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Linear atrophoderma of Moulin: a distinct entity?
Emily de Golian1, Kathryn Echols, Henna Pearl
1School of Medicine, Medical College of Georgia, Georgia Health Sciences University, Augusta, Georgia.
Pediatric Dermatology
|October 11, 2012
Summary
Linear atrophoderma of Moulin (LAM) presents with skin lesions along Blaschko lines. A case study suggests LAM, atrophoderma of Pasini and Pierini (APP), and morphea may be part of a single disease spectrum.
Area of Science:
- Dermatology
- Pathology
Background:
- Linear atrophoderma of Moulin (LAM) is a rare condition with hyperpigmented atrophoderma following Blaschko lines.
- Clinical and histological similarities exist between LAM, atrophoderma of Pasini and Pierini (APP), and morphea, leading to debate about their classification.
- The relationship between these conditions is often considered a spectrum of disorders.
Observation:
- A 16-year-old boy presented with clinical features supporting LAM, including lesions along Blaschko lines and perivascular lymphocytic infiltrate on biopsy.
- The patient exhibited bilateral lesions characteristic of APP and collagen entrapment of eccrine ducts seen in morphea.
- Histological examination revealed changes in dermal collagen consistent with features across all three conditions.
Findings:
- The case supports the hypothesis that LAM, APP, and morphea represent a spectrum of related disorders rather than distinct entities.
- The patient's presentation integrated features of LAM, APP, and morphea, suggesting a continuum of disease.
- A literature review indicates an evolving definition of LAM, incorporating features of APP and morphea.
Implications:
- This case contributes to understanding the potential spectrum encompassing LAM, APP, and morphea.
- Recognizing these conditions as a spectrum may refine diagnostic criteria and treatment approaches.
- Further research is warranted to elucidate the precise relationship and underlying mechanisms of these dermatologic disorders.
