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).
A 35-year-old woman presents with diffuse wrinkling on her trunk and neck. What are the possible mechanisms for this condition?
Possible mechanisms for middermal elastolysis (MDE) include elastolytic overactivity, altered reassembly of elastic fibers, UV exposure, autoimmunity, and damage by elastase.
What are the clinical features of Middermal Elastolysis (MDE) and how do they differ from other elastolytic disorders?
Clinical Features of MDE:
- Characterized by patches and plaques of diffuse, fine, wrinkled skin.
- Most often located on the trunk, neck, and arms.
- Types of MDE:
- Type I: Asymptomatic, well-demarcated or diffuse areas of fine wrinkling, usually symmetric.
- Type II: Discrete perifollicular papules, leaving the hair follicle as an indented center.
- Type III: Reticular pattern with erythematous patches and telangiectasia.
- Chronic appearance gives the skin a prematurely aged look.
- No history of preceding inflammatory dermatosis or systemic involvement.
Differences from Other Disorders:
- MDE shows larger areas of diffuse wrinkling without herniation, while anetoderma presents with smaller soft macules and papules.
Discuss the pathogenesis of Middermal Elastolysis (MDE) and its potential contributing factors.
Pathogenesis of MDE:
- May be secondary to an elastolytic overactivity or altered reassembly of elastic fibers.
- Contributing Factors:
- UV Exposure: Major factor in the degeneration of elastic fibers.
- Defects in Elastic Fiber Synthesis: Autoimmunity against elastic fibers.
What is the difference between DE and anetoderma?
DE shows larger areas of diffuse wrinkling without herniation, while anetoderma presents with smaller soft macules and papules.
What is the pathogenesis of Middermal Elastolysis (MDE)?
The pathogenesis of MDE may involve elastolytic overactivity or altered reassembly of elastic fibers.
What are the contributing factors to Middermal Elastolysis?
Contributing factors include UV exposure, defects in elastic fiber synthesis, inflammatory processes, dendritic fibroblasts, and decreased expression of certain proteins.
What are the differential diagnoses for Middermal Elastolysis?
Differential diagnoses include solar elastosis, anetoderma, perifollicular elastolysis, and postinflammatory elastolysis.
How can solar elastosis be distinguished from MDE?
Solar elastosis has onset in older age, affects sun-exposed areas, and presents with yellowish color and coarser wrinkling.
What are the clinical features of Middermal Elastolysis (MDE)?
Clinical features include asymptomatic areas of fine wrinkling, discrete perifollicular papules, and a reticular pattern with erythematous patches.
How do MDE and other elastolytic disorders differ?
MDE presents with larger areas of diffuse wrinkling without herniation, while other disorders like solar elastosis and anetoderma show different patterns.
What is the overall incidence of striae during pregnancy?
The overall incidence of striae during pregnancy is up to 90%.
What are the common sites for striae to appear in girls during puberty?
In girls, striae commonly appear on the breasts, thighs, hips, and buttocks.
What are the characteristics of striae rubra?
Striae rubra initially appear as red-to-violaceous elevated lines.
What is the difference between striae rubra and striae alba?
Striae rubra are red-to-violaceous elevated lines, while striae alba are atrophic lesions that gradually fade to a fine, white, wrinkled appearance.
What is the pathogenesis of striae?
Striae are caused by breaks in the connective tissue, resulting in dermal atrophy, influenced by hormones, mechanical stress, and genetic predisposition.
What is the treatment for striae using topical agents?
Topical treatments include tretinoin 0.1% cream, a combination of 0.05% tretinoin/20% glycolic acid, and 10% ascorbic acid/20% glycolic acid.
What are the clinical features of Idiopathic Atrophoderma of Pasini and Pierini?
It presents as well-demarcated depressed patches on the trunk, symmetric and bilateral distribution, usually asymptomatic and lacking inflammation.
What is the epidemiology of Idiopathic Atrophoderma of Pasini and Pierini?
It is more common in women than men (ratio 6:1) and typically starts insidiously in young individuals in the 2nd to 3rd decades of life.
What is the treatment for Idiopathic Atrophoderma of Pasini and Pierini?
No treatment has been proven effective, but oral hydroxychloroquine has shown improvement in some cases, and Q-switched alexandrite laser may decrease hyperpigmentation.
