Atrophia Maculosa Varioliformis Cutis 

Updated: Jun 25, 2018
Author: Julianne H Kuflik, MD; Chief Editor: Dirk M Elston, MD 

Overview

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.

 

Presentation

History

Patients with atrophia maculosa varioliformis cutis (AMVC) deny preceding lesions. They may report a slight erythema or pruritus followed 2 days later by a spontaneously formed scar.

Physical Examination

Atrophia maculosa varioliformis cutis (AMVC) 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.

The most frequently involved sites are the bilateral buccal regions (ie, the cheeks). The scars are almost always located on the face. The length of the scars varies from 2 mm to 1.3 cm, and the width varies from 1-2 mm. These shallow pit marks are arranged parallel, perpendicular, and curvilinear to one another. The depressions are devoid of pigmentary changes from the surrounding skin. Note the images below.

Atrophia maculosa varioliformis cutis on the cheek Atrophia maculosa varioliformis cutis on the cheek presents as curvilinear sharply defined scars or depressions of varying lengths.
Round well-demarcated varioliform scars of atrophi Round well-demarcated varioliform scars of atrophia maculosa varioliformis cutis on the temple.

Patients usually do not have facial milia, comedones, papules, or cysts. Patients deny a history of scarring secondary to acne or varicella infection, and they deny the presence of a preceding skin lesion, except for the slight erythema that fades upon scar formation. The remainder of their skin examination is unremarkable.

Because atrophia maculosa varioliformis cutis can run in families, physical examination of family members may be helpful to rule out a familial disorder and discover other family members with the disease.

 

DDx

Diagnostic Considerations

Also consider keratosis pilaris atrophicans, including keratosis pilaris atrophicans faciei, or ulerythema ophryogenes; atrophoderma vermiculatum or ulerythema acneiform; and follicularis keratosis spinulosa decalvans.[15] In addition, other common causes of facial scarring, such as a history of varicella zoster virus infection, molluscum contagiosum infection,[13] scarring acne, and herpes virus infection, should be considered.

Differential Diagnoses

 

Workup

Procedures

A skin biopsy specimen from the atrophia maculosa varioliformis cutis (AMVC) patient may be sent for histological examination, including special stains for collagen and elastic fibers. An ultrastructural study on the tissue specimen may be considered to help diagnose the disorder and to rule out other diseases in the differential diagnosis.

Histologic Findings

Skin biopsy specimens show decreased or fragmented elastic tissue in the superficial and mid dermis and occasional epidermal thinning or slight epidermal depressions or dells. Note the image below.

Histopathology of atrophia maculosa varioliformis 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.
 

Treatment

Medical Care

No standard of medical care or treatment for atrophia maculosa varioliformis cutis (AMVC) has been discussed in the literature.