Background
Atrophia maculosa varioliformis cutis (AMVC) is a rare disease that presents as spontaneously formed facial scars in young adults. In 1918, Heidingsfeld [1] 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.
Pedigree analysis suggests that AMVC is of autosomal dominant inheritance. [2, 3]
Pathophysiology
Although its etiology is unknown, atrophia maculosa varioliformis cutis (AMVC) may represent an underlying defect of dermal elastin as demonstrated by histologic and ultrastructural findings. [4] AMVC has been documented only in the skin.
Etiology
No definitive cause has been established for atrophia maculosa varioliformis cutis (AMVC), although skin biopsy specimens for histologic and ultrastructural studies suggest an underlying elastic tissue disorder. [14] Familial cases of AMVC have also been documented. [3, 7, 8, 9] The few associated findings noted were extrahepatic biliary disease [10] and pachydermodactyly. [11]
Epidemiology
Race
No racial predilection is reported for atrophia maculosa varioliformis cutis (AMVC).
Sex
The female-to-male ratio for atrophia maculosa varioliformis cutis (AMVC) is approximately equal.
Age
The reported age range of atrophia maculosa varioliformis cutis (AMVC) varies from 5-37 years, [5] with the disorder usually appearing in young adulthood.
Prognosis
In addition to being of cosmetic concern to a patient, the sudden unprecipitated appearance of atrophia maculosa varioliformis cutis (AMVC) causes the patient much anxiety.
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Atrophia maculosa varioliformis cutis on the cheek presents as curvilinear sharply defined scars or depressions of varying lengths.
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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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Round well-demarcated varioliform scars of atrophia maculosa varioliformis cutis on the temple.
