Presentation
The present patient is a 47-year-old-man with PPK who suffered from metastatic non-small-cell carcinoma of the lung. [ncbi.nlm.nih.gov]
Subsequently, a thick yellowish hyperkeratosis appeared and expanded to attain the present status. [indianpediatrics.net]
Lower panel : A similar phenotypic appearance were present in all family members. [bmcdermatol.biomedcentral.com]
All individuals who have inherited an AQP5 mutation will present with this rare skin condition. [news-medical.net]
Workup
Many melanin granules in basal cells HID syndrome, CIE Sepsis workup none Meleda type Mal de Meleda, MDM, Meleda Disease, Keratosis Palmoplantaris Transgradiens of Siemens 757.4 Inhabitants of the island of Mljet off the Dalmation Coast SLURP-1, Ly-6/ [dermatologyadvisor.com]
Treatment
[…] action=getRecordDetail&idt=21343636 Other title Surgical treatment of keratosis palmaris in Mal de Meleda (en) Author MARCHAC, A1 ; BLANCHET-BARDON, C2 ; REVOL, M1 ; SERVANT, J. [pascal-francis.inist.fr]
Early treatment with acitretin in childhood may allow patients to have normal adult dentition. [emedicine.medscape.com]
The treatment of Diffuse Hereditary Palmoplantar Keratoderma is based upon the cause of the condition. Symptomatic treatment of the various abnormalities associated with this form of keratoderma may be undertaken. [dovemed.com]
Etretinate and acitretin are effective, but as treatment must be continued lifelong, systemic retinoids should be used only in the most severe cases as side effects are problematic. [skincareguide.ca]
Prognosis
When PPK is associated with malignancy the prognosis is poor. Patients with these conditions should undergo frequent medical examinations, which should include chest radiography and cytologic examination of the sputum. [ncbi.nlm.nih.gov]
Prognosis - Diffuse palmoplantar keratoderma- Bothnian type Not supplied. Treatment - Diffuse palmoplantar keratoderma- Bothnian type Not supplied. Resources - Diffuse palmoplantar keratoderma- Bothnian type Not supplied. [checkorphan.org]
It may be the only change or a part of a more widespread dermatosis or even a manifestation of an internal malady. [1] The hereditary disorders present distinctive differences in their clinical features, mode of inheritance, associated defects and prognosis [ijdvl.com]
Etiology
The exact etiology and pathogenesis of this syndrome are still not fully understood. Several factors were attributed to the etiology of PLS-periodontitis including microbiologic, immunologic, and genetic. [omicsonline.org]
This would benefit identification of an etiological gene by localizing it to a smaller interval. [circ.ahajournals.org]
Epidemiology
Mutations in keratin K9 in kindreds with epidermolytic palmoplantar keratoderma and epidemiology in Northern Ireland. J Invest Dermatol 111:1207–1209, 1998. PubMed CrossRef Google Scholar 4. [link.springer.com]
Epidemiologic surveys in young individuals have been performed in many parts of the world and among individuals with a widely varied background. [omicsonline.org]
Disorder Synonyms PPK Column* ICD-9 Selected Epidemiologic Features Genetic Cause Syndromic Associations Distinguishing features Age of onset (for disorder manifistation) Erythema Transgrediens Other Epidermal Features** Systemic Features*** Relevant [dermatologyadvisor.com]
Epidemiological data are limited, but there is evidence to support geographical clustering, for example in northern Italy, 8 and this may suggest areas of high prevalence. [circ.ahajournals.org]
Prevention
Currently, Diffuse Hereditary Palmoplantar Keratoderma is a genetic disorder that cannot be prevented. [dovemed.com]
Summary of "Low-dose isotretinoin prevents digital amputation in loricrin keratoderma (Vohwinkel syndrome with ichthyosis)." No Summary Available Affiliation Journal Details This article was published in the following journal. [bioportfolio.com]
[…] for Diffuse palmoplantar keratoderma, Bothnian type includes the 2 symptoms listed below: * Thickened skin on palms * Thickened skin on soles Causes - Diffuse palmoplantar keratoderma- Bothnian type * Thickened skin on palms * Thickened skin on soles Prevention [checkorphan.org]
Emollients Use plenty of grease or other thick emollient to soften the dry skin to prevent fissures. Soak in warm water with emulsifying ointment for 10 minutes. [novatretin.co.nz]
Use of properly fitted footwear and socks can prevent fissuring of the sole, by protecting them from environmental insults and providing occlusive effects. [icliniq.com]