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Atrophoderma vermiculatum(AVA)

MedGen UID:
82666
Concept ID:
C0263429
Disease or Syndrome
Synonyms: Atrophodermia reticulata; ATROPHODERMIA RETICULATA SYMMETRICA FACIEI; Atrophodermia vermiculata; AVA; Folliculitis ulerythematosa; Folliculitis ulerythematosa reticulata; Honeycomb atrophy; Keratosis pilaris affecting the follicles of the eyebrow hairs; Type of genodermatosis; Ulerythema ophryogenesis
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Autosomal recessive inheritance refers to genetic conditions that occur only when mutations are present in both copies of a given gene (i.e., the person is homozygous for a mutation, or carries two different mutations of the same gene, a state referred to as compound heterozygosity).
Inheritance with unknown mechanism
MedGen UID:
831588
Concept ID:
CN227389
Intellectual Product
Describes an inherited disorder with unknown mode of inheritance.
SNOMED CT: Honeycomb atrophy of face (2736005); Folliculitis ulerythematosa reticulata (2736005); Atrophoderma vermiculatum (2736005); Acne vermoulante (2736005)
 
OMIM®: 209700
Orphanet: ORPHA79100

Definition

Atrophoderma vermiculata, a form of keratosis pilaris atrophicans, typically presents in childhood with erythema and follicular keratotic papules that slowly progress to characteristic atrophy, which has been described as worm-eaten, reticular, or honeycomb, and occurs on the cheeks, preauricular area, and forehead. More rarely, the atrophy may extend to the upper lip, helices, ear lobes, and, in some cases, the limbs. The degree of inflammation, the presence of milia, and the extent of follicular plugs are variable (summary by Luria and Conologue, 2009). [from OMIM]

Clinical features

Atrophodermia vermiculata
MedGen UID:
506547
Concept ID:
CN117726
Finding
Symmetrical vermiform facial atrophy that affects mainly the forehead, the chin, the ear lobes and helices. Atrophodermia vermiculata is characterized by erythema and follicular plugs on the cheeks, developing into painless reticular impressions.
Milia
MedGen UID:
504701
Concept ID:
CN000991
Finding
Presence of multiple small cysts containing keratin (skin protein) and presenting as tiny pearly-white bumps just under the surface of the skin.
Leukonychia
MedGen UID:
504963
Concept ID:
CN001649
Finding
White discoloration of the nails.
Atrophodermia vermiculata
MedGen UID:
506547
Concept ID:
CN117726
Finding
Symmetrical vermiform facial atrophy that affects mainly the forehead, the chin, the ear lobes and helices. Atrophodermia vermiculata is characterized by erythema and follicular plugs on the cheeks, developing into painless reticular impressions.
Abnormality of the cardiovascular system
MedGen UID:
427888
Concept ID:
CN001481
Finding
Any abnormality of the cardiovascular system.
Atrophodermia vermiculata
MedGen UID:
506547
Concept ID:
CN117726
Finding
Symmetrical vermiform facial atrophy that affects mainly the forehead, the chin, the ear lobes and helices. Atrophodermia vermiculata is characterized by erythema and follicular plugs on the cheeks, developing into painless reticular impressions.

Recent clinical studies

Etiology

Olaiwan A, Chandesris MO, Fraitag S, Lortholary O, Hermine O, Fischer A, de Prost Y, Picard C, Bodemer C
J Am Acad Dermatol 2011 Dec;65(6):1167-72. Epub 2011 Jun 24 doi: 10.1016/j.jaad.2010.09.714. [Epub ahead of print] PMID: 21703716

Diagnosis

Olaiwan A, Chandesris MO, Fraitag S, Lortholary O, Hermine O, Fischer A, de Prost Y, Picard C, Bodemer C
J Am Acad Dermatol 2011 Dec;65(6):1167-72. Epub 2011 Jun 24 doi: 10.1016/j.jaad.2010.09.714. [Epub ahead of print] PMID: 21703716
Luria RB, Conologue T
Cutis 2009 Feb;83(2):83-6. PMID: 19326693
van Steensel MA, Jaspers NG, Steijlen PM
Br J Dermatol 2001 Jun;144(6):1215-8. PMID: 11422044
Hsu S, Nikko A
J Am Acad Dermatol 2000 Aug;43(2 Pt 1):310-2. doi: 10.1067/mjd.2000.106514. PMID: 10906657
Arrieta E, Milgram-Sternberg Y
Arch Dermatol 1988 Jul;124(7):1101, 1104. PMID: 3389853

Therapy

Weightman W
Clin Exp Dermatol 1998 Mar;23(2):89-91. PMID: 9692315

Prognosis

Weightman W
Clin Exp Dermatol 1998 Mar;23(2):89-91. PMID: 9692315
Frosch PJ, Brumage MR, Schuster-Pavlovic C, Bersch A
J Am Acad Dermatol 1988 Mar;18(3):538-42. PMID: 3280621

Clinical prediction guides

Olaiwan A, Chandesris MO, Fraitag S, Lortholary O, Hermine O, Fischer A, de Prost Y, Picard C, Bodemer C
J Am Acad Dermatol 2011 Dec;65(6):1167-72. Epub 2011 Jun 24 doi: 10.1016/j.jaad.2010.09.714. [Epub ahead of print] PMID: 21703716

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