First case is a female, 38 years of age presenting with asymptomatic, slightly pigmented, round, scar-mark like atrophic lesions present on face, neck, upper chest, back, forearms and arms since last 18 years with gradual increase in the number of lesions [ijdvl.com]

It appears to only affect children and young adults, is considered to be quite rare, normally occurs on the cheeks, temple area and forehead, and is neither well understood nor presently treatable. [en.wikipedia.org]

Atrophia maculosa varioliformis cutis on the cheek presents as curvilinear sharply defined scars or depressions of varying lengths. [emedicine.medscape.com]

CASE REPORT A 42-year-old Korean woman presented with a few months history of unusual pitted scars on her left chin. [ncbi.nlm.nih.gov]

• Asymptomatic

  The lesions were totally asymptomatic in nature. Biopsy was not done as the parents did not consent for the same. [ijdvl.com]

  Most patients are asymptomatic. [13] The most frequently involved sites are the bilateral buccal regions (ie, the cheeks). The scars are almost always located on the face. [emedicine.medscape.com]

• Photosensitivity

  There was a slight aggravation of lesions in summer months as new lesions progressed more in size in these months, although progression was there in winters also; but no erythema or photosensitivity was reported. [ijdvl.com]

• Headache

  Presently she is on the same treatment with gradual tapering of the doses and proper follow-up in terms of monitoring of any history of depressive symptoms, headache, total and differential leucocyte counts, lipid profile and liver function tests. [ijdvl.com]

Figure 7: Post-isotretinoin treatment of patient showing significant improvement of lesions [ijdvl.com]

Otherwise, the correct diagnosis is usually not made, and even doing so provides little benefit; there is no treatment. It has been suggested in case reports that the condition, although rare, is likely underreported. [en.wikipedia.org]

Treatment No standard of medical care or treatment for atrophia maculosa varioliformis cutis (AMVC) has been discussed in the literature. [emedicine.medscape.com]

[…] histologic and ultrastructural studies suggest an underlying elastic tissue disorder. [5] Familial cases of AMVC have also been documented. [3, 6, 7, 8] The few associated findings noted were extrahepatic biliary disease [9] and pachydermodactyly. [10] Prognosis [emedicine.medscape.com]

Its etiology remains unknown, but elastic tissue pathology has been reported in histology findings. [emedicine.medscape.com]

Figure 5: Child of proband showing atrophia maculosa varioliformis cutis lesions on cheek region Figure 6: Pedigree chart of family showing disease across five generations of the disease Discussion The etiology and molecular basis of F-AMVC remains unclear [ijdvl.com]

The etiology of AMVC is unknown, although a familial occurrence has previously been reported2,3, which suggests that AMVC is an inherited disorder rather than a response to an environmental insult. [ncbi.nlm.nih.gov]

Topical and oral retinoids are sometimes utilized, with newer options including dermabrasion, collagen injections, or laser resurfacing techniques. [11] Epidemiology No racial predilection is reported for atrophia maculosa varioliformis cutis (AMVC). [emedicine.medscape.com]

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 [emedicine.medscape.com]