73: Panniculitis Flashcards
What are the two histopathologic classifications of panniculitis?
The two histopathologic classifications of panniculitis are septal and lobular. This classification is further expanded by noting the presence or absence of vasculitis and the composition of the inflammatory infiltrate.
What role do Toll-like receptors (TLRs) play in the immune response of adipocytes?
Toll-like receptors (TLRs) are a type of transmembrane pattern recognition receptor (PRR) that detect cell-surface microbial patterns. They activate proinflammatory signaling pathways and trigger adaptive immunity responses, including antibody responses and T-helper cell activation.
What are the main functions of adipocyte tissue (AT)?
Adipocyte tissue (AT) functions in:
1. Energy storage and expenditure
2. Appetite modulation
3. Insulin sensitivity
4. Endocrine and reproductive systems
5. Bone metabolism
6. Inflammation
7. Immunity
How do macrophage-derived cytokines affect adipocytes?
Macrophage-derived cytokines and chemokines, including TNF-α, induce adipocyte lipolysis, leading to the release of free fatty acids from the adipocyte, which in turn induces proinflammatory signaling.
What types of cells are found in white adipose tissue (AT)?
The types of cells found in white adipose tissue (AT) include:
1. Adipocytes (the most abundant cells)
2. Pericytes
3. Fibroblasts
4. Endothelial cells
5. Vascular smooth muscle cells
6. Inflammatory cells (especially macrophages)
What is Erythema Nodosum (EN) and its clinical significance?
Erythema Nodosum (EN) is the prototypic septal panniculitis characterized by tender, erythematous, warm nodules and plaques primarily on the lower legs. It is common and can have multiple etiologies, with many cases being idiopathic. The diagnosis is often clinical, supported by histopathology showing septal inflammation. Clinical findings are usually self-limiting.
What are the epidemiological features of Erythema Nodosum?
Erythema Nodosum occurs in all ages but predominantly affects young women in the second to fourth decades of life. Over 80% of EN patients are female, with a female-to-male ratio of 5:1. There is no gender difference in pediatric cases, and the prevalence in England and Spain is approximately 0.38% to 0.5% of patients seen in clinics.
What are the common clinical features associated with Erythema Nodosum?
Erythema Nodosum presents with:
1. Tender, erythematous, warm nodules and plaques on the lower legs.
2. Most commonly affects the anterior lower legs and ankles, but can also involve forearms, upper legs, trunk, and face.
3. Nodules may become confluent and violaceous, resembling bruises (erythema contusiformis) when hemorrhage is present.
4. Systemic symptoms may include fever, malaise, fatigue, arthralgia, and headache.
5. Ulceration and scarring are not typically seen.
What are the potential etiologies and pathogenesis of Erythema Nodosum?
Erythema Nodosum is associated with numerous etiologies, with 37% to 60% being idiopathic. Potential causes include:
- Infections (bacterial, viral, fungal, protozoan), particularly streptococcal respiratory tract infections in pediatric cases.
- Medications such as antibiotics, oral contraceptives, and nonsteroidal anti-inflammatories.
- Autoimmune diseases and inflammatory disorders.
- Malignancies, most commonly leukemias or lymphomas.
A patient presents with tender, erythematous nodules on the lower legs, accompanied by fever and fatigue. What is the likely diagnosis?
The likely diagnosis is erythema nodosum (EN). Systemic symptoms that might accompany this condition include fever, malaise, fatigue, arthralgia, arthritis, headache, and, more rarely, abdominal pain, vomiting, diarrhea, or cough.
A patient with erythema nodosum has a positive throat culture. What is the likely underlying cause?
The likely underlying cause is a streptococcal infection. Other laboratory findings might include leukocytosis and a positive purified protein derivative test suggesting tuberculosis infection.
A patient with erythema nodosum has a history of using oral contraceptives. How has the prevalence of this association changed?
The prevalence of oral contraceptive-induced erythema nodosum has decreased with the introduction of low-dose estrogen therapy. Other medications linked to this condition include antibiotics, nonsteroidal antiinflammatories, and leukotriene inhibitors.
A patient with erythema nodosum has a history of autoimmune disease. What are the common etiologies?
Common etiologies include infections, medications, malignancies, and autoimmune diseases. The condition typically presents with tender, erythematous nodules on the lower legs.
A patient with erythema nodosum has a history of streptococcal infection. What laboratory findings support this diagnosis?
Laboratory findings include a positive throat culture and leukocytosis. The clinical course is typically self-limiting, resolving within a few weeks.
What are the common clinical features of Erythema Nodosum (EN) and how do they present in patients?
