Widespread porokeratotic adnexal ostial nevus: Clinical features and proposal of a new name unifying porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus - 24/08/11

Doi : 10.1016/j.jaad.2009.03.036

Deborah S. Goddard, MD ^a, Maureen Rogers, MD ^b, Ilona J. Frieden, MD ^a, Alfons L. Krol, MD ^c, Clifton R. White, MD ^c, Anu G. Jayaraman, MD ^d, Leslie Robinson-Bostom, MD ^e, Anna L. Bruckner, MD ^f, Beth S. Ruben, MD ^a,^g,^⁎
^a Department of Dermatology, University of California, San Francisco, California
^b Department of Dermatology, The Children’s Hospital at Westmead, Sydney, Australia
^c Department of Dermatology, Oregon Health and Science University, Portland, Oregon
^d Park Dermatology Associates, Easton, Massachusetts
^e Department of Dermatology, The Warren Alpert Medical School of Brown University, Providence, Rhode Island
^f Departments of Dermatology and Pediatrics, Stanford University, Stanford, California
^g Department of Pathology, Dermatopathology Section, University of California, San Francisco, California

Correspondence to: Beth S. Ruben, MD, Departments of Dermatology and Pathology, Dermatopathology Section, University of California, San Francisco, 1701 Divisadero St, Third Floor, San Francisco, CA 94143.

Abstract

Porokeratotic eccrine ostial and dermal duct nevus and a similar condition, porokeratotic eccrine and hair follicle nevus, are rare disorders of keratinization with eccrine and hair follicle involvement. We describe the clinical features in 5 patients, all of whom had widespread skin involvement following the lines of Blaschko. Two patients presented with erosions in the newborn period as the initial manifestation of their disease; one had an associated structural anomaly, unilateral breast hypoplasia; and one adult had malignant transformation in the nevus with development of multifocal squamous cell carcinomas. Three patients had histologic involvement of both acrosyringia and acrotrichia. Based on the observation of overlapping histologic features, we propose the name “porokeratotic adnexal ostial nevus” to incorporate the previously described entities porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus.

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Key words : malignant transformation, neonatal erosions, porokeratotic eccrine and hair follicle nevus, porokeratotic eccrine ostial and dermal duct nevus

Abbreviations used : PAON, PEHFN, PEODDN, SCC

Plan

Case reports

Case 1

Case 2

Summary of histopathology for cases 3 to 5

Discussion

Clinical findings

Clinical differential diagnosis

Histopathology and rationale for unifying PEODDN and PEHFN as one disease: PAON

Histogenesis

Treatment

	Dr Jayaraman was a resident in the Department of Dermatology, Brown Medical School, and Dr Bruckner was a fellow in the Department of Dermatology, University of California, San Francisco, when this case series was initiated.
	Funding sources: None.
	Conflicts of interest: None declared.
	Reprints not available from the authors.

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Vol 61 - N° 6

P. 1060.e1-1060.e14 - décembre 2009 Retour au numéro

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Widespread porokeratotic adnexal ostial nevus: Clinical features and proposal of a new name unifying porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus - 24/08/11

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