You are in: eMedicine Specialties > Dermatology > PEDIATRIC DISEASES Atrophia Maculosa Varioliformis CutisArticle Last Updated: Feb 15, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Julianne H Kuflik, MD, Assistant Clinical Professor of Dermatology, Department of Dermatology, UMDNJ-New Jersey Medical School Julianne H Kuflik is a member of the following medical societies: American Academy of Dermatology Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School Editors: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: AMVC, spontaneously formed facial scars, spontaneous scarring, idiopathic scars, idiopathic scarring, elastic tissue defect, cheek scarring, cheek scars INTRODUCTIONSection 2 of 10
  BackgroundAtrophia maculosa varioliformis cutis (AMVC) is a rare disease that presents as spontaneously formed facial scars in young adults. In 1918, Heidingsfeld coined the disease name to describe the numerous spontaneously formed scars on the cheeks of a 20-year-old man. Most commonly located on the cheeks, the scars vary in shape and size, resembling those from smallpox. A slight erythema or pruritus precedes the appearance of the scars by 1-2 days. The number of reports on this eruption in literature is limited, and even fewer are documented with skin biopsy specimens. Its etiology remains unknown, but elastic tissue pathology has been reported in histology findings. Based on a pedigree assembled by Qu et al and reported in 2005, they suggested that AMVC is of autosomal dominant inheritance. PathophysiologyAlthough its etiology is unknown, AMVC may represent an underlying defect of dermal elastin as demonstrated by histologic and ultrastructural findings. AMVC has been documented only in the skin. Mortality/MorbidityIn addition to being of cosmetic concern to a patient, the sudden unprecipitated appearance of AMVC causes the patient much anxiety. RaceNo racial predilection is reported. SexThe female-to-male ratio is approximately equal. AgeThe reported age range varies from 5-37 years, with the disorder usually appearing in young adulthood. CLINICALSection 3 of 10
HistoryPatients with AMVC deny preceding lesions. They may report a slight erythema or pruritus followed 2 days later by a spontaneously formed scar. PhysicalAMVC may begin with a slight erythema and mild pruritus, followed in 1-2 days by a scar that does not change in size or shape. The shapes of the scars or depressions have been described as linear, round, irregularly round, and varioliform. They are sharply demarcated, flesh-colored shallow depressions of 1 mm subjective depth and may be located bilaterally on the temporal, infraorbital, buccal, mandibular, and mental regions.
CausesNo definitive cause has been established, although skin biopsy specimens for histologic and ultrastructural studies suggest an underlying elastic tissue disorder. Familial cases of AMVC have also been documented. The few associated findings noted were extrahepatic biliary disease and pachydermodactyly. DIFFERENTIALSSection 4 of 10
Anetoderma Ulerythema
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| Media file 1: Atrophia maculosa varioliformis cutis on the cheek presents as curvilinear sharply defined scars or depressions of varying lengths. | |
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| Media file 2: Histopathology of atrophia maculosa varioliformis cutis shows multiple small areas of diminished and fragmented elastic tissue with Verhoeff-van Gieson stain in the superficial and mid dermis, particularly in the lower aspects of this image. | |
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| Media file 3: Round well-demarcated varioliform scars of atrophia maculosa varioliformis cutis on the temple of the same patient as in Image 1. | |
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Atrophia Maculosa Varioliformis Cutis excerpt
Article Last Updated: Feb 15, 2007