What are the differential diagnoses for striae?
Differential diagnoses include linear focal elastosis and keloidal repair of striae distensae.
What is the condition for a 16-year-old girl with linear atrophic lesions on her thighs?
The condition is striae distensae. Histological changes include decreased dermal thickness and fragmented dermal elastin.
What is the diagnosis for a young adult male with depressed patches on his back?
The diagnosis is idiopathic atrophoderma of Pasini and Pierini. The typical course is progressive.
What is the diagnosis for a patient with yellow palpable striae-like bands on the lower back?
The diagnosis is linear focal elastosis. Histological findings include a focal increase in elastic fibers.
What is the diagnosis for a 30-year-old woman with depressed patches on her lumbosacral region?
The diagnosis is idiopathic atrophoderma of Pasini and Pierini. Histological findings include homogenization of collagen bundles.
What is the diagnosis for a 50-year-old man with yellow striae-like bands?
The diagnosis is linear focal elastosis. The most commonly affected demographic is elderly men.
What are the two stages of striae and how do they differ?
The two stages are striae rubra (initially red-to-violaceous) and striae alba (faded, atrophic lesions).
What is the clinical appearance of multiple lesions in idiopathic atrophoderma of Pasini and Pierini?
The clinical appearance is described as a Swiss cheese appearance.
What are the common sites for striae during puberty in girls?
Common sites include the breasts, thighs, hips, and buttocks.
What is the histological finding in linear focal elastosis?
Histological findings include a focal increase in the number of elongated or fragmented elastic fibers.
What are the histological changes in striae?
Histological changes include decreased dermal thickness, thinned collagen bundles, and fragmented dermal elastin.
What are the clinical features of striae rubra and striae alba?
Striae rubra are red-to-violaceous elevated lines, while striae alba fade to a fine, white, wrinkled appearance.
What factors contribute to striae development during pregnancy?
Factors include rapid increase in size, younger primigravidas developing more striae, and larger weight gain.
What is the differential diagnosis for striae?
Linear focal elastosis is characterized by yellow, palpable striae-like bands, while striae distensae are linear atrophic lesions.
What are the treatment options for striae?
Topical treatments have limited effectiveness, while laser treatments show moderate improvement.
What are the clinical features and epidemiology of idiopathic atrophoderma of Pasini and Pierini?
Epidemiology: Women > Men (ratio 6:1). Clinical features: Well-demarcated depressed patches, usually asymptomatic.
What is the pathophysiological mechanism behind the formation of striae?
Breaks in connective tissue lead to dermal atrophy, influenced by hormones, particularly corticosteroids.
How does idiopathic atrophoderma of Pasini and Pierini present clinically?
It is characterized by well-demarcated depressed patches on the trunk, symmetric and bilateral, usually asymptomatic.
What are the distinguishing features of linear focal elastosis compared to striae?
Linear focal elastosis shows yellow, raised lesions, while striae are depressed and white.
What are the differential diagnoses for striae?
Differential diagnoses for striae include linear focal elastosis, which is characterized by yellow, palpable striae-like bands on the lower back.
How can linear focal elastosis be distinguished from striae?
Linear focal elastosis shows raised yellow lesions, while striae appear depressed and white. Linear focal elastosis has a focal increase in elastic fibers and a thickened dermis, whereas striae show a decrease in dermal thickness and collagen.
What is follicular atrophoderma and how does it present clinically?
Follicular atrophoderma refers to dimple-like depressions at the follicular orifices, often seen as ice-pick depressions around hair follicles, commonly found on cheeks and back of hands or feet.
What are the characteristics of atrophoderma vermiculatum?
Atrophoderma vermiculatum is characterized by lesions on the cheeks, with a slow, progressive course, and pitted, atrophic, and depressed scars in a reticulated or honeycomb pattern.
What is keratosis pilaris atrophicans and how does it differ from atrophoderma vermiculatum?
Keratosis pilaris atrophicans includes atrophoderma vermiculatum and is characterized by keratotic follicular papules and secondary atrophic scarring, primarily affecting the lateral portion of the eyebrows.
What are some associated syndromes with follicular atrophoderma?
