Atrophoderma of pasini and pierini clinical presentation. Dermnet nz does not provide an online consultation service. Atrophoderma vermiculatum av is a rare skin disorder, typically presenting in childhood with a reticular pattern of skin atrophy on the cheeks, preauricular area, and forehead that seems to result from inflammation around follicular plugs. The disorder usually begins during adolescence or early adulthood with a slightly erythematous lesion appearing on the trunk, commonly on the back or lumbosacral region, followed in frequency by the chest, arms, and abdomen. What is atrophoderma of pasini and pierini atrophoderma of pasini and pierini is a rare dermal atrophy disorder characterised by wellcircumscribed slightly depressed areas, usually on the patients trunk. Atrophoderma vermiculata is a rare genodermatosis with usual onset in childhood, characterized by a honeycombed reticular atrophy of the cheeks. Hello i have atrophoderma vermiculata and also milia, this condition has been slowly worsening over the years. It is characterized by symmetric reticular or honeycomb atrophy of the cheeks that may extend to. Atrophoderma of pasini and pierini primary care dermatology.
Laser treatment of atrophoderma vermiculata journal of the. The underlying pathogenesis appears to be abnormal follicular hyperkeratinization. Multiple, sharply defined, depressed lesions that look. Atrophoderma vermiculatum typically presents in childhood with erythema and follicular keratotic papules that slowly progress to the characteristic reticular atrophy, which has been described as wormeaten, reticular, or honeycomb, and occurs on the cheeks, preauricular area, and forehead. A case of atrophoderma vermiculatum responding to systemic. Atrophodermia vermiculata altmeyers enzyklopadie fachbereich. Atrophoderma vermiculatum was originally described by in 1896. He had no associated other diseases, such as congenital heart block, other cardiac anomalies, neurofibromatosis, oligophrenia, or down syndrome. Sep 21, 2011 linear atrophoderma is a rare disease, first described by moulin in 1992 in 5 patients.
The other two subtypes of kpa are keratosis follicularis spinulosa decalvans and keratosis pilaris atrophicans faciei. Keratosis pilaris and other inflammatory follicular. Atrophoderma vermiculatum, a disorder limited to the face usually has its onset during childhood or puberty and has a slow progressive course. It is an acquired unilateral hyperpigmented, depressed bandlike areas following the lines of blaschko. A case of atrophoderma vermiculatum showing a good response. The morphologic hallmark is a wormeaten or honeycombed reticular atrophy of the skin typically localized at the cheeks, preauricular regions, and temples. It is characterized by symmetric reticular or honeycomb atrophy of the cheeks that may extend to the ears and forehead. Skin lesion cicatricial alopecia ulerythema list of cutaneous conditions en.
Follicular atrophoderma is a skin condition consisting of follicular indentations without hairs, notably occurring on extensor surfaces of the hands. Atrophoderma vermiculatafolliculitis ulerythematosa reticulata. The typical presentation is of single or multiple sharply demarcated, mildly depressed, hyperpigmented lesions on the trunk. May 03, 2018 since the original description, much debate has occurred regarding whether idiopathic atrophoderma of pasini and pierini is a distinct entity or an atrophic, nonindurated variant of morphea, despite differences in the origin, development, and outcome of the lesions. The border between normal skin and the area of atrophy has a welldefined, cliffdrop edge. Follicular atrophoderma and basal cell epitheliomata. A 25yearold male had multiple 12 mm sized pitted scars with ridges producing a reticulated honeycomb appearance on the left cheek since childhood. The classic clinical manifestations of app are hyperpigmented or hypopigmented, depressed areas of skin on the trunk or extremities. A case of atrophoderma vermiculatum showing a good response to topical tretinoin. A variety of names have been given to this condition, including atrophoderma vermicularis, folliculitis ulerythematosa, atrophodermia reticulata, and honeycomb atrophy.
The chief characteristics of this rare, benign condition that usually begins in childhood are a symmetrie wormeaten or reticular atrophy of the cheeks that may extend to the ears or forehead. Atrophoderma of pasini and pierini is a rare dermal atrophy disorder characterised by wellcircumscribed slightly depressed areas, usually on the patients trunk. Signs and symptoms of atrophoderma of pierini and pasini include multiple oval, darkened hyperpigmented plaques in which tissue under the skin breaks down so that there is a depression dent within the skin. Atrophoderma vermiculatum av, also known as folliculitis ulerythematosa reticulata, is a rare variant of keratosis pilaris.
It is believed to be a distinct entity from morphoea. Keratosis pilaris in a characteristic distribution on upper outer arm in a patient with ichthyosis. Anetoderma and other atrophic disorders of the skin. Idiopathic atrophoderma of pasini and pierini iapp is a form of dermal atrophy that manifests as single or multiple sharply demarcated, hyperpigmented, nonindurated patches.
