Punctate keratoderma jama dermatology jama network. Palmoplantar keratoderma with deafness is a disorder characterized by skin abnormalities and hearing loss. Punctate palmoplantar keratoderma type i genetic and. Epidermolytic palmoplantar keratoderma heppk hereditary epidermolytic palmoplantar keratoderma or vorners keratoderma is a rare genodermatosis first described by vorner in 1901.
Idiopathic acquired palmoplantar keratoderma in a patient with no. If a person has questions or concerns regarding keratoderma, ask an expert for medical insight. Punctate palmoplantar keratoderma punctate palmoplantar keratoderma is often familial, where it tends to be inherited as an autosomal dominant trait. The prognosis of acquired keratoderma depends upon the severity of the symptoms, the response to treatment, and most importantly upon the severity of the underlying condition who gets acquired keratoderma. I have a female patient also with punctate keratoderma 62 years of age. Case reports epidermolytic palmoplantar keratoderma.
Punctate keratosis of the palms and soles buschkefischerbrauer type synonyms include punctate ppk type i, keratosis punctata palmaris et plantaris, buschkefischerbrauer disease, and keratosis papulosa. Punctate palmoplantar keratoderma type i, also called keratosis punctate palmoplantaris type buschkefisherbrauer, is a rare autosomal dominant hereditary skin disease characterized by multiple hyperkeratotic centrally indented papules that develop in early adolescence or later and are irregularly distributed on the palms and soles. Her father had the same condition and her daughter has also inherited the condition. Palmoplantar keratoderma an overview sciencedirect topics. Findings of discrete hyperkeratotic papules on the palms and soles. Punctate palmoplantar keratoderma pppk is a rare entity with an estimated prevalence rate of about 1. Palmoplantar keratoderma is a term used to define a marked thickening of the skin on the palms and soles, either as a focal entity, or diffuse. Punctate keratoderma definition of punctate keratoderma.
T2 guttate hypopigmentation and punctate palmoplantar keratoderma. Two unrelated patients had numerous palmoplantar music box spine keratotic plugs and pits of 11 and years duration. Unilateral linear punctate palmoplantar keratoderma is not yet reported in the literature. B nonepidermolytic keratoderma with greater thickness in sites of pressure. A 36yearold woman, who had taken acitretin 20 mgday for palmoplantar keratoderma for about 3 months, complained of dysphonia.
She has treatment every 4 weeks she finds this gives her relief, she applys heel balm daily. An 83yearold caribbean female presented with multiple lesions on her hands and feet that appeared about 2 years prior to her presentation figure 1. In focal palmoplantar keratoderma, usually only pressure or friction points are affected. In general, epidermolytic and nonepidermolytic forms cannot be distinguished clinically. Palmoplantar keratoderma is the name given to a group of conditions where there is abnormal thickening of the skin on the palms of the hands and soles of the feet.
In some cases, the presence of a positive family history is not observed. Acquired keratosis keratoderma palmaris et plantaris. Transgradient palmoplantar keratoderma with illdefined margins. Punctate palmoplantar keratoderma can also be secondary to previous arsenic exposure, and in this case usually appear in middle age or later. All content on this website, including dictionary, thesaurus, literature, geography, and other reference data is for informational purposes only. Pdf acquired palmoplantar keratoderma researchgate. The exact etiology of the disorder is not known but a dual influence of. Punctate porokeratotic keratoderma ppk is manifested by multiple, small 0.
Keratosis punctata definition of keratosis punctata by. Palmoplantar keratoderma genetic and rare diseases. Unlike more classic types, the clinical descriptions and photographs are typified by spiny, filiform and acuminate, seedlike, raspiform keratotic lesions. Some individuals are better classified as having porokeratosis palmaris et plantaris disseminata, in which. Such lesions tend to be present by early adulthood. Abstract hereditary palmoplantar keratodermas ppks comprise a large and heterogeneous group of disorders characterized by persistent. She had bilateral vocal fold edema and congestion, consistent with vocal abuse. Ppk can also be a feature of various underlying syndromes. Punctate ppk keratotic papules, some coalescing to form plaques, on the palm a and sole b. Case of punctate palmoplantar keratoderma type i treated with combination of lowdose oral acitretin and topical salicylic acid and steroid. When moisturizing rashes acquire a clearly marked white color. Pdf punctate palmoplantar keratoderma brauerbuschke.
This chapter provides a practical overview of keratoderma, and is set out as below. Reduction of the dose of acitretin to 10 mgday did not produce and. In rare forms of ppk, organs other than the skin may also be affected. Unilateral linear punctate palmoplantar keratoderma sharma. Gudelines on management of palmoplantar keratoderma.
