Cigarette Paper Scars: Characteristic of Acrodermatitis Chronica Atrophicans
Cigarette paper scars (also called "cigarette paper skin") are characteristic of acrodermatitis chronica atrophicans, a late-stage cutaneous manifestation of Lyme disease caused by Borrelia infection. 1
Clinical Context and Pathophysiology
- Acrodermatitis chronica atrophicans develops insidiously several years after initial Borrelia infection (range 0.5-8 years), making it a late manifestation of Lyme disease 1
- The condition is caused most commonly by B. afzelii, making it far more prevalent in Europe than in the United States 1
- Approximately 20% of patients report a preceding erythema migrans lesion, typically on the same extremity 1
- The condition is diagnosed most frequently in women over 40 years of age 1
Characteristic Skin Changes
The evolution of skin changes follows a predictable pattern:
- Early lesions present with bluish-red discoloration and doughy swelling, most commonly on the extensor surfaces of hands and feet 1
- Initially unilateral, the lesion may later become bilateral 1
- The lesion enlarges slowly over months to years, with resolution of edema and progressive development of skin atrophy that produces the characteristic "cigarette paper skin" appearance 1
- Nodules may develop over bony prominences such as the elbow or patella 1
- Sclerosing lesions can develop in some patients 1
- Prominent veins become visible due to skin atrophy, which may lead to misdiagnosis as venous insufficiency 1
Associated Neurological Features
- Approximately two-thirds of patients develop an associated peripheral neuropathy, typically involving the affected extremity and manifested primarily as local sensory loss 1
Diagnostic Approach
The diagnosis requires multiple elements:
- Appropriate epidemiological exposure history (endemic area for Lyme disease) 1
- Clinical characteristics as described above 1
- Histopathological findings showing pronounced lymphoplasmacellular infiltration of the skin and sometimes subcutis, with or without atrophy 1
- IgG seropositivity for Borrelia 1
Important Clinical Distinction
This term "cigarette paper" should not be confused with the scarified "cigarette paper" skin lesions seen in atretic meningoceles, which are congenital spinal malformations 1. The context and associated clinical features clearly distinguish these two entities—acrodermatitis chronica atrophicans is an acquired infectious disease with characteristic distribution and progression, while atretic meningocele is a congenital spinal defect.