Pdf p peeling skin syndrome is a rare autosomal recessive disorder of cornification that starts either at birth or later in childhood. These files will have pdf in brackets along with the filesize of the download. Peeling skin syndrome inclusion criteria 36543 generalised peeling skin with or without atopy, or acral peeling skin tgm5 mutations excluded, or peeling skin in association with other features, or and diagnosis of peeling skin syndrome confirmed by consultant dermatogist peeling skin syndrome exclusion criteria 36543 peeling skin cases that have not had the genetic. Skin peeling typically follows healing of a first degree burn or sunburn. Peeling skin syndrome pss is a group of rare inherited skin disorders in which the normal gradual process of invisible shedding of the outermost skin layers is hastened andor aggravated. We report a case of idiopathic peeling skin syndrome with. It may also be a sign of an immune system disorder or other disease. The first case of peeling skin syndrome was reported by fox in 1921 and termed keratolysis exfoliativa congenita.
Several of my fingertips have severe dry, cracked, peeling skin. Toxic shock syndrome, a potentially fatal immune system reaction to a bacterial infection such as staphylococcus aureus, can cause severe desquamation. Often associated with infants who are post dates, many term babies exhibit this kind of peeling as well. Individuals with acral peeling skin syndrome should avoid these condition, when possible. Webmd symptom checker helps you find the most common medical conditions indicated by the symptoms numbness or tingling and skin peeling, cracking or scaling including peripheral neuropathy, carpal tunnel syndrome, and. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for peeling skin syndrome. Biophysical profile, which includes skin hydration, transepidermal water loss, sebum content, skin ph, of this patient was assessed from 7 different sites using the corneometer, tewameter, sebumeter, skin phmeter. Acral peeling skin syndrome is a skin disorder characterized by painless peeling of the top layer of skin. Sunburn is one of the most common acute causes of peeling skin caused by ultraviolet light. Peeling skin syndrome pss is a rare genodermatoses characterized by asymptomatic, localized or generalized, continuous exfoliation of the stratum corneum. Peeling skin may occur because of direct damage to the skin, such as from sunburn or infection. Peeling skin syndrome is a very rare autosomal recessive disease. Most forms of pss manifest at birth or during infancy with shedding or peeling of the outermost layer of the skin horny layer, aka stratum corneum. For example, once the rash of measles fades, there is desquamation.
Acral peeling skin syndrome jama dermatology jama network. Pss4 presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of nonerythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. The acral peeling skin syndrome apss is a rare autosomal recessive disorder clinically characterized by asymptomatic desquamation of the skin limited to the hands and feet and histologically by cleavage at the stratum granulosum and stratum corneum level kiritsi et al j invest dermatol 2010. The acral peeling skin syndrome apss congenital or familial acral peeling is extremely rare. While it typically manifests from early childhood, in this first reported case from south africa, the patient did not manifest clinically until the fourth decade of life. Peeling skin conditions primary care dermatology society. Peeling skin syndrome belongs to the groups of congenital ichthyosis and skin fragility disorders with autosomal recessive inheritance.
Like other staphylococcal infections, staphylococcal scalded skin syndrome is contagious. Skin peeling syndrome sps is a rare genodermatosis, with variable age of onset from birth 1 to adulthood. Pss has been associated with a heterogeneous group of mutations in various genes. The latter is subclassified into noninflammatory or type a pss and inflammatory or type b pss. The term acral refers to the fact that the skin peeling in this condition is most apparent on the hands and feet. We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. It doesnt hurt, but your skin often itches and can get red, dry, thick, and blistered. Light dressings should be applied to prevent infection. Clinical and histopathological picture corresponded to the noninflammatory variant of peeling skin syndrome type a. Ehlersdanlos syndrome is a group of connective tissue disorders that are characterized by unstable, hypermobile joints, loose, stretchy skin, and tissue fragility.
