Presentation
Atrophodermia vermiculata presents with erythematous follicular papules on the cheeks in childhood and, with time, the lesions develop into pit-like depressions. Skin lesion Cicatricial alopecia Ulerythema List of cutaneous conditions [en.wikipedia.org]
Two patients, a father and a daughter, with atrophoderma vermiculatum are presented. Pedigree of a family with atrophoderma vermiculatum. [sayarujoyujy.cf]
Two patients, a father and a daughter, with atrophoderma vermiculatum are presented. [medigoo.com]
Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. [howlingpixel.com]
Entire Body System
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Asymptomatic
Erythema and pinhead follicular plugs developing mainly on the cheeks are soon followed by asymptomatic, sharply demarcated, pit-like or net-like impressions. The forehead, chin, helices and air lobes may also be affected. [dermis.net]
Erythema and pinhead follicular plugs developing mainly on the cheeks are soon followed by asymptomatic, sharply demarcated, pit-like or net-like impressions. Symptoms - Atrophodermia vermiculata Not supplied. [checkorphan.org]
Gastrointestinal
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Nausea
Infantile onset Intellectual disability, severe Vomiting Acidosis Palmar telangiectasia Sparse hair Malabsorption Metabolic acidosis Recurrent fractures Postural instability Abnormal bleeding Brain atrophy Stage 5 chronic kidney disease Fine hair Nephritis Nausea [mendelian.co]
Cardiovascular
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Cyanosis
Atrophoderma vermiculatum also is a key feature of Rombo syndrome, an autosomal dominant disorder characterized by AV, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas, and peripheral vasodilation with cyanosis. [medigoo.com]
Rombo syndrome is a very rare genetic disorder characterized mainly by atrophoderma vermiculatum of the face, : 580 multiple milia, telangiectases, acral erythema, peripheral vasodilation with cyanosis and a propensity to develop basal cell carcinomas [sayarujoyujy.cf]
[…] producing cribiform or honeycomb surface Worm-eaten(vermiculate) appearance Cause is undertermined Skin covering the ridges is even with normal skin 63 Keratosis Follicularis Spinulosa Decalvans(Siemens-1 Syndrome) Rombo syndrome Atrophodermia vermiculata, cyanosis [slideplayer.com]
Patients typically have a history of intrauterine growth retardation, low birth weight, feeble cry, weak suck and failure to thrive and present with inspiratory stridor, recurrent episodes of dyspnea or apnea, cyanosis and absent deep tendon reflexes. [mendelian.co]
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Heart Block
Atrophoderma vermiculatum usually is an isolated skin finding, but it also has been reported in association with dioxin-induced chloracne, steatocystoma multiplex, epidermal cysts, leukokeratosis oris, leukonychia, congenital heart block, atrial septal [medigoo.com]
block and atrial septal defect with Eisenmenger complex.8 Hsu et al. described a patient with unilateral AV, an ipsilateral congenital cataract, ipsilateral alopecia and a seizure disorder, and in view of the association with eye and central nervous [docksci.com]
Skin
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Skin Lesion
Skin lesion Cicatricial alopecia Ulerythema List of cutaneous conditions [en.wikipedia.org]
See also Skin lesion Cicatricial alopecia Ulerythema List of cutaneous conditions [wikidoc.org]
lesion Cicatricial alopecia Ulerythema List of cutaneous conditions References James, William; Berger, Timothy; Elston, Dirk (2005). [popflock.com]
Clinically, the diagnosis of any particular skin condition is made by gathering pertinent information regarding the presenting skin lesion(s), including the location (such as arms, head, legs), symptoms (pruritus, pain), duration (acute or chronic), arrangement [howlingpixel.com]
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Follicular Plugging
The degree of inflammation, the presence of milia, and the extent of follicular plugs are variable. As seen in our patient, AV tends to be symmetric, but unilateral involvement has been reported. [medigoo.com]
Atrophodermia vermiculata is characterized by erythema and follicular plugs on the cheeks, developing into painless reticular impressions. [ncbi.nlm.nih.gov]
Erythema and pinhead follicular plugs developing mainly on the cheeks are soon followed by asymptomatic, sharply demarcated, pit-like or net-like impressions. The forehead, chin, helices and air lobes may also be affected. [dermis.net]
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Acne Vulgaris
[…] and suicidal behavior in patients treated with isotretinoin for acne vulgaris. [stanfordchildrens.org]
RABBIOSI 2 Topical Spironolactone Inhibits Dihydrotestosterone Receptors In Human Sebaceous Glands - An Autoradiographic Study In Subjects With Acne-vulgaris 1988 E. BERARDESCA;P. GABBA;G. UCCI;G. BORRONI;G. [iris.unipv.it]
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Leukonychia
Atrophoderma vermiculatum usually is an isolated skin finding, but it also has been reported in association with dioxin-induced chloracne, steatocystoma multiplex, epidermal cysts, leukokeratosis oris, leukonychia, congenital heart block, atrial septal [medigoo.com]
Basaran et al. (1995) reported a family in which 3 members had congenital hypotrichosis, characterized by trichorrhexis nodosa and trichoptilosis (splitting of the hair), dry skin, keratosis pilaris, and leukonychia totalis. [findzebra.com]