Associated syndromes include Rombo syndrome, Nicolau-Balus syndrome, Tuzun syndrome, Loeys-Dietz syndrome, and Braun-Falco-Marghescu syndrome.
What are the treatment options for follicular atrophoderma?
Treatment options include systemic isotretinoin, dermabrasion, and laser treatments such as carbon dioxide and 585-nm pulsed-dye lasers.
What is the genetic location associated with Bazex-Dupré-Christol syndrome?
The genetic location associated with Bazex-Dupré-Christol syndrome is Xq24-q27.
What is the diagnosis for a child with multiple inflammatory symmetric papules on the cheeks that develop into pitted scars?
The diagnosis is atrophoderma vermiculatum, with typical onset in childhood or puberty.
What is the diagnosis for keratotic follicular papules on the malar area leading to scarring alopecia?
The diagnosis is keratosis pilaris atrophicans, with an underlying defect of abnormal follicular hyperkeratinization.
What is the diagnosis for ice-pick depressions around hair follicles on a child’s cheeks?
The diagnosis is follicular atrophoderma, associated with atrophoderma vermiculatum and keratosis pilaris atrophicans.
What is the diagnosis for ichthyosiform scaling erythroderma that resolves in the first year of life?
The diagnosis is Conradi-Hünermann-Happle syndrome, with a mutation in the emopamil-binding protein gene at Xp11.23-p11.22.
What is the diagnosis for multiple basal cell carcinomas and follicular atrophoderma?
The diagnosis is Bazex-Dupré-Christol syndrome, inherited in an X-linked dominant manner.
What is the diagnosis for keratotic follicular papules on the lateral portion of the eyebrows?
The diagnosis is ulerythema ophryogenes, differing from atrophoderma vermiculatum by primarily affecting the lateral portion of the eyebrows.
What are the associated features of Conradi-Hünermann-Happle syndrome?
Associated features include hyperpigmentation, cataracts, scarring alopecia, saddle-nose deformity, and stippled calcifications of the epiphyses.
What is the inheritance pattern for keratosis pilaris atrophicans?
The inheritance pattern in some cases is X-linked recessive.
What are the histological findings in newborns with Conradi-Hünermann-Happle syndrome?
Histological findings include ichthyosis with keratotic follicular plugs containing dystrophic calcification.
What are the clinical features of Atrophoderma Vermiculatum?
Atrophoderma Vermiculatum features lesions on the cheeks, onset during childhood or puberty, and a slow, progressive course with pitted, atrophic scars.
How does Bazex-Dupré-Christol Syndrome present clinically?
Bazex-Dupré-Christol Syndrome is characterized by follicular atrophoderma, milia, basal cell carcinomas, and other findings like facial hyperpigmentation.
What is the significance of systemic isotretinoin in treating follicular atrophoderma?
Systemic isotretinoin stops progression and induces remission of follicular atrophoderma.
What are the clinical implications of Conradi-Hünermann-Happle Syndrome?
Conradi-Hünermann-Happle Syndrome is an X-linked dominant disorder with ichthyosiform scaling erythroderma and other associated features.
How does Keratosis Pilaris Atrophicans differ from Atrophoderma Vermiculatum?
Keratosis Pilaris Atrophicans affects multiple areas including the malar region and leads to scarring alopecia, while Atrophoderma Vermiculatum is limited to the cheeks.
What are the therapeutic options for managing Follicular Atrophoderma?
Therapeutic options include systemic isotretinoin, dermabrasion, and laser treatments.
What are the key features of associated syndromes with Follicular Atrophoderma?
Key features include milia, basal cell carcinomas, and hypotrichosis in Rombo syndrome, and syringomas in Nicolau-Balus syndrome.
What protein is expressed in lower levels in anetoderma?
Fibulin (involved in elastic fiber reassembly)
What sign is seen in anetoderma?
Buttonhole sign
MC age and sex involved in anetoderma?
15-30 yo / female
MC sites involved in anetoderma?
chest, back, upper neck, extremities
Type of primary anetoderma with preceding inflammatory lesions, mainly erythema?
Jadassohn-Pellizzari type
Type of primary anetoderma with no preceding inflammatory lesions?
Schweninger-buzzi type
MC cause of secondary anetoderma? Give 2.
Acne and Varicella
