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]

• Recurrent Bacterial Infection

  Papillon-Lefevre syndrome is an autosomal recessive keratoderma associated with inflammation of the gums and loss of teeth, sparse hair and recurrent bacterial infections of the skin and internal organs. [dermnetnz.org]

• Abdominal Lymphadenopathy

  In 2001 the patient revealed inguinal and abdominal lymphadenopathy first diagnosed as extramedullary plasmacytoma and then as multiple myeloma on the basis of bone marrow infiltration and monoclonal gammopathy. [ncbi.nlm.nih.gov]

• Aspiration

  Fine needle aspiration from the pre-auricular swelling was consistent with epidermoid cyst. [ncbi.nlm.nih.gov]

• Sputum

  Patients with these conditions should undergo frequent medical examinations, which should include chest radiography and cytologic examination of the sputum. [ncbi.nlm.nih.gov]

• Acrocyanosis

  Four members of a family, in which 8 suffered from diffuse palmoplantar keratoderma associated with an uncommon form of acrocyanosis, are reported. [ncbi.nlm.nih.gov]

  […] creases Abnormal plantar creases Absence of fingerprints with congenital milia syndrome Acquired acanthosis nigricans Acquired perforating pseudoxanthoma elasticum Acquired pseudoxanthoma elasticum Acral Darier's disease Acral peeling skin syndrome Acrocyanosis [icdlist.com]

  […] mepacrine, proguanil, mexiletine, methyldopa, lithium, venlafaxine, gold, fluorouracil, bleomycin, hydroxyurea, practolol and doxorubicin. 5 Systemic diseases like myxedema, diabetes mellitus, chronic lymphedema and other circulatory disorders such as acrocyanosis [jemds.com]

  Nielsen, P G : Diffuse palmoplantar keratoderma associated with acrocyanosis: a family study. Acta derm.-vener. (Stockh.) 69, 156–161 (1989) Google Scholar 25. [link.springer.com]

• Palpitations

  The cardiac abnormality (Fig 2 ) was not clinically manifest until the patient was ≈15 years of age, and onset was usually with palpitation or syncope. [circ.ahajournals.org]

• Strabismus

  There are other less common abnormalities reported in Clouston syndrome, which include conjunctivitis, strabismus, congenital cataract, oral leucoplakia, diffuse eccrine poromatosis, sensorineural hearing loss, thickened skull bones, and tufting of the [ncbi.nlm.nih.gov]

  Sensorineural deafness, polydactyly, syndactyly, clubbing of fingers, mental retardation, dwarfism, photophobia, and strabismus are associated features. Clouston syndrome is mapped to 13q11. [emedicine.medscape.com]

• Hypoplastic Nails

  (scleroatrophic changes on the dorsal aspect of the hands and hypoplastic nails in addition to the PPK). [indianpediatrics.net]

  […] sclerotylosis") is an autosomal dominant genodermatosis, characterized by the triad of congenital diffuse scleroatrophy of the distal extremities, mild or lamellar keratoderma of the palms and to a lesser extent the soles, presence from birth and ridging or hypoplastic [e-ijd.org]

• Sparse to No Eyebrows

  The eyelashes are short and sparse, and the eyebrows as well as axillary and pubic hair are also sparse or absent (1), as in our case. During infancy, the nails are typically milky white, gradually thickening throughout childhood. [ncbi.nlm.nih.gov]

• Short Nails

  Hair, nail and teeth abnormalities Hidrotic ectodermal dysplasia is an autosomal dominant keratoderma associated with very sparse or absent hair on the head, face and body and thickened, short nails which are easily lost. [dermnetnz.org]

• Dry, Scaly Skin

  Loricrin keratoderma is a variant of Vohwinkel syndrome which also results in dry, scaly skin over the rest of the body ( ichthyosis ). [dermnetnz.org]

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]

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]

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]

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]

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]

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]

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]