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Grand Round Cases : 2009-10 Academic Year Last Updated: Jul 8th, 2013 - 01:13:07


Title: Itchy Brown Patch on Axilla
Presenter: Krina Chavda, D.O., Suzanne Sirota Rozenberg, D.O.
Dermatology Program: St. Johns Episcopal Hospital, Far Rockaway, NY
Program Director: Marvin Watsky, D.O.
Submitted on: Oct 18, 2008

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CHIEF COMPLAINT:  Itchy brown patch on left axilla.

CLINICAL HISTORY:

Signs and symptoms:  A 6 years old African American male presented to our clinic with the above chief complaint. As per his father the patient developed this patch approximately 4 to 5 months prior to his visit to us. He states that it recently started increasing in size.

Previous Treatment:  None

Other information:  Patient was on Trileptal for seizure disorder. Also had recently developed flaccid paraparesis for which he had an appointment scheduled for Pediatric neurologist.

PHYSICAL EXAM:

A 6 years old African American male with verrucous hyperpigmented pruritic linear plaques distributed over left axilla and left upper chest along the lines of Blaschko.


LABORATORY TESTS:

None

DERMATOHISTOPATHOLOGY:

Hyperkeratosis, papillomatosis, and acanthosis with rete ridge elongation in a psoriasiform pattern.



DIFFERENTIAL DIAGNOSIS:

1.   Lichen Striatus
2.   Inflammatory linear verrucous epidermal nevus
3.   Epidermal Nevi
4.   Epidermal Nevus Syndrome
5.   Incontinentia pigmenti


SCROLL DOWN FOR ANSWER AND DISCUSSION.


CORRECT DIAGNOSIS:

Epidermal Nevus Syndrome

DISCUSSION:

Epidermal Nevus Syndrome also known as inflammatory linear verrucous epidermal nevus (ILVEN) syndrome is a rare neurocutaneous disorder characterized by specific skin lesions with significant involvement of the nervous, ophthalmologic, and skeletal systems. It represents a dysembryogenesis with both ectodermal and mesodermal malformations occurring in a regional pattern.
There are four clinical variants of this syndrome
-Linear Sebaceous Nevus(LSN)
-Linear nevus comedonicus (NC)
-Linear epidermal nevus (LEN)
-Inflammatory linear verrucous epidermal nevus (ILVEN).
ILVEN is a linear, persistent, pruritic plaque, usually first noted on a limb in early childhood. ILVEN is characterized by tiny, discrete, erythematous, slightly warty papules, which tend to coalesce in a linear formation.

Other key features of this syndrome are involvement of central nervous system characterized by mental retardation, Seizures, hemiparesis, paraparesis, sensorineural deafness, cerebral hemangiomas. Seizures are reported in 75% of patients and the morphology of the seizures varies from infantile spasms or focal motor seizures to generalized tonic or tonic-clonic seizures. In some children, seizures are drug resistant and may result in progressive mental retardation.
Skeletal involvement is associated with location of nevi and may include hemihypertrohpy, kyphoscoliosis, Vitamin D-resistant rickets and foot/ankle defrormities.
Eye involvement is due to extension of nevus to lid and bulbar conjunctiva which may lead to corneal opacity, blindness or nystagmus.
In rare cases variety of neoplasms are associated with this syndrome which include Wilm’s tumor, astrocytoma, rhabdomyosarcoma, syringocystadenoma papilliferum and salivary gland adenocarcinoma.

Laboratory data:
-Skin biopsy: Reveals hyperkeratosis, papillomatosis, and acanthosis with rete ridge elongation in a psoriasiform pattern.
-EEG findings are abnormal in approximately 90% of patients. In almost all patients who had focal paroxysmal electroencephalographic abnormalities, the epileptiform focus was ipsilateral to the major skin lesions.
-MRIs can be used to evaluate intracranial involvement. MRIs may show cerebral atrophy, dilated ventricles, hemimegalencephaly, pachygyria, or enlarged white matter.
-Serum calcium/phosphorus. Patient with epidermal nevus syndrome have hypophosphatamia and excision of nevus or supplemental phosphate with vitamin D may help halt progression of bone and CNS changes.

TREATMENT:

Topical steroids, topical lactic acid in propylene glycol, surgical removal, dermabrasion, Co2 laser. Also referral to neurologist, ophthalmologist and orthopedics to manage other associated problems.

REFERENCES:

Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. Mar 1989;20(3):476-88.
Altman J, Mehregan AH. Inflammatory linear verrucose epidermal nevus. Arch Dermatol. Oct 1971;104(4):385-9.
Fink-Puches R, Soyer HP, Pierer G, Kerl H, Happle R. Systematized inflammatory epidermal nevus with symmetrical involvement: an unusual case of CHILD syndrome?. J Am Acad Dermatol. May 1997;36(5 Pt 2):823-6.
Golitz LE, Weston WL. Inflammatory linear verrucous epidermal nevus. Association with epidermal nevus syndrome. Arch Dermatol. Oct 1979;115(10):1208-9.

Additional Comment:


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