Erythema Nodosum (EN) presents with:
- Tender, erythematous, warm nodules and plaques on the lower legs.
- Most commonly affects the anterior lower legs and ankles, but can also involve the forearms, upper legs, trunk, and face.
- Nodules may become confluent and violaceous, resembling bruises (erythema contusiformis) when hemorrhage is present.
- No ulceration or scarring is observed.
- Associated systemic symptoms may include fever, malaise, fatigue, arthralgia, arthritis, headache, and occasionally abdominal pain, vomiting, diarrhea, or cough.
Discuss the epidemiology of Erythema Nodosum (EN) and its demographic characteristics.
The epidemiology of Erythema Nodosum (EN) includes:
- Occurs in all ages, but predominantly affects young women in the second to fourth decades of life.
- Over 80% of EN patients are female, with a female-to-male ratio of 5:1.
- No gender difference is noted in pediatric cases.
- Prevalence rates in England and Spain range from 0.38% to 0.5% of patients seen in clinics.
What are the potential etiologies associated with Erythema Nodosum (EN) and how do they impact diagnosis?
Erythema Nodosum (EN) is associated with various etiologies:
- 37% to 60% of cases are idiopathic.
- Documented causes include infections, medications, malignancies, autoimmune diseases, and inflammatory disorders.
- Infectious causes can be bacterial, viral, fungal, or protozoan, with streptococcal respiratory tract infections being the most common in pediatric cases.
What are some known etiologies of Erythema Nodosum (EN)?
Known etiologies of Erythema Nodosum include:
- Sarcoidosis
- Inflammatory bowel disease
- Temporal arteritis
- Hypersensitivity reactions to various conditions
What is the typical pattern of inflammation seen in Erythema Nodosum (EN) biopsies?
Erythema Nodosum (EN) is generally characterized by a septal pattern of inflammation, which is confined predominantly to the septa. In contrast, the lobular form implies inflammation predominantly involving the fat lobule itself.
What are the clinical features of early and late Erythema Nodosum (EN)?
Early Erythema Nodosum (EN) features:
- Edema of the adipose septae
- Presence of neutrophils and extravasated red blood cells
Late Erythema Nodosum (EN) features:
- Fibrotic, wide septae often with granulomas
- Fat lobules may be encroached upon and partially effaced
- An overlying superficial and deep dermal perivascular infiltrate is frequently present.
What is the prognosis for Erythema Nodosum (EN) after initial presentation?
Erythema Nodosum (EN) is a benign, self-limiting subcutaneous disease that typically resolves a few weeks after initial presentation. The course may vary based on the etiology.
A patient with erythema nodosum has a history of recent travel and exposure to tuberculosis. What diagnostic tests should be performed?
Diagnostic tests should include a purified protein derivative test, chest radiograph, and possibly a biopsy. Histopathologic features of erythema nodosum include septal inflammation, edema of the adipose septae with neutrophils, and late-stage fibrotic septae with granulomas.
A patient with erythema nodosum has a history of sarcoidosis. How does this association affect the prognosis?
The association with sarcoidosis is linked to a less severe and shorter duration of the disease, especially in those carrying the HLA-DRB1 03-positive leukocyte antigen.
A patient with erythema nodosum has a history of streptococcal infection. What systemic symptoms are common?
Systemic symptoms include fever, malaise, fatigue, and arthralgia. The condition is typically self-limiting and resolves a few weeks after initial presentation.
A patient with erythema nodosum has a history of tuberculosis. What histopathologic features are characteristic?
Histopathologic features include septal inflammation, edema, and fibrotic septae. Miescher granuloma, a grouping of histiocytes surrounding a central stellate cleft, is characteristic but not specific.
What are the potential etiologies associated with Erythema Nodosum (EN) and how do they relate to immune responses?
Potential etiologies of Erythema Nodosum (EN) include:
- Sarcoidosis
- Inflammatory bowel disease
- Temporal arteritis
These conditions are linked to hypersensitivity reactions, immune complex deposition, and neutrophilic involvement.
How does the histopathological presentation of early versus late Erythema Nodosum differ?
The histopathological presentation of Erythema Nodosum (EN) varies between early and late stages:
- Early EN: Shows edema of the adipose septae, presence of neutrophils and extravasated red blood cells.
- Late EN: Exhibits fibrotic, wide septae, often contains granulomas, and an overlying superficial and deep dermal perivascular infiltrate is frequently present.
What is the significance of genetic mutations in the context of Erythema Nodosum and its inflammatory responses?
