Porokeratotic Eccrine Ostial Research Articles

Porokeratotic Eccrine Ostial and Dermal Duct Naevus and Aberrantly Regulated Keratinization

Porokeratotic Eccrine Ostial and Dermal Duct Naevus and Aberrantly Regulated Keratinization

Porokeratotic Eccrine Ostial and Dermal Duct Nevus

PEODDN is a rare benign cutaneous disorder that clinically resembles comedo nevus but favors the palms and soles, where pilosebaceous follicles are absent. Widespread involvement along Blaschko's lines can also occur. It is a disorder of keratinization involving the intraepidermal eccrine duct (acrosyringium) and is characterized by eccrine hamartoma and cornoid lamellation in pathology. The patient is a 29-year-old man with an 8-year history of pruritic skin lesions on his right lateral ankle. In the pathologic examination, multiple small epidermal invagination with overlying parakeratotic cornoid lamellation, loss of granular layer, and few dyskeratotic cells at the base of epidermal invagination are revealed. After clinic-pathologic correlation, the diagnosis of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) was made. Late-onset and rare clinical presentation as pruritic lesion are the characteristic features that make this patient an extraordinary presentation of PEODDN.

Palmar purpuric lichen nitidus—clinicopathological and dermoscopic findings

Lichen nitidus (LN) is an uncommon dermatosis of unknown etiology. LN confined to the palms is an unusual presentation and only a few cases have been reported. The clinical features may mimic pompholyx, pitted keratosis, and porokeratotic eccrine ostial and dermal duct nevus. We herein report a rare case of palmar purpuric LN with characteristic dermoscopic findings.

Bilateral systematized porokeratotic eccrine ostial and dermal duct nevus showing aberrant keratin and profilaggrin expression

Bilateral systematized porokeratotic eccrine ostial and dermal duct nevus showing aberrant keratin and profilaggrin expression

Porokeratotic Eccrine Nevus May Be Caused by Somatic Connexin26 Mutations

Porokeratotic eccrine ostial and dermal duct nevus, or porokeratotic eccrine nevus (PEN), is a hyperkeratotic epidermal nevus. Several cases of widespread involvement have been reported, including one in association with the keratitis–ichthyosis–deafness (KID) syndrome (OMIM #148210), a rare disorder caused by mutations in the GJB2 gene coding for the gap junction protein connexin26 (Cx26). The molecular cause is, as yet, unknown. We have noted that PEN histopathology is shared by KID. The clinical appearance of PEN can resemble that of KID syndrome. Furthermore, a recent report of cutaneous mosaicism for a GJB2 mutation associated with KID describes linear hyperkeratotic skin lesions that might be consistent with PEN. From this, we hypothesized that PEN might be caused by Cx26 mutations associated with KID or similar gap junction disorders. Thus, we analyzed the GJB2 gene in skin samples from two patients referred with generalized PEN. In both, we found GJB2 mutations in the PEN lesions but not in unaffected skin or peripheral blood. One mutation was already known to cause the KID syndrome, and the other had not been previously associated with skin symptoms. We provide extensive functional data to support its pathogenicity. We conclude that PEN may be caused by mosaic GJB2 mutations.

Psoriasiform variant of porokeratotic eccrine ostial and dermal duct nevus

International Journal of DermatologyVolume 51, Issue 6 p. 742-743 Correspondence Psoriasiform variant of porokeratotic eccrine ostial and dermal duct nevus Bhavana R. Doshi MD, Bhavana R. Doshi MDSearch for more papers by this authorUday Khopkar MD, DNB, Uday Khopkar MD, DNBSearch for more papers by this author Bhavana R. Doshi MD, Bhavana R. Doshi MDSearch for more papers by this authorUday Khopkar MD, DNB, Uday Khopkar MD, DNBSearch for more papers by this author First published: 21 May 2012 https://doi.org/10.1111/j.1365-4632.2010.04561.xCitations: 1 Conflicts of interest: The authors have no conflict of interest to declare. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume51, Issue6June 2012Pages 742-743 RelatedInformation

Ultrapulse Carbon Dioxide Laser Treatment of Porokeratotic Eccrine Ostial and Dermal Duct Nevus

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare, benign, cutaneous hamartoma. Approximately 45 cases of PEODDN have been reported, with little information regarding treatment. We report a patient with PEODDN treated successfully using an ultrapulse carbon dioxide laser.

Multiple keratotic papules on palm

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare nonhereditary malformation of the eccrine duct. A relationship with linear porokeratosis is not yet established; some consider it as a rare variant of porokeratosis involving the acrosyringium, whereas others consider it a separate entity based on distinctive clinical features and histologic accentuation within ostial structures. A 7-year-old girl presented with multiple asymptomatic keratotic papules over her right palm, present since the age of 6 months. These papules were arranged in a linear distribution over the palm and middle finger of the right hand. Most of the papules were discrete. However, lesions on the middle finger coalesced to form a plaque. Histology revealed a keratin filled deep invagination of the epidermis, notable for a column of parakeratosis ("cornoid lamella"). The dermis was notable for dilated eccrine ducts and absent inflammation. Considering the clinical and histological evidence, a diagnosis of PEODDN was made. Its clinical resemblance to linear lichen planus and linear porokeratosis is discussed. Also, we provide a brief review of this rare condition.

