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]
Entire Body System
- Pain
Treatment Most cases of palmoplantar keratodermas are permanent, but the thickened skin can be treated so it’s less painful and less noticeable. [footfiles.com]
As far as the back pain and day to day pain that is always there just from the disease I smoke Marijuana for mild pain and the depression that comes along with the disease. [netmums.com]
A painful paronychia develops with no evidence of bacterial or fungal infection. [pcds.org.uk]
We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019 [patientslikeme.com]
There was no history of consanguinity or of recurrent painful paronychia or abnormality in sweating. The patient denied any history of deafness, diminution of vision, redness, or watering of the eyes. [ncbi.nlm.nih.gov]
- El Salvador
El Salvador : La Libertad, San Salvador, etc. Equatorial Guinea : Malabo, etc. Eritrea : Asmara, etc. Estonia : Haapsalu, Kuressaare, Narva, Pärnu, Saaremaa, Tallinn, Tartu, etc. Ethiopia : Addis Ababa, Bahir Dar, Gondar, etc. [maria-online.com]
- Asymptomatic
It was largely asymptomatic; however, brisk walking caused excessive sweating, pain, and widening of the fissures on the soles of the feet. He was unable to walk barefooted. [ispub.com]
Skin
- Sweating
The classical triad of onychodystrophy, universal hypotrichosis, and palmoplantar hyperkeratosis with normal sweating and teeth indicated a diagnosis of Clouston syndrome. [ncbi.nlm.nih.gov]
Diffuse non-epidermolytic palmoplantar keratoderma (NEPPK) is a rare condition in which individuals have thickened, yellowish skin over their palms and soles, thickened nails and suffer from excessive sweating. [news-medical.net]
It seems likely that the changes of stratum corneum in PPKB may obstruct the sweating and induce the miliaria rubra like changes. [bmcdermatol.biomedcentral.com]
[…] glands resulting in decreased or absent sweating, heat intolerance, and recurrent hyperpyrexia. [idoj.in]
Increased sweating ( hyperhidrosis ) is quite common and there is a tendency to fungal and bacterial infections of the feet. [dermnetnz.org]
- Hyperhidrosis
The hyperhidrosis may respond to aluminum chloride-containing products or other therapeutic options of hyperhidrosis. [bmcdermatol.biomedcentral.com]
Finally, patients may have malodorous hyperhidrosis. [emedicine.medscape.com]
Diffuse palmoplantar keratoderma is distinct from palmoplantar keratoderma (KERATODERMA, PALMOPLANTAR), as the former exhibits autosomal dominant inheritance and hyperhidrosis is frequently present. [tititudorancea.com]
It is distinguished from the other PPKs in terms of transgressive soft hyperkeratosis, inflammatory episodes and hyperhidrosis, as well as by its non-progressive nature. [okina.univ-angers.fr]
- Blister
Abstract Keratin gene mutations affecting nonhelical head and tail domains are not usually associated with prominent skin blistering and keratin filament clumping. [ncbi.nlm.nih.gov]
Patients may have blistering in affected areas triggered by trauma, such as friction, however the blistering often appears to improve with age. [genedx.com]
Caution is advised if the patient has a blistering form, since large erosions may occur with oral retinoids therapy. [firstskinfoundation.org]
B Painful focal plantar keratoderma w/associated erythema& blistering 29. Pachyonychia Congenita 30. Howel–Evans syndrome. Focal palmoplantar keratoderma inassociation with carcinoma of the esophagus. 31. Focal type 1. [slideshare.net]
recurrent blistering following minor physical trauma Palmoplantar Bullous vesicles, patchy hyperkeratosis Birth-Early Childhood No No M, B No Disintegration of the basal and suprabasilar cells, intralepidermal blistering with evidence of cytolysis of [dermatologyadvisor.com]
- Palmoplantar Keratosis
A simple classification incorporates 3 factors: specific form and structure and distribution of the palmoplantar keratosis, presence of associated skin and other tissue (such as nails, hair, mucous membranes) disease in sites other than the palms and [firstskinfoundation.org]
In punctuate PPK, multiple tiny punctuate keratosis over the entire palmoplantar surface starting from the lateral edge of the digits is seen. [12] It is not essential that all the clinical features are expressed in early childhood. [ijpd.in]
This sequence-tagged site segregates with the autosomal dominant human disease focal nonepidermolytic palmoplantar keratosis (NEPKK; "tylosis"), which is associated with an increased risk of oesophageal cancer. [cancer-genetics.org]
Features that are similar in both PL and H.M syndromes include palmoplantar keratosis and progressive early onset periodontal destruction. [omicsonline.org]
- Plantar Hyperkeratosis
CONSUMERS: Click here for the Consumer Version Topic Resources Palmoplantar keratodermas are rare inherited disorders characterized by palmar and plantar hyperkeratosis. Most palmoplantar keratodermas are not severe and are autosomal dominant. [merckmanuals.com]
hyperkeratosis, e.g. of weight-bearing skin; severe forms are associated with constricting digital bands, leading to autoamputation; treated by regular hyperkeratosis reduction, keratolytic ointments (e.g. 5–10% salicylic acid in white soft paraffin) [medical-dictionary.thefreedictionary.com]
Diffuse, yellowish palmar and plantar hyperkeratosis (‘keratoderma’) with a red border. Patients may have blistering in affected areas triggered by trauma, such as friction, however the blistering often appears to improve with age. [genedx.com]
The main aspect of PPK is thickening and hyperkeratosis of the palmar and plantar skin, that may be hereditary or acquired; diffuse, focal, or punctuate; and transgrediens or progrediens. [omicsonline.org]
Psychiatrical
- Anger
France : Aix-en-Provence, Ajaccio, Alsace, Angers, Annecy, Antibes, Aquitaine, Arles, Armagnac, Auch, Auvergne, Avignon, Avoriaz, Bastia, Bayonne, Beaune, Besançon, Biarritz, Bonifacio, Bordeaux, Boulogne-sur-Mer, Briançon, Brittany, Burgundy, Cabourg [maria-online.com]
Neurologic
- Abnormal Gait
Oftentimes, keratodermas create walking difficulties, as the pain causes an abnormal gait. Children and adults with Palmoplantar keratodermas are more susceptible to foot odor and fungal infections like Athlete’s Foot. [footfiles.com]
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
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]
Clinically, inherited PPK can be divided into 3 forms: diffuse, striated, and punctate. 3 The pathogenesis of PPK remains unknown, and the treatment is purely symptomatic; there is no definitive treatment or cure. 4 Treatment modalities have consisted [mdedge.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]
Early treatment with acitretin in childhood may allow patients to have normal adult dentition. [emedicine.medscape.com]
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]
[…] 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]
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]
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]