Certain genetic mutations associated with enhanced inflammatory reactions may confer resistance to specific pathogens in Erythema Nodosum (EN). These mutations can influence the innate immune system’s function.
What reactions may confer resistance to specific pathogens in Erythema Nodosum (EN)?
Mutations can influence the innate immune system’s function, leading to high expression of cytokines such as TNF-α, IL-6, and IL-8, and increased levels of monocyte chemoattractant protein-1 and granulocyte colony-stimulating factor.
What are the clinical implications of the differential diagnosis of Erythema Nodosum?
The differential diagnosis includes conditions such as Erythema induratum, Sarcoidosis, Cellulitis, Lipodermatosclerosis, and Pancreatic granulomatosis. Understanding these conditions is crucial because they can present with similar clinical features but require different management strategies.
What is the management focus for Erythema Nodosum (EN)?
The management focuses on eliminating exposure to provoking factors, treating underlying diseases, seeking and treating potential infections, discontinuing suspected medications, and providing supportive care.
What are the clinical features of Erythema Induratum (EI) and Nodular Vasculitis (NV)?
Clinical features include recurrent, erythematous to violaceous nodules and deep plaques primarily affecting the calves, which may be tender but often are not painful. Ulceration can lead to scarring.
What is the epidemiology of Erythema Induratum and Nodular Vasculitis (EI/NV)?
EI/NV is the most common diagnosis of a panniculitis with vasculitis, predominantly affecting young to middle-aged women with a 9:1 ratio, and lesions develop more frequently during winter.
What is the etiology and pathogenesis of Erythema Induratum (EI)?
EI is considered a hypersensitivity disorder mediated by immune complexes or cell-mediated hypersensitivity, evidenced by positive tuberculin skin tests and interferon-γ release assays to Mycobacterium tuberculosis.
What condition should be considered for a young woman in her 30s with erythematous nodules on her calves?
The condition to consider is erythema induratum (EI), which is most commonly associated with Mycobacterium tuberculosis (MTB).
What is the likely diagnosis for a patient with a history of Crohn’s disease who develops tender nodules on the lower legs?
The likely diagnosis is erythema nodosum (EN), which is more likely to occur in patients with Crohn’s disease and colonic involvement.
What treatments are effective for erythema nodosum, and what are the risks of corticosteroid use?
Effective treatments include etanercept and infliximab. Corticosteroids are not first-line therapy due to the risk of infectious etiology in idiopathic cases.
What factors increase the risk of recurrence for a patient with erythema nodosum who has a history of pregnancy?
Factors include sarcoidosis, hormonal therapy, and pregnancy. Management focuses on treating the underlying cause and supportive care.
What are the management strategies for Erythema Nodosum (EN)?
Management includes eliminating exposure to potential triggers, treating underlying diseases, seeking and treating potential infections, and providing supportive care.
How does Erythema Induratum (EI) differ from Nodular Vasculitis (NV)?
EI is strongly associated with Mycobacterium tuberculosis (MTB), while NV is not. EI presents with ulcerated nodules on the calves, whereas NV presents similar nodular lesions.
What are the clinical features and epidemiology of Erythema Induratum/Nodular Vasculitis (EI/NV)?
EI/NV is most common in young to middle-aged women, with a mean age of 36.6 years, and presents as recurrent, erythematous to violaceous nodules on the calves.
What infections and disorders are associated with Nodular Vasculitis (NV)?
Associated conditions include Hepatitis B and C, Ulcerative colitis, Leukemia, Rheumatoid arthritis, Hypothyroidism, and Crohn disease.
What are the histopathological features of early lesions in Erythema Induratum/Nodular Vasculitis (EI/NV)?
Early lesions show fat lobules containing discrete aggregates of inflammatory cells, predominantly neutrophils, and adipocyte necrosis.
What is the clinical course and prognosis of Erythema Induratum/Nodular Vasculitis (EI/NV)?
EI/NV has a protracted course with recurrent episodes over months to years, and the course of EI is often more chronic than EN.
What is Lipodermatosclerosis (LDS) and its epidemiology?
LDS is a form of sclerosing panniculitis involving the lower legs, most common in overweight women older than 40 years, with a female-to-male ratio of 4:1.
What is the pathogenesis of erythema induratum, and what are its histopathologic findings?
Erythema induratum is a hypersensitivity disorder mediated by immune complexes, with histopathologic findings including granulomatous appearance and caseous necrosis.
What factors might contribute to the chronicity of erythema induratum?
Factors include underlying Mycobacterium tuberculosis infection, venous insufficiency, and obesity.
What other conditions are associated with erythema induratum?
