica tríade: 1) nevos facial; 2) crises focais; e 3) retardo mental progressivo, aparentemente. Apresenta-se no primeiro estadio de desenvolvimento, quando as glândulas sebáceas estão pouco desenvolvidas, à biopsia de pele. À TAC apresenta atrofias córtico-subcorticais discretas, contrastando com a intensidade do retardo mental e das crises convulsivas. Estas, foram controladas após várias combinações medicamentosas.

Basal cell epitheliomas developing in a localized linear epidermoid nevus

Basal cell epitheliomas developing in a localized linear epidermoid nevus

Linear Eccrine Nevus With Comedones

We report herein a case of a linear nevus comedonicus associated with tumors of sweat gland origin. We propose the term "linear eccrine nevus with comedones" as a suitable title for this disorder. Several similar cases in the literature are reviewed.

Linear Verrucous Nevus

Linear verrucous nevi may be troublesome lesions to extirpate with satisfactory cosmetic results. Conventional practice has been to excise then in full-thickness skin. A simpler technique of shaving them off to a depth between the papillary and reticular dermis and allowing spontaneous re-epithelization from the remaining portions of adnexa is recommended as better and cosmetically more satisfactory.

Linear nevus sebaceus syndrome: A case report

The linear nevus sebaceus syndrome, a neurocutaneous disorder, is characterized by anomalies of ectodermal and mesenchymal structures. We briefly describe the diagnostic features and management of this condition in a 2-year-old child.

Inflammatory Linear Verrucose Epidermal Nevus

We treated three patients with inflammatory linear verrucose epidermal nevus. The lesions were either verrucose or psoriasiform and all had an inflammatory component. Histologic features included psoriasiform acanthosis with spongiosis and parakeratosis. Features that distinguish this entity and the differential diagnoses are discussed.

Linear Nevus Sebaceous Syndrome: Neurological Aspects Documented by Brain Scans Correlated with Developmental History and Radiographic Studies

Linear Nevus Sebaceous Syndrome: Neurological Aspects Documented by Brain Scans Correlated with Developmental History and Radiographic Studies

Generalized Pustular Psoriasis and Pustulation on the Linear Epidermal Nevus

A 9-year-old Japanese girl with generalized pustular psoriasis (GPP) had a linear epidermal nevus (LEN) which exhibited psoriasislike features in a noneruptive phase of GPP. In advance of the exacerbation of the disease, crops of confluent pustules appeared on the linear lesions and afterwards continued during the flare-up. Although several cases of ordinary psoriasis with a LEN presenting inflammatory changes have been reported, it seems that there is only one paper in the literature concerning pustular formation on a LEN in a patient with GPP. This particular phenomenon may be interpreted as a kind of Koebner reaction in a person with active GPP.

Inflammatory linear verrucose epidermal nevus.

A typical case of inflammatory linear verrucose epidermal nevus (ILVEN), with quite a psoriasiform histologic pattern, is reported. Intralesionally administered paramethasone acetate caused a temporary suppression of the clinical and histopathologic inflammatory features. The concept of ILVEN is briefly discussed.