It is a subtype of punctate palmoplantar keratoderma. Current concepts in diagnosing and treating keratoderma. Punctuate keratodermas result in tiny bumps of thickened skin on the palms and soles and usually appears in late childhood. The disease is characterized by the appearance of punctate, rounded grooves on the inner surfaces of both feet from 1 to 8 mm, that is, the areas that closely touch with shoes. Kertatoderma can be inherited, acquired, and rarely, paraneoplastic ie secondary to an internal malignancy. Keratoderma is a term that means marked thickening of the skin. Physical examination revealed numerous annular papules with a firm hyperkeratotic rim on the medial sides of both feet and the palmar creases ranging from 2 mm to 1 cm in diameter. Palmoplantar refers to the skin on the soles of the feet and palms of the hands. Download scientific diagram transgradient palmoplantar keratoderma with ill defined margins, conical tapering of the fingers, flexion deformity, and digital.
Acquired and congenital ppk may be the only feature, ppk may accompany other diseases or be a part of a syndrome. Palmoplantar keratoderma ppk is a group of skin conditions characterized by thickening of the skin on the palms of the hands and soles of the feet. She also complains of burning when plantar areas are heavily callused. It can occur in the first few months of life and almost always presents by age 3. Punctate porokeratotic keratoderma jama dermatology. The thickening can present as a diffuse, focal or punctate pattern.
Histologic examination revealed a compact column of parakeratosis resembling that of a cornoid lamella of porokeratotic conditions. Ppk can be either acquired during the lifetime more commonly or inherited. Age and sex distribution acquired keratoderma may be observed in a wider age range of individuals. Punctatetype palmoplantar keratoderma is a type of palmoplantar keratoderma that is caused by genetic abnormalities that may be inherited in an autosomal dominant or autosomal recessive manner. In punctate palmoplantar keratoderma there are tiny skin spots on the palms and soles. Mim175860 horny papules over the palms, soles, and digits that develop central plugs. Three recent articles have described a previously unrecognized type of punctate palmoplantar keratoderma. Classification of keratodermas depends on whether or not it is inherited. Punctate palmoplantar keratoderma type i does not affect a persons lifespan. Additionally, the symptoms of punctate palmoplantar keratoderma type i and some of the treatments for the condition may have side effects that impact a persons quality of life.
The term spiny keratoderma is used for a dermatosis, presenting with multiple keratotic protrusions that resemble music box spines, and located on palms and soles. Affected individuals develop unusually thick skin on the palms of the hands and soles of the feet palmoplantar keratoderma beginning in childhood. None condition characterized by histologic punctate thickening of the stratum corneum, possibly as part of a generalized condition or a disorder primarily involving the hands and feet punctate keratoderma springerlink. Here we report a case 12yearold child presented with asymptomatic linear punctate plaque on the left sole and hand. Punctate palmoplantar keratoderma type i is a rare condition that affects the skin. Punctate porokeratotic keratoderma is characterized by many keratotic papules on the palms and soles. Spiny keratoderma also known as porokeratosis punctata palmaris et plantaris, punctate keratoderma, and punctate porokeratosis of the palms and soles is an autosomal dominant keratoderma of late onset that develops in patients aged 12 to 50, characterized by multiple tiny keratotic plugs, mimicking the spines on a music box. Acrokeratoelastoidosis focal acral hyperkeratosis keratosis punctata palmaris et plantaris porokeratosis punctate palmaris et plantaris. We report the case of a 70yearold male patient with punctate palmoplantar keratodermic lesions for more than. A nonepidermolytic keratoderma with sharp demarcation at the wrist and side of the thumb. Also, ppk may be a component of a generalized disorder of keratinisation such as lamellar ichthyosis. He noted the condition was characterized by the development of hyperkeratotic patches occurring primarily in pressure areas of menopausal women. Haxthausen disease, also known as keratoderma climactericum, was first described in 1934 by the danish dermatologist holger haxthausen, md. Palmoplantar keratoderma is also sometimes known as keratosis palmaris et plantaris.
Many people with this condition do find that they develop more skin lesions with age. The exact etiology of the disorder is not known but a dual influence of genetic and environmental factors may trigger the disease. Without the assistance of an expert, many people may miss diagnose the issue and try to self medicate. Punctate keratoderma this ladys daughter had exactly the same lesions. Palmoplantar keratoderma with deafness genetics home. Case of punctate palmoplantar keratoderma type i treated with. Keratoderma treatment questions about keratoderma on. A case of keratoderma sole complete cured by homoeopathy. Punctate type palmoplantar keratoderma is a type of palmoplantar keratoderma that is caused by genetic abnormalities that may be inherited in an autosomal dominant or autosomal recessive manner. Keratoderma is a skin condition that may be very uncomfortable. C epidermolytic keratoderma with prominent erythema at the border. Palmoplantar keratodermas ppks are a diverse entity of disorders that are characterized by abnormal thickening of the skin on the palms and soles. In diffuse palmoplantar keratoderma, the skin on the palms and or soles appears thickened and may be hard, yellowish in colour.
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