Associated conditions include hepatitis B, ulcerative colitis, leukemia, rheumatoid arthritis, and hypothyroidism.
What are the key histopathologic findings in erythema induratum?
Key findings include granulomatous appearance, caseous necrosis, and vascular damage.
What are the clinical features of erythema induratum?
Clinical features include ulcerated nodules on the calves and scarring, differing from erythema nodosum, which is associated with Mycobacterium tuberculosis.
What are the recommended treatments for erythema induratum?
Recommended treatments include anti-TB therapy with multiple agents, confirmed by a positive tuberculin skin test or interferon-γ release assay.
What is the significance of Langerhans cells in hypersensitivity reactions?
Langerhans cells may suggest a type IV hypersensitivity reaction, indicating an immune response leading to various dermatological conditions.
What is the significance of extrataneous Mycobacterium tuberculosis (MTB) in patients with EI/NV?
Extrataneous MTB can complicate the clinical presentation and management of EI patients.
How does the clinical course of EI/NV compare to erythema nodosum (EN)?
EI/NV often has a more chronic course with recurrent episodes, leading to worse cosmetic outcomes compared to EN.
What are the recommended management strategies for nodular vasculitis not associated with Mycobacterium tuberculosis?
Management includes the use of SSKI, nonsteroidal anti-inflammatory agents, corticosteroids, and treatment of venous insufficiency.
What are the epidemiological characteristics of lipodermatosclerosis (LDS)?
LDS primarily affects overweight women older than 40 years, with a female-to-male ratio of 4:1, often associated with venous insufficiency.
What are the clinical features of acute Lipodermatosclerosis (LDS)?
Acute LDS is characterized by painful, poorly demarcated erythematous plaques that evolve to violaceous or indurated plaques, commonly seen on the lower legs.
What are the key pathological features associated with Lipodermatosclerosis (LDS)?
Key features include elevated hydrostatic pressure-induced vascular permeability and the presence of microthrombi.
What is the rate of unilateral involvement in Lipodermatosclerosis (LDS)?
Unilateral involvement occurs in 55% of cases.
What are the common misdiagnoses for lesions in Lipodermatosclerosis (LDS)?
Lesions can lead to misdiagnoses of erythema nodosum (EN), cellulitis, or thrombophlebitis.
What do vascular studies typically show in Lipodermatosclerosis (LDS)?
Vascular studies typically show venous insufficiency.
What are the key pathological features associated with Lipodermatosclerosis (LDS)?
Key pathological features include elevated hydrostatic pressure-induced vascular permeability, microthrombi, hypoxia, upregulation of intercellular adhesion molecules, and inflammation leading to fibrosis.
How is Lipodermatosclerosis (LDS) diagnosed?
Diagnosis is primarily clinical, involving biopsy, MRI, and histopathologic findings.
What is the likely diagnosis for a patient with erythematous plaques on the lower legs and a history of venous insufficiency?
The likely diagnosis is lipodermatosclerosis (LDS). The stages include an acute inflammatory stage and a chronic fibrotic stage.
What is the pathogenesis of Lipodermatosclerosis (LDS) in a patient with obesity and deep vein thrombosis?
The pathogenesis involves elevated hydrostatic pressure, hypoxia, and chronic inflammation leading to fibrosis.
What diagnostic tests should be performed for a patient with lipodermatosclerosis?
Diagnostic tests include venous Doppler examinations and duplex sonography.
What are the clinical features of the chronic stage of Lipodermatosclerosis (LDS)?
The chronic stage presents as indurated, sclerotic, hyperpigmented skin, often in a stocking distribution.
What are the key pathogenic features of Lipodermatosclerosis (LDS)?
Key pathogenic features include elevated hydrostatic pressure, microthrombi, and hypoxia.
What are the histopathologic changes in advanced lesions of Lipodermatosclerosis (LDS)?
Advanced lesions show septal sclerosis, fat lobule atrophy, and lipomembranous changes.
What are the clinical features of the acute stage of Lipodermatosclerosis (LDS)?
The acute stage features painful, erythematous plaques that may ulcerate.
What are the key management strategies for Lipodermatosclerosis (LDS)?
Key management strategies include compression therapy, weight loss, and medications like pentoxifylline.
What are the clinical features of acute Lipodermatosclerosis (LDS) and how can they lead to misdiagnosis?
Acute LDS is characterized by painful, poorly demarcated, cellulitis-like erythematous plaques.
What are the key histopathologic findings in Lipodermatosclerosis (LDS)?
Key histopathologic findings include dermal stasis changes, capillary congestion, and lipomembranous changes.
