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Grand Round Cases : 2011 Academic Year Last Updated: Sep 4th, 2011 - 20:38:38


Title: Lesions on the Neck
Presenter: John Stoner, D.O.
Dermatology Program: PCOM
Program Director: Tanya Ermolovich
Submitted on: Sep 2, 2011

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CHIEF COMPLAINT:  The patient presented to our office complaining of an asymptomatic “rash” on the neck and axillae that has been present for a few years. The lesions do not wax or wane.

CLINICAL HISTORY:

Signs and symptoms:  No itching, burning, or bleeding. The lesions are getting more red.

Previous Treatment:  None

Other information: 

PHYSICAL EXAM:

Multiple pink to flesh colored papules coalescing into a plaque across the front and sides of the neck and bilateral anterior axillae

Left Axilla


Lesions on neck


Close up of lesion on neck

LABORATORY TESTS:

DERMATOHISTOPATHOLOGY:

Right neck: “Multiple basaloid proliferations arranged in nests and comma-shaped configurations, localized to the upper dermis, with no connection to the epidermis.”

H&E - 4X


H&E - 10X

DIFFERENTIAL DIAGNOSIS:

1.   Nevus Comedonicus
2.   Irritant Dermatitis
3.   ILVEN
4.   Verruca Vulgaris
5.  


SCROLL DOWN FOR ANSWER AND DISCUSSION.


CORRECT DIAGNOSIS:

Eruptive Syringomas

DISCUSSION:

Eruptive syrinogmas were first described by Jacquet and Darier, in 1987, as a rare variant of syringomas. Friedman and Butler proposed a classification of syringomas as four variants: a localized form, a familial form, a form associated with Down syndrome, and a generalized form that encompassed multiple and eruptive syringomas. Historically, syringomas have been described as neoplasms with eccrine duct differentiation. Recently, clinicopathologic observations suggest some syringomas result from autoimmune inflammation that triggers a limited, reactive proliferation of the eccrine ducts and surrounding fibrous tissue. Eruptive syringomas share histological features with syringomas but have a distinct clinical presentation.
Eruptive syringomas appear suddenly and sometimes in successive crops on the neck, chest, axillae, upper arms and periumbillically. Typically, they present in a bilateral, symmetrical distribution, but there have been reports of unilateral, unilateral nevoid, bathing trunk and generalized distributions. As opposed to the very common presentation of localized eyelid syringomas, eruptive syringomas present uncommonly, with less than 100 reported cases in the literature. Both entities appear more commonly in women. Eruptive syringomas are generally seen around puberty or in adolescents in contrast to eyelid syringomas, which have a peak incidence between the third and fourth decades. Syringomas tend to be asymptomatic but pruritus has been sometimes associated with eruptive syringomas.
A few familial cases of eruptive syringomas have been reported in the literature. They are also seen in the Nicolau-Balus syndrome, which is characterized by eruptive syringomas, milia and atrophodermia vermiculata.

TREATMENT:

Surgically, light electrodesiccation with curettage, cryotherapy, scissors excision, dermabrasion or laser can be used with variable effectiveness. Chemically, topical or systemic retinoids can be used with variable success. Furthermore, it has been reported that the combination of trichloacetic acid and carbon dioxide laser destruction produces a favorable outcome. Interestingly, a case report using 1% topical atropine in aqueous solution in a case of pruritic eruptive syringomas yielded alleviation of the pruritus and a discrete size reduction of lesions. Topical atropine is believed to be effective due to its ability to inhibit sweat secretion given the eccrine sweat duct differentiation of these tumors. Despite numerous treatment options, effective options are limited since the tumors are located in the dermis and the risk of recurrence is high.

REFERENCES:

1. Soler-Carrillo J, et al. Eruptive syringoma: 27 new cases and a review of the literature. JEADV 2001;15:242-246.
2. Chandler WM, Bosenber MW. Autoimmune acrosyringitis with ductal cysts: reclassification of a case of eruptive syringoma. J Cutan Pathol 2009;36:1312-1315.
3. Sanchez TS, Dauden E. Eruptive pruritic syringomas: Treatment with topical atropine. J Am Acad Dermatol 2001;44:148-149.
4. Frazier CC, et al. The treatment of eruptive syringomas in an African American patient with a combination of trichloacetic acid and CO2 laser destruction. Dermatol Surg 2001;27:489-492.

Additional Comment:


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