Atrophoderma vermiculatam primary care dermatology society uk. Atrophoderma of pasini and pierini differential diagnoses. Pedigree of a family with atrophoderma vermiculatum. Atrophodermia vermiculata presents with erythematous follicular papules on the cheeks in childhood and, with time, the lesions develop into pitlike depressions. Keratosis follicularis spinulosa decalvans in a female. Follicular atrophoderma and basal cell epitheliomata bioinformatics tool laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on cocitations. I am 24 and had it diagnosed by a top australian skin specialist dr maureen rogers when i was a child. Atrophoderma vermiculata is a rare genodermatosis that presents as an inflammatory follicular atrophy. Jan 21, 2010 atrophoderma of pierini and pasini is thought to possibly represent a late stage of morphea a type of localized scleroderma. Atrophoderma vermiculatum is an autosomal dominant disorder with small pitted scars over cheek and forehead. These patches are marked by a slight depression of the skin with an abrupt edge, usually on the backs of adolescents or young adults. Atrophoderma vermiculatum av, also known as folliculitis ulerythematosa reticulata, is a rare follicular disorder and is one of the variants of keratosis pilaris atrophicans kpa. In this case of a child with atrophoderma vermiculatum isotretinoin stopped atrophy progression and improved cosmetic appearance of the skin.
Atrophoderma vermiculatum definition of atrophoderma. Atrophodermalike guttate morphea mdedge dermatology. Extensive unilateral atrophoderma vermiculatum associated. The face was symmetrically involved by numerous closely crowded small areas of atrophy separated by narrow ridges, producing a cribriform or honeycomb surface. Atrophoderma vermiculata is a skin disorder that develops when hair follicles get plugged by dead skin. Mar 17, 2018 atrophoderma vermiculata or folliculitis ulerythematosa reticulata is a rare subtype of keratosis pilaris or kp, a common skin condition. The leading primary care society for dermatology and skin surgery. If you have any concerns with your skin or its treatment, see a dermatologist for advice. It affects children or adolescents of both genders involving.
Atrophoderma vermiculatum medigoo health medical tests. Dermatopathology reference describes atrophoderma of pasini and pierini histopathology including histologic features and provides links to additional medical references. Laser treatment of atrophoderma vermiculata sciencedirect. Atrophoderma definition of atrophoderma by medical dictionary.
Go to previous content download this content share this content add article to favorites go to next content. May 03, 2018 idiopathic atrophoderma of pasini and pierini is typically a benign, asymptomatic condition. May 02, 2018 atrophoderma vermiculata is a skin disorder that develops when hair follicles get plugged by dead skin. Papules and cysts around the elbow, pitted scars over the healed lesions, atrophoderma vermiculatum and hypo pigmented streaks over the scalp can be associated with apl 7. Atrophodermalike guttate morphea is a potentially underreported or undescribed entity consisting of a combination of clinicopathologic features widespread hypopigmented macules on the trunk and extremities marked by thinned collagen, fibroplasia, and altered fragmented elastin in the papillary dermis and upper reticular dermis are the key features. For more information on how to use laverne, please read the how to guide. When i was a teenager i had laser treatmentdermabrasion on both my cheeks and it had no effect, it came back.
Dermis atrophodermia vermiculata information on the diagnosis. Apr 19, 2011 keratosis follicularis spinulosa decalvans, a relatively rare disorder, was first described by macleod, however, it was siemens who first used this descriptive term in 1926. The lesions were unilateral and there was no other family member with similar lesions. Atrophoderma of pierini and pasini genetic and rare. Monarchs tools are designed to make it easier to compare the signs and symptoms phenotypes of different diseases and discover common features. The border between normal skin and the area of atrophy has a welldefined, cliffdrop edge atrophoderma of pasini and pierini. Patients with this follicular dermatosis typically present in childhood with a reticular pattern of atrophic pits on the cheeks, preauricular region, and forehead. Atrophodermia vermiculata atrophoderma vermiculatum. Atrophying definition of atrophying by the free dictionary. Atrophoderma vermiculatum is a rare, benign follicular disorder that primarily affects children. The forehead, chin, helices and air lobes may also be affected. History a 9yearold boy presented with honeycomb like atrophy on both cheeks. Atrophoderma vermiculatum av is a rare follicular disorder primarily affecting children with reticular or honeycomb atrophy of the cheeks and. Atrophoderma vermiculatum av, also known as folliculitis ulerythematosa reticulata, is a rare variant of keratosis pilaris atrophicans.
Atrophoderma of pasini and pierini app is a rare skin disorder affecting dermal collagen and presenting with dermal atrophy. Atrophodermia vermiculata presents with erythematous follicular papules on the cheeks in. Atrophoderma vermiculata genetic and rare diseases. Erythema and pinhead follicular plugs developing mainly on the cheeks are soon followed by asymptomatic, sharply demarcated, pitlike or netlike impressions. Acne vermoulante, acne vermoulanti, atrophoderma reticulata symmetrica faciei, atrophoderma reticulatum, atrophoderma vermiculata, atrophoderma vermiculatum, atrophodermia reticulata symmetrica faciei, atrophodermia ulerythematosa, atrophodermie vermiculee des joues avec keratoses folliculaires, folliculitis ulerythema reticulata, folliculitis. Atrophodermia vermiculata is a rare inflammatory follicular atrophic condition which must be considered in the differential diagnosis of acne scarring. Mar 01, 2020 the monarch initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Laser treatment of atrophoderma vermiculata request pdf. Atrophoderma vermiculatum bioinformatics tool laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on cocitations.
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