How does hypoxia contribute to the pathogenesis of Lipodermatosclerosis (LDS)?
Hypoxia induces chronic inflammation, macrophage infiltration, and fibrosis.
What are the differential diagnoses for Lipodermatosclerosis (LDS)?
Differential diagnoses include erythema nodosum, cellulitis, thrombophlebitis, neoplasms, and other panniculitides.
What factors influence the clinical course and prognosis of Lymphedema Distichiasis Syndrome (LDS)?
Factors include patient’s comorbidities, compliance with treatment, and the form of the syndrome.
What are the management strategies for Lymphedema Distichiasis Syndrome (LDS)?
Management strategies include diagnostic tests, compression therapy, daily use of stockings, and medications.
What is infection-induced panniculitis (IIP) and what are its clinical features?
IIP is caused by an infectious agent, with clinical features including fluctuant lesions and erythematous plaques.
What are the epidemiological factors associated with infection-induced panniculitis (IIP)?
Epidemiological factors include host susceptibility, infectious etiology, and associations with autoimmune disorders.
What are the benefits and risks of compression therapy for lipodermatosclerosis?
Compression therapy reduces edema and inflammation but may be difficult to adhere to.
How does infection-induced panniculitis (IIP) differ in immunocompromised versus immunocompetent patients?
Immunocompromised patients may have widespread lesions, while immunocompetent patients may have localized lesions.
What are the common infectious agents that can cause idiopathic inflammatory panniculitis (IIP)?
Common infectious agents include Fusariosis, cutaneous aspergillosis, and Pseudomonas aeruginosa.
How does immunosuppression affect the diagnosis of infections in patients with idiopathic inflammatory panniculitis (IIP)?
Immunosuppression complicates diagnosis due to varied immune responses and atypical presentations.
What histopathologic features are associated with infections acquired by direct inoculation compared to those from hematogenous spread in panniculitis?
Direct inoculation shows more inflammation in the superficial dermis, while hematogenous spread affects deeper layers.
What role do adipocytes play in the immune response related to panniculitis?
Adipocytes act as innate immune cells, responding to infections and producing pro-inflammatory cytokines.
What are the additional features of idiopathic inflammatory panniculitis (IIP) observed in histopathologic evaluations?
Additional features include hemorrhage, vascular proliferation, and dermal inflammatory infiltrate.
What are the common infectious agents in a patient with infection-induced panniculitis presenting with abscess-like lesions?
Common infectious agents include bacteria, mycobacteria, fungi, protozoa, and viruses.
What are the histopathologic features of infection-induced panniculitis?
Histopathologic features include lobular panniculitis, hemorrhage, vascular proliferation, and granulomas.
What are common infectious agents in infection-induced panniculitis?
Common infectious agents include bacteria, mycobacteria, fungi, protozoa, and viruses.
How should the diagnosis of infection-induced panniculitis be confirmed?
Diagnosis should be confirmed with histopathologic studies, tissue culture, and molecular PCR techniques.
What clinical features are expected in immunocompromised patients with infection-induced panniculitis?
Clinical features include fluctuant or abscess-like lesions, erythematous plaques, and purpuric plaques.
How does immunosuppression affect the presentation of infection-induced panniculitis?
Immunosuppression is associated with more widespread and abscess-type lesions.
What are the common infectious agents in renal transplant patients with infection-induced panniculitis?
Common infectious agents include Cryptococcus, Pseudomonas aeruginosa, and Mycobacterium.
How should infection-induced panniculitis in renal transplant patients be managed?
Management includes appropriate antimicrobial therapy based on culture and sensitivity.
What are the common clinical features of infection-induced panniculitis in patients with HIV?
Common clinical features include abscess-like lesions and erythematous plaques.
How does infection-induced panniculitis differ in immunocompetent patients?
In immunocompetent patients, granulomas are more common, and lesions are less widespread.
What diagnostic tests are recommended for infection-induced panniculitis?
Recommended tests include histopathologic studies, tissue culture, and molecular PCR.
How do diagnostic tests vary with the patient’s immune status in infection-induced panniculitis?
In immunosuppressed patients, microorganisms may be numerous and easier to detect.
What are the common infectious agents in diabetic patients with infection-induced panniculitis?
Common infectious agents include Staphylococcus aureus, Mycobacterium, and fungi.
How should infection-induced panniculitis in diabetic patients be managed?
Management includes appropriate antimicrobial therapy and addressing underlying immunosuppression.
What are the histopathologic features of suppurative granulomas in infection-induced panniculitis?
