Nail dystrophy affects both toes and fingers; it can be present at birth or developed during early childhood. Palmoplantar keratoderma becomes apparent in later childhood. [accessanesthesiology.mhmedical.com]

[…] hyperkeratosis-spastic paralysis syndrome Powell-Venencie-Gordon syndrome Prevalence: Inheritance: Autosomal dominant Age of onset: All ages ICD-10: - OMIM: 148360 UMLS: C1835671 C2931828 MeSH: C536153 GARD: 3095 MedDRA: - The documents contained in this web site are presented [orpha.net]

This condition can present a diagnostic challenge for clinicians as the clinical presentation can be relatively subtle. [statpearls.com]

Maintenance therapy was required to prevent relapse.[5] This case is presented for its clinical rarity. [ijdvl.com]

• Hyperkeratosis

  Fitzsimmons-McLachlan-Gilbert syndrome Focal Palmoplantar and Gingival Keratosis Hyperkeratosis-Hyperpigmentation Syndrome Hypotrichosis-Osteolysis-Periodontitis-Palmoplantar Keratoderma Syndrome Judge Misch Wright Syndrome Keratoderma Palmoplantaris [rgd.mcw.edu]

  Palmar hyperkeratosis Figure 5. Family tree showing affected members in different generations Figure 6. Histological sections of the skin biopsy of the lesions of palmar and plantar hyperkeratosis Figure 7. [pubs.sciepub.com]

  Focal acral hyperkeratosis exhibits only hyperkeratosis, whereas acrokeratoelastoidosis has the additional finding of elastorrhexis. [mdedge.com]

  Acanthosis nigricans Confluent and reticulated papillomatosis Callus Ichthyosis acquisita Arsenical keratosis Chronic scar keratosis Hyperkeratosis lenticularis perstans Hydrocarbon keratosis Hyperkeratosis of the nipple and areola Inverted follicular [sapientiait.com]

  Histopathological examination of biopsy specimen showed hyperkeratosis, hypergranulosis, acanthosis, elongation of rete ridges and sparse dermal mononuclear cell infiltrate. [ijdvl.com]

• Keratosis

  Inverted follicular keratosis Lichenoid keratosis Multiple minute digitate hyperkeratosis PUVA keratosis Reactional keratosis Stucco keratosis Thermal keratosis Viral keratosis Warty dyskeratoma Waxy keratosis of childhood other hypertrophy: Keloid Hypertrophic [sapientiait.com]

  […] sole of foot 37136002 attributes - group3 Associated morphology Hyperkeratosis 26996000 Finding site Skin structure of palmar area of hand 70887009 parents Autosomal dominant hereditary disorder 11164009 Inherited disorder of keratinization 254214009 Keratosis [findacode.com]

  Transgrediens Keratoderma-Ichthyosis-Deafness Syndrome, Autosomal Recessive Keratosis Palmaris et Plantaris with Clinodactyly keratosis palmoplantaris striata + mal de Meleda mutilating palmoplantar keratoderma with periorificial keratotic plaques + Naegeli-Franceschetti-Jadassohn [rgd.mcw.edu]

  Carcinoma of the oesophagus with keratosis palmaris et plantaris (tylosis): a study of two families. Quart. J. [link.springer.com]

  Cutis Current and Past Issues For Authors CME COVID-19 Updates Diseases & Conditions Acne Actinic Keratosis Aesthetic Dermatology Atopic Dermatitis Autoimmune Diseases Contact Dermatitis Hair & Nails Melanoma Nonmelanoma Skin Cancer Pigmentation Disorders [mdedge.com]

• Palmoplantar Keratosis

  Hereditary Palmoplantar Keratosis, Current Genetics in Dermatology, Dr. Naoki Oiso (Ed.), ISBN: 978-953-51-0971-6, In Tech. [pubs.sciepub.com]

• Skin Disease

  Conclusion The KPP 1 is a rare inherited skin disease. Its association with an increased risk of tumors, neurological, rheumatological and other cutaneous disorders is known. [pubs.sciepub.com]

• Spastic Paralysis

  SNOMED CT code SNOMED code 785725008 name Palmoplantar keratoderma, spastic paralysis syndrome status active date introduced 2019-07-31 fully specified name(s) Palmoplantar keratoderma, spastic paralysis syndrome (disorder) synonyms Palmoplantar keratoderma [findacode.com]

  […] and soles and/or slowly progressive spastic paralysis, predominantly affecting the lower limbs. [orpha.net]

  paralysis syndrome, Powell-Venencie-Gordon syndrome See more Don’t fight Keratoderma palmoplantar spastic paralysis alone. [rareguru.com]

  Preferred Label : powell venencie gordon syndrome; MeSH synonym : punctate keratoderma and spastic paralysis; keratoderma and spastic paralysis; [cismef.org]

In addition to obtaining a skin biopsy and cultures, workup may sometimes include colonoscopy, and extensive blood and urine screening. Pathophysiology PG is now considered to be an aberrant and possibly autoreactive immune response. [medicaljournals.se]

However, devising an appropriate treatment strategy is essential. [medicaljournals.se]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

No exploration or treatment was carried out. The research of similar cases in the family had revealed the presence of the KPP 1 in many other family members over four generations. [pubs.sciepub.com]

Differential diagnosis considered include corns, calluses, secondary syphilis, AIDS associated keratoderma, Reitre′s syndrome, Bazex syndrome and arsenical keratoses.[4] Prior to the advent of retinoids there was no satisfactory treatment for punctate [ijdvl.com]

PMID: 18389286Free PMC Article Prognosis Takigawa T, Tanaka M, Nakahara S, Sugimoto Y, Ozaki T Eur Spine J 2008 Sep;17 Suppl 2:S331-7. Epub 2008 Apr 4 doi: 10.1007/s00586-008-0663-4. PMID: 18389286Free PMC Article [ncbi.nlm.nih.gov]

There are several lines of evidence supporting an immunologic etiology of PG. [medicaljournals.se]

Pathophysiology Mutations in two different genes KRT1 and MPZ on 12q.13 and 1q23 appear to be responsible for this particular association. [accessanesthesiology.mhmedical.com]

Its pathophysiology is still unclear. However the combination of impairment of genetic and environmental factors is most likely. The transmission is often autosomal dominant, rarely autosomal recessive. [5, 6, 7]. [pubs.sciepub.com]

Thus, there is strong correlative evidence in support of PG having an underlying immunologic mechanism; however, its exact pathophysiology is not well understood. [medicaljournals.se]

Maintenance therapy was required to prevent relapse.[5] This case is presented for its clinical rarity. [ijdvl.com]

[…] nuclear ribonucleoprotein (snRNP) particles.[9] Scl-70 antibodies interfere with DNA replication by binding to Topoisomerase I[10], and anti-centromere antibodies affect cell division by binding to centromeres during interphase. [11] Antibodies to Jo-1 prevent [statpearls.com]

The chronic nature of the disease usually requires long-term maintenance therapy to prevent relapses from occurring. [medicaljournals.se]