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Skin Atrophy
Atrophoderma vermiculatum ( AV) is a rare skin disorder, typically presenting in childhood with a reticular pattern of skin atrophy on the cheeks, preauricular a. Atrophoderma Vermiculata, also known as atrophoderma vermiculatum,. [sayarujoyujy.cf]
A rare autosomal recessive symmetric dermatopathy (genodermatosis) (OMIM:209700) characterised by skin atrophy with sharply demarcated “pits”, variably accompanied by cardiac defects, mental retardation and neurofibromas. [medical-dictionary.thefreedictionary.com]
Workup
Subsequent workup, with particular attention to his history and presentation, led to his diagnosis. [stanfordchildrens.org]
Biopsy
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Hepatocellular Carcinoma
carcinoma Ulcerative colitis Prolonged prothrombin time Vitamin D deficiency Vitamin E deficiency Cholangiocarcinoma Hyperammonemia Systemic lupus erythematosus Proximal muscle weakness in lower limbs Decreased number of peripheral myelinated nerve fibers [mendelian.co]
Treatment
A) Patient before treatment: grouped, pitlike atrophic scars over the cheek, B) Improved cosmetic appearance of the skin after systemic treatment with isotretinoin. [ncbi.nlm.nih.gov]
Treatment: Management of atrophoderma vermiculatum is challenging. Atrophoderma vermiculatum is mainly a cosmetic problem. [medigoo.com]
Unfortunately, there was no improvement and the treatment was discontinued. At the age of 12 years, the patient underwent a rectus transposition procedure to correct his strabismus. [docksci.com]
Prognosis
Prognosis - Atrophodermia vermiculata Not supplied. Treatment - Atrophodermia vermiculata Not supplied. Resources - Atrophodermia vermiculata [checkorphan.org]
Atrophoderma vermiculata information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. [sayarujoyujy.cf]
PMID: 9218894 Prognosis Marqueling AL, Gilliam AE, Prendiville J, Zvulunov A, Antaya RJ, Sugarman J, Pang ML, Lee P, Eichenfield L, Metz B, Goldberg GN, Phillips RJ, Frieden IJ Arch Dermatol 2006 Dec;142(12):1611-6. doi: 10.1001/archderm.142.12.1611. [ncbi.nlm.nih.gov]
The prognosis is good, with many instances of spontaneous regression. Primarily a cosmetic problem, therapy for this condition is aimed at reassurance, genetic counseling, and dermabrasion where appropriate. [medigoo.com]
(Outcomes/Resolutions) The prognosis of Atrophoderma Vermiculata/Folliculitis Ulerythematosa Reticulata is excellent in a majority of cases, since it is a benign/harmless condition. [dovemed.com]
Etiology
Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. [howlingpixel.com]
Our ability to treat acne and rosacea ef fectively has outpaced the vastly expanded understanding of their etiologies. Acne today is not only an eminently treatable disease; in some cases, for exam is actually curable. [books.google.ro]
Etiology J Eur Acad Dermatol Venereol 2003 Jan;17(1):70-2. PMID: 12602976 Diagnosis Schaller J, Rytina E, Rütten A, Hendricks C, Ha T, Requena L J Cutan Pathol 2010 Sep;37(9):1002-9. Epub 2010 Feb 18 doi: 10.1111/j.1600-0560.2010.01527.x. [ncbi.nlm.nih.gov]
Epidemiology
Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology [prolekare.cz]
Epidemiology Frequency United States The exact incidence of idiopathic atrophoderma of Pasini and Pierini is not known. The small number of cases reported may reflect its asymptomatic nature rather than its true incidence. [emedicine.medscape.com]
Epidemiological and clinicopathological study of oral leukoplakia. Indian J Dermatol Venereol Leprol 2005;71:161-5. [ PUBMED ] 102. Liu W, Wang YF, Zhou HW, Shi P, Zhou ZT, Tang GY. [e-ijd.org]
Pathophysiology
Pathophysiology The cause of atrophoderma of Pasini and Pierini is not known. The pathophysiologic events that cause the discrete lesions seen clinically, as well as the timing of their appearance, are also unknown. [emedicine.medscape.com]
Etiology, Pathophysiology, and Nosology of Acne.- Prevalence.- A Precis of Pathogenesis.- Anatomy of Follicles.- Epidermal Lipids.- Sebaceous Glands.- Anatomy of Sebaceous Glands.- Physiology of Sebaceous Glands.- Micro-organisms.- Cocci.- Yeasts.- [hoepli.it]
Palm-plantar hyperkeratosis, photophobia, corneal abnormalities and atopies can be associated. 1 Men are more seriously affected by the disease. 1 The pathophysiology of the destruction of the pilar follicle is not well known yet. [scielo.br]
The pathophysiology of disease in patients with paroxysmal nocturnal hemoglobinuria. Hematology Am Soc Hematol Educ Program 2008; 2008:104-10. 31. Arepally GM, Ortel TL. Heparin-induced thrombocytopenia. N Engl J Med 2006;355;809-17. 32. [e-ijd.org]
Prevention
Topical comedolytic agents such as retinoids prescribed at the incipient stage might then prevent the evolution of the full-fledged disorder. This therapeutic maneuver could prevent the dreaded sequel of scarring. [books.google.ro]
Prevention - Atrophodermia vermiculata Not supplied. Diagnosis - Atrophodermia vermiculata Not supplied. Prognosis - Atrophodermia vermiculata Not supplied. Treatment - Atrophodermia vermiculata Not supplied. Resources - Atrophodermia vermiculata [checkorphan.org]
Atrophoderma vermiculata information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. [sayarujoyujy.cf]
Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [icdlist.com]