70: Anetoderma and Other Atrophic Disorders of the Skin Flashcards
What is the key defect in the pathogenesis of anetoderma?
The key defect in the pathogenesis of anetoderma is damage to the dermal elastic fibers.
What are the characteristic lesions of anetoderma?
The characteristic lesions of anetoderma include:
- Flaccid circumscribed areas of slack skin
- Impression of loss of dermal substance forming depressions, wrinkling, or sac-like protrusions
- Size ranges from 5 to 30 mm in diameter
- Skin surface can be wrinkled, thinned, and often depigmented
- Central depression may be seen
- Buttonhole sign: finger sinks without resistance into a distinct pit with sharp borders.
What are the types of anetoderma and their subtypes?
Anetoderma is classified into two main types:
1. Primary anetoderma
- No underlying associated skin disease
- Subtypes:
- Jadassohn-Pellizzari type: with preceding inflammatory lesions, mainly erythema
- Schweninger-Buzzi type: without preceding inflammatory lesions
2. Secondary anetoderma
- Characteristic atrophic lesion appears in the exact same site as a previous specific pathology
- Most common causes include acne and varicella.
What is the predominant defect observed in the pathology of anetoderma?
The predominant defect observed in the pathology of anetoderma is the focal partial or complete loss of elastic tissue in the papillary and/or midreticular dermis.
What are the differential diagnoses for primary anetoderma?
The differential diagnoses for primary anetoderma include:
- Keloids: form nodules that are much firmer on palpation, often with a history of trauma.
- Glucocorticoid-induced atrophy: occurs most commonly over the triceps or buttocks at sites where injections are usually given, resembling atrophoderma, and may show steroid crystals in the dermis upon polarization.
A patient with a history of acne presents with atrophic lesions at the exact sites of previous acne lesions. What type of anetoderma is this, and what is the most common cause?
This is secondary anetoderma. The most common cause is acne.
A 25-year-old woman presents with flaccid, circumscribed areas of slack skin on her chest and back. The lesions are 5-30 mm in diameter, with central depressions and a buttonhole sign. What is the most likely diagnosis, and what are the key pathological findings?
The most likely diagnosis is anetoderma. Key pathological findings include focal partial or complete loss of elastic tissue in the papillary and/or midreticular dermis, with some residual abnormal, irregular, and fragmented elastic fibers.
A 20-year-old woman presents with flaccid, circumscribed areas of slack skin on her upper extremities. The lesions have a buttonhole sign. What are the two subtypes of primary anetoderma, and how do they differ?
The two subtypes of primary anetoderma are Jadassohn-Pellizzari type (with preceding inflammatory lesions, mainly erythema) and Schweninger-Buzzi type (without preceding inflammatory lesions).
A 25-year-old woman presents with flaccid, circumscribed areas of slack skin on her chest and back. What are the most common sites for this condition?
The most common sites for anetoderma are the chest, back, neck, and upper extremities.
A 20-year-old woman presents with flaccid, circumscribed areas of slack skin on her upper extremities. What is the key defect in this condition?
The key defect in anetoderma is damage to the dermal elastic fibers, resulting in loss of dermal elastin.
What are the key clinical features of primary anetoderma and how do they differ from secondary anetoderma?
Primary Anetoderma:
- No underlying associated skin disease.
- Characterized by flaccid circumscribed areas of slack skin, forming depressions and wrinkles.
- Subtypes include:
1. Jadassohn-Pellizzari type – with preceding inflammatory lesions, mainly erythema.
2. Schweninger-Buzzi type – without preceding inflammatory lesions.
Secondary Anetoderma:
- Characteristic atrophic lesion appears in the exact same site as a previous specific pathology.
- Most common causes include acne and varicella.
Discuss the pathogenesis of anetoderma and the significance of dermal elastic fiber damage.
The pathogenesis of anetoderma is largely unknown, but it is characterized by:
- Key defect: Damage to the dermal elastic fibers.
- Loss of dermal elastin may result from:
- Increased elastin destruction.
- Decreased elastin synthesis.
- There is also a decrease in fibulin protein, which is crucial for maintaining skin elasticity.
What are the differential diagnoses for primary anetoderma and how can they be distinguished?
The differential diagnoses for primary anetoderma include:
| Condition | Key Features |
|———————————-|——————————————————————————|
| Keloids | - Nodules that are firmer on palpation.
- (+) history of trauma.
- Distinct pathology. |
| Glucocorticoid-induced atrophy | - Commonly occurs over triceps or buttocks at injection sites.
- Lesions resemble atrophoderma.
- Histopathology may show steroid crystals in the dermis. |
What are the clinical features of Middermal Elastolysis (MDE)?