Several patients have been reported with high ige levels. Acral peeling skin syndrome is characterized by painless peeling of the top layer of skin the epidermis. Peeling skin syndrome is a rare genetic disorder, which causes continuous peeling of skin. Since i gathered some information from the internet i found out that this condition is called acral peeling skin syndrome apss because the only affected areas are skin on palms and soles, and that is caused by some gene mutation and that is a very rare disease. Some conditions that cause peeling of the skin on hands and feet in children include.
Acral peeling skin syndrome genetic and rare diseases. Acral peeling skin syndrome resembling epidermolysis bullosa. Peeling skin syndrome genetic and rare diseases information. I used to live in phoenix, az and currently live in chicago, il. Pss4 presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of nonerythematous skin on the palms and soles, which. A proper diagnosis of the condition that caused peeling skin is established by taking into consideration all kinds of factors, such as. Peeling skin conditions primary care dermatology society uk. Peeling skin syndrome pss is a rare recessively inherited ichthyosiform genodermatoses. Acral peeling skin syndrome is a rare condition, with several dozen cases reported in the medical literature. The fragile tissues, stretchy skin, and unstable joints found in ehlersdanlos syndrome are due to a gene mutation in collagen. Peeling of the skin is loss of the epidermis, the upper layer of the skin. In this patient, the skin and nails look at the thumbnail are stained yellow from the presence of meconium in utero. Acral refers to the fact that the peeling is most apparent on the hands and feet, although peeling may also occur on the arms and legs. Peeling skin can affect different parts of the body and can be a symptom of various medical conditions.
Skin peeling syndrome in a kurdish family jama dermatology. Racgp topical corticosteroid addiction and withdrawal. Acral peeling skin syndrome genetic and rare diseases nih. This peeling was more severe on the soles than palms and on. Filaggrin 2 deficiency results in abnormal cellcell. Acral peeling skin syndrome apss is an autosomal recessive skin disorder characterized by acral blistering and peeling of the outermost layers of the epidermis. The two major forms of pss are acral pss apss and generalized pss 36. Peeling skin syndrome localized to the acral surfaces represents a new variant. It is an ichthyosiform erythroderma characterized by lifelong patchy peeling of the entire skin with onset at birth or shortly after. Acral peeling skin syndrome resembling epidermolysis. The term acral refers to the fact that the peeling is most apparent on the hands and feet. The signs and symptoms of peeling skin syndrome usually appear soon after birth, but they may also develop later in life.
Aug 07, 20 the acral peeling skin syndrome apss is a rare autosomal recessive disorder clinically characterized by asymptomatic desquamation of the skin limited to the hands and feet and histologically by cleavage at the stratum granulosum and stratum corneum level kiritsi et al j invest dermatol 2010. Normally, the lifespan of skin cells is about two weeks, and after that, they fall off individually during the desquamation process and are replaced by the new cells, so the whole process is unnoticeable. Peeling of the skin desquamation is a natural process which represents the final phase of skin cell life cycle. A genodermatosis characterized by congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of siemens and annular epidermolytic ichthyosis. Peeling skin syndrome in eight cases of four different families from. Symptoms typically develop soon after birth, but may also begin in childhood or early adulthood. Peeling of the skin might be accompanied by rash, itching, irritation and dryness of the skin.
Pss is characterized by painless, continual, spontaneous skin peeling exfoliation due to a separation of the outermost layer of the epidermis stratum corneum from the underlying layers. Sep 01, 20 generalized peeling skin syndrome pss is a form of pss see this term presenting with a generalized distribution. Only several dozen cases have been reported in the literature, making it rare, but because its symptoms are mild and similar to other disorders it could very well be underdiagnosed. Acral peeling skin syndrome genetics home reference nih. Peeling skin syndrome nord national organization for rare. Histopathological study confirmed the clinical diagnosis of peeling skin syndrome. Peeling skin syndrome, acral type definition of peeling. Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. If you do not have it you can download adobe reader free of charge. Alerts and notices synopsis acral peeling skin syndrome pss. Rash, itching, dryness and other irritating skin problems may accompany peeling skin. Occasionally, peeling also occurs on the arms and legs. Generalized peeling skin syndrome genetic and rare diseases.