Suppurative granulomas are formed by epithelioid histiocytes surrounding aggregated neutrophils.
What organisms are commonly associated with suppurative granulomas?
They are commonly associated with atypical mycobacteria.
What are the clinical implications of immunocompromised patients developing infectious panniculitis?
Immunocompromised patients are at increased risk for disseminated infections leading to infectious panniculitis.
How does the presence of urate crystals in gouty panniculitis inform treatment?
It suggests that the condition may be secondary to metabolic disturbances, necessitating a dual approach.
What role do adipocytes play in the immune response to infections causing panniculitis?
Adipocytes are innate immune cells that produce proinflammatory cytokines in response to infections.
How can histopathologic features of infectious panniculitis guide clinical decision-making?
They can help differentiate infectious panniculitis from other types, guiding appropriate treatment.
What factors influence the prognosis of organisms treated with antibiotics?
A prompt diagnosis and appropriate antibiotic treatment portend a good prognosis.
How does treatment vary for infections involving bacteria and parasites?
Treatment varies depending on the suspected or known organisms and their cultures and sensitivities.
What is Alpha1-Antitrypsin (A1AT) and its primary function?
Alpha1-Antitrypsin (A1AT) is a serine protease inhibitor produced mainly in the liver. It functions as an acute phase reactant during times of stress, helping to regulate protease activity and complement activation.
What are the common clinical features of A1AT panniculitis?
Common clinical features of A1AT panniculitis include:
- Painful erythematous nodules and plaques
- Early lesions may appear as cellulitic or abscess-type
- Lesions can ulcerate with oily or serosanguinous discharge
- Most commonly located on the lower trunk (buttocks) and proximal extremities
- May coexist with autoimmune disease, cancer, or infection
- Associated with pulmonary and hepatic diseases.
What is the epidemiology of A1AT panniculitis?
The epidemiology of A1AT panniculitis includes:
- Most commonly presents in individuals aged 30 to 60 years
- More than 60% involve ZZ phenotypes and 65% affect women
- Homozygous MM represents 90% to 97% of the population
- 35% of cases have a history of preceding trauma.
What are the possible mechanisms leading to the development of panniculitis in A1AT deficiency?
Possible mechanisms leading to the development of panniculitis include:
- Lack of interference with various proteases, leading to activation of lymphocytes and macrophages.
- Activation of the autoinflammatory cascade mediated by IL-1 and IL-1b.
- Lysis and destruction of connective tissue at sites of inflammation.
- Trauma to adipocytes causing release of adipokines and cytokines that attract inflammatory cells, leading to severe tissue damage due to the absence of A1AT.
How are A1AT phenotypes classified according to gel electrophoresis migration?
A1AT phenotypes are classified according to gel electrophoresis migration as follows:
Phenotype | Migration Speed |
|———–|—————-|
| F | Fast |
| M | Medium |
| S | Slow |
| Z | Very Slow |
| Null | No production of A1AT |
What is the serum level of A1AT in the ZZ phenotype?
The serum level of A1AT in the ZZ phenotype is 10% to 15% of normal.
What percentage of A1AT-deficiency panniculitis cases have preceding trauma?
Approximately 35% of cases have preceding trauma.
What is the characteristic discharge from ulcerated lesions in A1AT-deficiency panniculitis?
The characteristic discharge is oily or serosanguinous.
What is the likely diagnosis for a patient with painful erythematous nodules and a ZZ phenotype?
The likely diagnosis is Alpha1-Antitrypsin (A1AT) panniculitis, and the associated genetic phenotype is most commonly homozygous ZZ.
What are the clinical features associated with Alpha1-Antitrypsin (A1AT) deficiency?
- Painful erythematous nodules and plaques: Early lesions may appear as cellulitic or abscess-like.
- Ulceration: Lesions can ulcerate with oily or serosanguinous discharge, leading to atrophic scars.
- Common locations: Primarily on the lower trunk (buttocks) and proximal extremities, but may also affect lower legs and other sites.
- Coexisting conditions: May coexist with autoimmune diseases, cancer, or infections.
- Associated diseases: A1AT deficiency is linked to pulmonary and hepatic diseases, particularly in ZZ phenotype individuals.
What is the age of presentation for A1AT deficiency?
Most commonly seen between ages 30 and 60 years.
What is the prevalence of ZZ phenotypes in A1AT deficiency cases?
Over 60% of cases involve ZZ phenotypes, affecting 65% of women.
What does the MM phenotype represent in the population?
Homozygous MM represents 90% to 97% of the population.
What is the significance of trauma in A1AT deficiency cases?
