Multiple eruptive verrucous dermatofibromas in the setting of lupus erythematosus and epidermal nevus

Document Type

Conference Proceeding

Publication Date

6-2017

Publication Title

J Am Acad Dermatol

Abstract

A 43-year-old African American woman with a history of systemic lupus erythematosus on hydroxychloroquine and prednisone presented for a several year history of progressive hyperpigmented nodules on all four extremities, some of which were tender and pruritic, as well as a life-long history of thickened skin on her right palm and third volar finger and a “birth-mark” on her right arm and chest. Examination revealed multiple hyperpigmented dome-shaped nodules, several of which were tender, some with a central hyperkeratotic core, involving the right upper extremity, bilateral lower extremities and left flank. Hyperpigmented patches were also noted in a blaschkolinear distribution extending from the right chest to the right shoulder and down the right upper extremity, with a thickened hyperkeratotic skin-colored linear plaque on the right palm and third volar digit. Excisional biopsy of a hyperkeratotic nodule revealed a broad dermal proliferation of spindled and stellate fibroblasts with collagen fibrosis, prominent overlying follicular induction, acanthosis, marked hyperkeratosis, and peripheral trapping of collagen. The diagnosis of multiple eruptive verrucous dermatofibromas in the setting of systemic lupus erythematosus was made, with concomitant epidermal nevus. The syndrome of multiple eruptive dermatofibromas (MEDFs) is a rare clinical entity often reported in the setting of immunosuppression or autoimmune disease, most commonly systemic lupus erythematosus. In the absence of a known systemic trigger, the finding of MEDFs warrants further workup to rule out an underlying autoimmune or immunosuppressive process. To the authors’ knowledge, a verrucous or hyperkeratotic variant has not yet been described, posing an interesting question as to the significance of the concomitant epidermal nevus in our patient. Dermatofibromas are difficult to treat. Observation alone may be the best available therapy, though surgical or energy-based modalities can be utilized for symptomatic lesions.

Volume

76

Issue

6 Suppl 1

First Page

AB172

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