Linear Systematized Porokeratosis—A Rare Case and Dermoscopic Clues to Diagnosis

Nayak, Manoj Kumar; Dhanta, Aditi; Hazarika, Neirita; Kumar, Arvind

doi:10.4103/idoj.idoj_419_20

Cited by 3 publications

(9 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is the rarest subtype of porokeratosis (estimated incidence 1:200,000) and considered to be a mosaic form of DSAP [373,374]. It predominantly affects children and newborns, but may also develop in adults, including the elderly [375][376][377][378]. A slight female predominance has been reported [78].…”

Section: Linear Porokeratosis (Lp) 61 Clinical Presentationmentioning

confidence: 99%

“…Raised, irregularly shaped pigmented or nonpigmented macules with a blaschkoid distribution and surrounded with cornoid lamellae usually develop on the extremities (Figure 10) [114,379], whereas facial, genital and plantar distribution is rare [52,378,380]. The lesions are usually unilateral, yet bilateral or generalised LPs have also been also reported [52,114,377,[381][382][383]. The plaques are anhidrotic and alopecic [384] and rarely may start as erosions [376].…”

Section: Linear Porokeratosis (Lp) 61 Clinical Presentationmentioning

confidence: 99%

“…There are two reports on phalangeal nail and bone abnormalities [387,391] and pseudoainhum [392] associated with LP. This subtype is regarded to be a form of post-zygotic cutaneous mosaicism and should be differentiated at the clinical level from other Blaschko-linear papulokeratotic dermatoses, both congenital and acquired, including inflammatory linear verrucous epidermal nevus (ILVEN), porokeratotic eccrine ostial and dermal duct nevus (PEODDN), lichen striatus, linear lichen planus, linear Darier's disease, linear psoriasis, linear incontinentia pigmenti, elastosis perforans serpiginosa, ichthyosis linearis circumflexa and linearly arranged viral warts [377,378,384,393]. Liver disease [394] and Bardet-Biedl syndrome [395] were suggested to be linked to LP.…”

Section: Genetics and Epigeneticsmentioning

confidence: 99%

“…In dermoscopy, linear porokeratosis may feature a white to yellow keratotic rim surrounded by brown dots or clods or white areas (Figure 11) [404,405]. Some pigmented lesions may display a blackish aspect of the inner annular ring, grey clods and hair casts in the proximity of the cornoid lamella [377]. The central aspect of the lesion was reported to feature hyperpigmented structureless area or grey-to-brown lines reticular with grey clods [377].…”

Section: Dermatoscopymentioning

confidence: 99%

See 3 more Smart Citations

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

Pietkiewicz,

Korecka,

Salwowska

et al. 2023

Metabolites

View full text Add to dashboard Cite

Porokeratosis is a heterogeneous group of keratinising disorders characterised by the presence of particular microscopic structural changes, namely the presence of the cornoid lamella. This structure develops as a consequence of a defective isoprenoid pathway, critical for cholesterol synthesis. Commonly recognised variants include disseminated superficial actinic porokeratosis, disseminated superficial porokeratosis, porokeratosis of Mibelli, palmoplantar porokeratosis (including porokeratosis palmaris et plantaris disseminata and punctate porokeratosis), linear porokeratosis, verrucous porokeratosis (also known as genitogluteal porokeratosis), follicular porokeratosis and porokeratoma. Apart from the clinical presentation and epidemiology of each variant listed, this review aims at providing up-to-date information on the precise genetic background, introduces imaging methods facilitating the diagnosis (conventional and ultraviolet-induced fluorescence dermatoscopy, reflectance confocal microscopy and pathology), discusses their oncogenic potential and reviews the literature data on the efficacy of the treatment used, including the drugs directly targeting the isoprenoid–mevalonate pathway.

show abstract

Section: Linear Porokeratosis (Lp) 61 Clinical Presentationmentioning

confidence: 99%

Section: Linear Porokeratosis (Lp) 61 Clinical Presentationmentioning

confidence: 99%

Section: Genetics and Epigeneticsmentioning

confidence: 99%

Section: Dermatoscopymentioning

confidence: 99%

See 2 more Smart Citations

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

Pietkiewicz,

Korecka,

Salwowska

et al. 2023

Metabolites

View full text Add to dashboard Cite

show abstract

“…1,2 LP demonstrates heterogeneous clinical features of mosaicism with unilateral blaschkoid segmental linear plaques on the trunk and extremities appearing congenitally or arising in infancy or childhood. 1,3 A combination of heterozygous germline and second-hit postzygotic somatic (in utero) loss-of-function mutations in mevalonate pathway genes (PMKV, MVK, MVD) have been implicated. 4 Familial (autosomal dominant) inheritance or de novo mutations are possible.…”

mentioning

confidence: 99%

A Review of Therapeutic Options for Linear Porokeratosis

Verma,

M. K. Shalabi,

C. Hartmann

et al. 2024

J of Skin

View full text Add to dashboard Cite

Linear porokeratosis (LP) is a rare and uncommon variant of porokeratosis presenting as linear annular papules or plaques along Blaschko’s lines. It may arise spontaneously, although evidence of genetic predisposition exists via point mutations in the mevalonate pathway of cholesterol biosynthesis. Malignant transformation is the most serious complication of this chronic, progressive dyskeratosis. We review the currently available treatment options for LP, including topical, systemic, laser, photodynamic, and surgical therapies.

show abstract

Syringotropic Lichen Planus: An Unusual Presentation of a Common Dermatosis – A Report of 2 Cases

Mohaghegh,

Khodashenas,

Saber

et al. 2024

Case Rep Dermatol

View full text Add to dashboard Cite

Introduction: Lichen planus (LP) is a chronic inflammatory dermatosis that causes plaques and itchy papules on the skin, as well as erosion and ulcers in the mucous membranes. LP is characterized by a dense dermal T-cell infiltration. Perieccrine inflammation can be seen in a variety of dermatoses, but genuine lymphocyte permeation of the secretory coil or lymphocytic syringotropism is a rare sign that is typically seen in mycosis fungoides. Case Presentation: In this study, we present 2 uncommon instances of lymphocytic syringotropism in LP. Histopathological examination revealed dense T-cell infiltration and lymphocytic involvement of eccrine glands, confirming syringotropism. Conclusion: Lymphocytic syringotropism is an uncommon finding in LP. Its presence broadens the histopathological spectrum of LP and highlights the need to differentiate it from lymphoproliferative disorders like mycosis fungoides.

show abstract

Linear Systematized Porokeratosis—A Rare Case and Dermoscopic Clues to Diagnosis

Cited by 3 publications

References 6 publications

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

A Review of Therapeutic Options for Linear Porokeratosis

Syringotropic Lichen Planus: An Unusual Presentation of a Common Dermatosis – A Report of 2 Cases

Contact Info

Product

Resources

About