Inflammatory pss type b is associated with generalized erythema, pruritus and atopy. Pdf novel tgm5 mutations in acral peeling skin syndrome. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for acral peeling skin. Peeling skin syndrome its like having lifelong sunburn, where you can pull up a sheet of the top layer of skin. Peeling of the skin is an uncommonly encountered disorder.
However, because its signs and symptoms tend to be mild and similar to those of other skin disorders, the condition is likely underdiagnosed. In an acral form of the disorder pss2, the dorsa of the hands and feet are predominantly affected, and ultrastructural analysis shows separation at the junction between the granular cells and the stratum corneum in the outer epidermis summary by cassidy et al. Find more information on this unusual disorder in this article peeling skin syndrome pss is a rare genetic disorder characterized by continuous peeling of the stratum corneum, which is the outermost layer of the skin. Staphylococcal scalded skin syndrome occurs almost exclusively in infants and children under the age of 6. Peeling skin syndrome pss is a group of rare inherited skin disorders in which the. Webmd symptom checker helps you find the most common medical conditions indicated by the symptoms numbness or tingling and skin peeling, cracking or scaling including peripheral neuropathy, carpal tunnel syndrome, and generalized anxiety disorder.
A histological examination revealed a split above the granular layer without inflammatory response. I have the same problem that lasts for approximately same period of time several months and there are identical symptoms and problems. Pdf peeling skin syndrome pss is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. Review the causal information about the various more general categories of medical conditions. The acral type of peeling skin syndrome is caused by a mutation in the transglutaminase5 gene, or tgm5. Ive read about a dermatological problem posted by a person from philippines about peeling skin from fingertips leaving wrinkles and cracks on surface. Causes and types causes of broader categories of peeling skin syndrome, acral type.
Since then, this rare disorder has been designated peeling skin syndrome, deciduous skin, or familial continual skin peeling syndrome. Peeling skin syndrome pss refers to a group of rare autosomal recessive forms of ichthyosis. Filaggrin 2 deficiency results in abnormal cellcell adhesion. Derangement was observed in all the above parameters. The two major forms of pss are acral pss apss and generalized pss. Biophysical profile of a case of peeling skin syndrome a. To open a pdf file you will need compatible software such as adobe reader. The peeling skin syndrome stanley b levy, m d, and lowell a goldsmith, m d durham, nc a untque form of congemtal lchthyosls m two unrelated pattents 1s described and characterized hlstologtcally by separation of the epdermls between the stratum corneum and the stratum granulosum the chntcal history, genettcs, sermlly performed skin bmps. Occurrence of vesicles and bullae in peeling skin syndrome is very rare. The condition may be made worse by hot temperatures, high humidity, and friction. Biophysical profile, which includes skin hydration, transepidermal water loss, sebum content, skin ph, of this patient was assessed from 7 different sites using the corneometer, tewameter, sebumeter, skinphmeter. Clinically, it is characterized by a pruritic or nonpruritic spontaneous superficial peeling of the skin, which sometimes is accompanied by erythema or vesiculation. The true prevalence of the disease has not been worked out.
Pss has been associated with a heterogeneous group of mutations in various genes involved in corneocyte. Peeling skin syndrome pss refers to a complex group of autosomal recessive disorders of cornification featuring superficial detachment of the epidermal cornified cell layers with no mucosal fragility kose et al. Generalized peeling skin syndrome genetic and rare. Pdf peeling skin syndrome in eight cases of four different families. Staphylococcal scalded skin syndrome skin disorders msd. The hands and feet are especially common places to observe peeling. Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. Peeling skin syndrome nord national organization for. Staphylococcal scalded skin syndrome skin disorders. Stevensjohnson syndrome and toxic epidermal necrolysis are two forms of the same lifethreatening skin disease that cause rash, skin peeling, and sores on the mucous membranes.