Clinical and histopathological analysis of tumors arising in nevus sebaceous: 16 case reports

Objective To assess the histopathological characteristics of tumors arising in nevus sebaceous.Methods The clinical and pathological data on 16 patients with tumors arising in nevus sebaceous were collected and retrospectively reviewed.Histopathological characteristics were analyzed.Results Of the 16 patients,12 aged ≥ 18 years,4 aged 11 to 17 years; 7 had syringocystadenoma papilliferum(SCAP),4 tubular apocrine adenoma (TAA),3 trichilemmoma,2 basal cell carcinoma (BCC),1 porokeratotic eccrine ostial and dermal duct nevus,and 1 nevus flammeus.Six patients suffered from more than one kind of tumor,of these tumors,concurrent syringocystadenoma papilliferum and apocrine adenoma predominated and were observed in 4 of these patients.Conclusion Any tumor arising from nevus sebaceous should be closely followed up.

Extending the Phenotypic Spectrum of Keratitis‐Ichthyosis‐Deafness Syndrome: Report of a Patient with GJB2 (G12R) Connexin 26 Mutation and Unusual Clinical Findings

Keratitis-ichthyosis-deafness (KID) syndrome is a rare ectodermal dysplasia, characterized mainly by the presence of hyperkeratotic skin lesions, neurosensory hearing loss, and vascularizing keratitis. Most mutations that have been discovered as a cause of KID syndrome are autosomal dominant, found in exon 2 of the Connexin (Cx) 26 gene. A G12R (p.Gly12Arg) is a GJB2 mutation reported in only two patients with KID syndrome to date. This article describes a patient with the G12R mutation and KID syndrome with interesting additional features, which include a porokeratotic eccrine ostial and dermal duct nevus, follicular occlusion triad, and unusual persistent oral mucosal papules. We compare this patient's phenotype with the only two other patients described with the same (G12R) mutation. The phenotypic heterogeneity of KID syndrome, inexplicable according to our current understanding of these proteins, speaks to the complexity of the connexin system and its overlapping expression patterns in different tissues.

Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO laser: A case and brief review

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon disease that presents early in childhood and is characterized by keratotic papules, often in a linear configuration. We describe a 12-year-old girl with characteristic lesions of PEODDN and describe her response to treatment with a combination CO2/Erbium laser. We also briefly review the literature on PEODDN.

Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO 2 laser: A case and review

Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO 2 laser: A case and review

Porokeratotic Eccrine Ostial and Dermal Duct Nevus Showing Partial Remission by Topical Photodynamic Therapy

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon, benign dermatosis and is characterized by asymptomatic grouped keratotic papules and plaques with a linear pattern on the extremities. Various treatments, including topical steroids, topical calcipotriol, topical 5-fluorouracil, retinoid, cryotherapy, and carbon dioxide laser ablation have been tried for PEODDN, but the results were unsatisfactory. Recently, topical photodynamic therapy (PDT) has been shown to be effective for various cutaneous disorders. We report a case of PEODDN showing partial remission with PDT using topical 5-aminolevulanic acid in a 4-year-old girl.

Porokeratotic eccrine ostial and dermal duct nevus

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.

Porokeratotic Eccrine Duct and Hair Follicle Nevus (PEHFN) Associated with Keratitis-Ichthyosis-Deafness (KID) Syndrome

Porokeratotic eccrine ostial and dermal duct nevus is a rare hamartomatous malformation, histologically characterized by cornoid lamellae overlying dilated eccrine ostia. The nevus most commonly presents in the form of multiple filiform keratotic spines in a linear arrangement, usually on the distal extremities. Porokeratotic eccrine and hair follicle nevus is thought to be a variant of porokeratotic eccrine ostial and dermal duct nevus that additionally involves hair follicle infundibula. We report a case of widespread Porokeratotic eccrine and hair follicle nevus that developed in a 15-year-old woman with keratitis-ichthyosis-deafness syndrome.

Porokeratotic eccrine ostial and dermal duct naevus: report of 10 cases

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a congenital hamartoma that has rarely been documented. To describe the clinical and histological features of 10 patients with PEODDN. A retrospective study of 10 patients with PEODDN attended in our paediatric hospital between 1999 and 2009 was performed. The clinical and histopathological features were reviewed. Nine children and one adult were included in the study. In 6 cases the lesion was present at birth and they were unique in 5 cases and systematized in the other 5 cases. Two cases in the same family are reported. The lesions were most commonly found on the extremities, although the palms or soles were involved in 9 cases and only in one case there was not affection of this area. The lesions were mostly asymptomatic but there was moderate itching in two cases. Clinical examination revealed no extracutaneous involvement. PEODDN is a rare disorder that usually appears at birth or early childhood. It has a linear distribution following the Blaschko lines. To our knowledge this is the largest series reported in English literature and we also describe the first family case.

Porokeratotic Eccrine Ostial and Dermal Duct Nevus: A Report of 2 Cases and Review of the Literature

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare disorder of keratinization involving the intraepidermal eccrine duct (acrosyringium). We detail two examples of this unique clinicopathological entity--one with a more typical clinical presentation and one with a solitary lesion and late adult onset. In addition, we discuss the distinctive histologic and immunohistochemical findings and review the literature.

Diffuse Squamous Cell Carcinoma in Porokeratotic Eccrine Ostial and Dermal Duct Nevus

Diffuse Squamous Cell Carcinoma in Porokeratotic Eccrine Ostial and Dermal Duct Nevus

Generalized porokeratotic eccrine ostial and dermal duct nevus associated with deafness

Generalized porokeratotic eccrine ostial and dermal duct nevus associated with deafness

Bowenʼs Disease on Porokeratotic Eccrine Ostial and Dermal Duct Nevus

Bowenʼs Disease on Porokeratotic Eccrine Ostial and Dermal Duct Nevus