MDE is characterized by:
- Patches and plaques of diffuse, fine, wrinkled skin
- Most often located on the trunk, neck, and arms
- Types of MDE:
1. Type I: Asymptomatic, well-demarcated or diffuse areas of fine wrinkling, usually symmetric.
2. Type II: Discrete perifollicular papules, leaving the hair follicle as an indented center.
3. Type III: Reticular pattern with erythematous patches and telangiectasia.
- Chronic appearance, giving the skin a prematurely aged look.
- No history of preceding inflammatory dermatosis or systemic involvement.
What are the differential diagnoses for Middermal Elastolysis (MDE)?
The differential diagnoses for MDE include:
| Condition | Key Features |
|——————————-|————————————————————————————————–|
| Solar elastosis | - Onset in older age group
- Only affects sun-exposed areas
- Yellowish color and coarser wrinkling
- Hyperplasia and abnormalities of elastic fibers in the papillary dermis |
| Anetoderma | - Smaller soft macules and papules instead of diffuse wrinkling
- Elastolysis can occur in any layer of the dermis |
| Perifollicular elastolysis | - Selective loss of elastic fibers surrounding hair follicles
- Preservation of elastic fibers around follicles in MDE |
| Postinflammatory elastolysis and cutis laxa | - Affects young girls of African descent
- Inflammatory phase with indurated plaques or urticaria, malaise, and fever preceding diffuse wrinkling and atrophy |
What are the treatment options for Middermal Elastolysis (MDE)?
Currently, there is no known effective treatment for MDE. However, some options include:
- Sunscreens
- Colchicine
- Chloroquine
- Vitamin E
- Topical retinoic acid (ineffective)
- Topical soybean extract and eicosapentaenoic acid (other options)
What is the pathogenesis of Middermal Elastolysis (MDE)?
The pathogenesis of MDE may involve:
- Elastolytic overactivity or altered reassembly of elastic fibers
- UV exposure as a major contributing factor in the degeneration of elastic fibers
- Other mechanisms:
- Defects in the synthesis of elastic fibers
- Autoimmunity against elastic fibers
- Damage to elastic fibers through the release of elastase by inflammatory cells or fibroblasts
- Inflammatory processes and an imbalance between matrix metalloproteinases and tissue inhibitors of metalloproteinases may occur.
A dermatologist observes a patient with diffuse wrinkling on the trunk and neck, without herniation. Elastic tissue stains reveal a selective band-like loss of elastic fibers in the middermis. What is the diagnosis, and what are the contributing factors?
The diagnosis is middermal elastolysis (MDE). Contributing factors include UV exposure, defects in elastic fiber synthesis, autoimmunity, and damage to elastic fibers by elastase from inflammatory cells or fibroblasts.
A 35-year-old woman presents with diffuse, fine wrinkling on her arms and trunk, giving a prematurely aged appearance. There is no history of preceding inflammatory dermatosis. What is the diagnosis, and what are the three clinical types?
The diagnosis is middermal elastolysis (MDE). The three clinical types are: Type I (asymptomatic, well-demarcated or diffuse areas of fine wrinkling), Type II (discrete perifollicular papules with indented centers), and Type III (reticular pattern with erythematous patches and telangiectasia).
A 40-year-old woman presents with yellowish, coarser wrinkling on sun-exposed areas. Histology shows hyperplasia and abnormalities of elastic fibers, along with basophilic degeneration of collagen in the papillary dermis. What is the diagnosis?
The diagnosis is solar elastosis.
A 45-year-old woman presents with diffuse wrinkling on her trunk and arms. Optical coherence microscopy reveals selective band-like loss of elastic fibers in the middermis. What is the diagnosis, and what are the possible mechanisms?
The diagnosis is middermal elastolysis (MDE). Possible mechanisms include elastolytic overactivity, altered reassembly of elastic fibers, UV exposure, autoimmunity, and damage by elastase.
A 35-year-old woman presents with diffuse wrinkling on her trunk and neck. Histology shows macrophagic elastophagocytosis. What is the diagnosis, and what are the histological features?
The diagnosis is middermal elastolysis (MDE). Histological features include selective band-like loss of elastic fibers in the middermis and preservation of normal elastic tissue in the superficial papillary dermis and reticular dermis.
A 40-year-old woman presents with diffuse wrinkling on her trunk and arms. What diagnostic tools can be used to confirm the condition?
Diagnostic tools include optical coherence microscopy and high-frequency ultrasound.
A 45-year-old woman presents with diffuse wrinkling on her trunk and arms. What are the three clinical types of this condition, and how do they differ?
The three clinical types of middermal elastolysis (MDE) are: Type I (asymptomatic, well-demarcated or diffuse areas of fine wrinkling), Type II (discrete perifollicular papules with indented centers), and Type III (reticular pattern with erythematous patches and telangiectasia).