It is an autosomal recessive disorder in which, unlike the generalized form, skin peeling is limited to the hands and feet 1,2. Generalized peeling skin syndrome pss is a form of pss see this term presenting with a generalized distribution. Occasionally, peeling may also occur on the arms and legs. Peeling skin is unintended damage to and loss of the upper layer of your skin epidermis. It appears after a few hours of direct sun exposure or from sunlight reflected off snow, sand and water.
This has been a constant condition for over a year now, at times flaring up and becoming worse than other times. Pdf most of the documents on the racgp website are in portable document format pdf. Find more information on this unusual disorder in this article peeling skin syndrome pss is a rare genetic disorder characterized by continuous peeling of the stratum corneum, which is the outermost layer of. Electron microscopy analysis of skin biopsies, reveals mostly normalappearing upper layers. Adultonset acral peeling skin syndrome in a nonidentical. There is currently no cure for apss, therefore treatment is centred on preventing skin damage and helping to reduce symptoms.
Affected individuals may also experience itching and reddening of the skin. Since the peeling skin syndrome is quite typical in nature, the symptoms of peeling skin syndrome vary from person to person. This means that a person with apss has inherited a defective copy of the gene from both parents. Peeling skin syndrome pss is a heterogeneous group of rare autosomal recessive disorders characterized by superficial painless peeling and blistering of the skin without mucosal fragility.
Peeling skin syndrome is a rare genodermatosis with variable age of onset from birth to adulthood. The signs and symptoms of peeling skin usually appear soon after birth, but they may also develop later in life. Acral peeling skin syndrome in two eastafrican siblings. This may occur due to direct damage to the skin, any immune system disorder or other diseases. Two major clinical types of pss have been reported. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum. Acral peeling skin syndrome is a clinical variant of the rare, genetic, and recurrent exfoliative dermatosis known as peeling skin syndrome. Acral peeling skin syndrome is a rare autosomal recessive disorder in which skin exfoliation is limited to the hands and feet. See also introduction to hypersensitivity reactions and inflammatory skin disorders. A rare peeling skin syndrome characterized by superficial peeling of the skin predominantly affecting the dorsa of the. The acral peeling skin syndrome apss is a rare autosomal recessive disorder clinically characterized by asymptomatic desquamation of the skin limited to the hands and feet and histologically by cleavage at stratum granulosum and stratum corneum level.
Treatment treating peeling skin syndrome by applying skin softening. If blister develop, they may be lanced with a sterile needle. Radiation exposure to the skin, also known as cutaneous radiation syndrome crs, causes damaged to the outer and even inner layers of the skin. What is peeling skin syndromecausessymptomstreatment. As i mentioned, this condition has existed for more than a year, summer, fall, winter, all climates. Peeling skin syndrome is a genetic disorder characterized by continual peeling of the skin. The overwhelming majority of reported cases started manifesting clinically from early childhood and occurred in singleton patients. Here you can read posts from all over the web from people who wrote about peeling and raynauds phenomenon, and check the relations between peeling and raynauds phenomenon. Webmd symptom checker helps you find the most common symptom combinations and medical conditions related to skin peeling, cracking or scaling. Mar 19, 2012 acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. Only a few reports of peeling skin syndrome have been published and they all describe widespread peeling.
Symptoms of peeling skin syndrome, acral type including 8 medical symptoms and signs of peeling skin syndrome, acral type, alternative diagnoses, misdiagnosis, and correct diagnosis for peeling skin syndrome, acral type signs or peeling skin syndrome, acral type symptoms. Pdf on mar 1, 2004, mukhopadhyay amiya kumar and others published peeling skin syndrome. Peeling skin information including symptoms, diagnosis, treatment, causes, videos, forums, and local community support. Peeling skin syndrome pss is an autosomal recessive genodermatosis characterized by the shedding of the outer epidermis. Numbness or tingling and skin peeling, cracking or scaling. Peeling skin syndrome serves as a model for studying the stratum corneum. It rarely occurs in older people except for those with kidney failure or a weakened immune system.
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