35% of cases have a history of preceding trauma.
What are the histopathologic findings associated with early nodular lesions in A1AT deficiency panniculitis?
The histopathologic findings for early nodular lesions include:
- Splaying of neutrophils between collagen bundles in the overlying reticular dermis.
- Edema and degeneration of adipocytes, with ruptured and collapsed cell membranes and a perivascular mononuclear infiltrate.
- A mild infiltrate of neutrophils and macrophages in septa and lobules, with foci of early necrosis of subcutaneous fat.
What is the clinical significance of prompt diagnosis and treatment in A1AT deficiency panniculitis?
Prompt diagnosis and treatment of severe disease with A1AT augmentation may significantly improve the panniculitis and induce clinical remission. This requires augmentation therapy infusions only with recurrence, which is crucial as cutaneous and subcutaneous necrosis can develop rapidly and fatal cases have been reported, particularly in the ZZ variant.
What management strategies are recommended for A1AT deficiency panniculitis?
Management strategies for A1AT deficiency panniculitis include:
- Colchicine, antimalarials, dapsone, doxycycline
- Plasma infusion and plasma exchange
- Intravenous augmentation therapy of A1AT protein
- Liver transplantation in severe cases
- Steroids, immunosuppressives, and cytotoxic agents are less effective.
- For severe panniculitis unresponsive to standard treatment, protein-replacement therapy (augmentation therapy) is required, typically at 60 mg/kg with repeat infusions for recurrence.
What are the differential diagnoses for A1AT deficiency panniculitis?
The differential diagnoses for A1AT deficiency panniculitis include:
Diagnosis | Characteristics |
|———–|—————–|
| Infection-induced panniculitis | Erythema nodosum-like nodules, deep inflamed nodules or abscesses; may show fibrosis. |
| Factitial panniculitis | Geometric morphology; may have an underlying psychiatric disturbance. |
| Erythema induratum/nodular vasculitis | Tender nodules and plaques on call areas; vasculitis on histopathology. |
| Pancreatic panniculitis | Occurs anywhere on the face, neck, or trunk; ulceration and drainage may be present. |
| Subcutaneous Sweet syndrome | Erythematous nodules on the face, neck, upper trunk, and extremities. |
| Pyoderma gangrenosum | Volaceous, undermined borders with isomorphic response.
What management strategy should be avoided in A1AT-deficiency panniculitis with a history of trauma?
Debridement should be avoided because trauma may induce lesions in one-third of patients with A1AT-deficiency panniculitis.
What advanced therapy should be considered for severe A1AT-deficiency panniculitis unresponsive to standard treatment?
Protein-replacement therapy (augmentation therapy) with 60 mg/kg infusions should be considered for severe panniculitis unresponsive to standard treatment.
What is the critical factor contributing to poor wound healing in A1AT-deficiency panniculitis?
The critical factor is the tendency of A1AT-deficiency panniculitis for poor wound healing, often exacerbated by trauma.
What are the common clinical features of pancreatic panniculitis?
Pancreatic panniculitis typically presents as:
- Erythematous subcutaneous nodules that ulcerate spontaneously.
- Lesions primarily appear on lower extremities (around ankles and knees) but can also be found on arms, wrists, thighs, and trunk.
- Lesions may present as ill-defined erythematous to red-brown nodules, which can resolve with atrophic hyperpigmented scars.
What is the etiology of pancreatic panniculitis?
Pancreatic panniculitis results from the release of pancreatic enzymes (lipase, amylase, trypsin) into circulation, promoting vascular permeability and fat necrosis. It can occur even with normal serum levels of pancreatic enzymes.
What are the extrcutaneous manifestations associated with pancreatic panniculitis?
Extracutaneous manifestations include:
- Secondary infections as a significant complication.
- Periarticular fat necrosis with arthritis, and painful medullary fat necrosis in bone.
- PPP syndrome: a triad of pancreatic disease, panniculitis, and polyarthritis, which is rare and associated with both pancreatitis and pancreatic carcinoma.
What is the epidemiology of pancreatic panniculitis?
Pancreatic panniculitis is uncommon, developing in 0.3% to 3% of patients with pancreatic disorders such as acute or chronic pancreatitis, pancreatic carcinoma, or pancreatic pseudocysts.
What is the underlying pathogenesis for lesions that drain oily material in pancreatic panniculitis?
The pathogenesis involves the release of pancreatic enzymes (lipase, amylase, trypsin) into circulation, causing fat necrosis.
What is the associated syndrome for eosinophilia in pancreatic panniculitis?
The associated syndrome is Schmid triad (pancreatic tumor/pancreatitis, panniculitis, and eosinophilia), indicating a poor prognosis.
What is the prognosis for a patient with pancreatic carcinoma and erythematous nodules?
The triad is PPP syndrome (pancreatic disease, panniculitis, and polyarthritis). The prognosis is poor, especially if associated with pancreatic carcinoma.
What condition might develop in a patient with pancreatic panniculitis and periarticular fat necrosis?
The patient might develop monoarticular or oligoarticular arthritis secondary to periarticular fat necrosis.
What are the characteristics of cutaneous nodules in pancreatic panniculitis?
Cutaneous nodules often ulcerate spontaneously and appear as crops on the lower legs, especially in periarticular areas, but can also be found on the arms, wrists, thighs, and trunk.
How do the lesions in pancreatic panniculitis present?
They present as ill-defined erythematous to red-brown edematous and tender nodules, which may involute and resolve with atrophic hyperpigmented scars.
What are some features of lesions in pancreatic panniculitis?
Some lesions may have central softer areas or become fluctuant, abscess-like, and drain an oily material.
How may cutaneous manifestations relate to pancreatic disease?
The cutaneous manifestations may precede the diagnosis of the associated pancreatic disease by weeks to months in up to 45% of patients.
What are the potential extraneous complications associated with pancreatic panniculitis?
Potential complications include secondary infections, periarticular fat necrosis with concomitant arthritis, PPP syndrome, osteonecrosis, pleural effusions and serositis, and eosinophilia.
What is PPP syndrome?
PPP syndrome is a triad of pancreatic disease, panniculitis, and polyarthritis, which is very rare and associated with both pancreatitis and pancreatic carcinoma.
What is the etiology of pancreatic panniculitis?
The etiology is primarily due to the release of pancreatic enzymes such as lipase, amylase, and trypsin into the circulation, leading to increased vascular permeability and fat necrosis.
What are the characteristic histological findings in pancreatic panniculitis?
Characteristic findings include lobular fat necrosis, ghost cells, saponification, and older lesions showing necrosis replaced by foamy histiocytes, multinucleated giant cells, lymphocytes, and fibrosis.
What is the clinical prognosis for patients with pancreatic panniculitis?
When pancreatic pathology resolves, cutaneous symptoms improve. However, the mortality rate associated with pancreatic disease may be as high as 24% to 42%, and near 100% in those with pancreatic carcinoma.
What management strategies are recommended for pancreatic panniculitis?
Management strategies include supportive care, octreotide, plasmapheresis, and surgical resection with chemotherapy for pancreatic cancer.
What are the epidemiological characteristics of lupus panniculitis?
Lupus panniculitis predominantly affects women (4:1 ratio) and is most commonly seen in individuals aged 30-60 years, with rare occurrences in childhood.
What does saponification on histopathology indicate in pancreatic panniculitis?
Saponification indicates calcification, producing fine, granular basophilic deposits within and around necrotic adipocytes.
What are ghost cells in histopathology?
Ghost cells are adipocytes that have lost their nuclei but maintain peripheral outlines, indicating lobular fat necrosis.
What are the key histopathological features of pancreatic panniculitis?
Key features include lobular fat necrosis, saponification, and inflammatory infiltrate with neutrophils and lymphocytes.
What is the significance of mortality rates in pancreatic panniculitis?
Mortality rates linked to pancreatic disease range from 24% to 42%, with nearly 100% in pancreatic carcinoma, indicating a dire prognosis.
What is the incidence of systemic lupus erythematosus (SLE) in patients with lupus erythematosus panniculitis (LEP)?
The incidence of SLE in patients with LEP ranges from 10% to 41%, with only 2% to 5% of SLE patients having LEP.
What are the common clinical features of lupus erythematosus panniculitis (LEP)?
Common features include tender and painful lesions, deep subcutaneous nodules, and ulceration in up to 28% of cases.
What are the histopathologic features of discoid lupus erythematosus (DLE) in LEP?
Histopathologic features include lobular or mixed lobular and septal panniculitis, lymphoid follicles, and variable hyaline fat necrosis.
What role does the innate immune system play in LEP?
The innate immune system, particularly adipocytes, plays a significant role in the development of LEP by expressing various TLRs.
What are some differential diagnoses for lupus erythematosus panniculitis (LEP)?
Differential diagnoses include tumid lupus, cutaneous lymphoma, erythema induratum, morphea/systemic sclerosis, and pancreatic panniculitis.
What is the average disease duration for Cytophagic Histiocytic Panniculitis (CHP)?
The average disease duration for CHP is 6 years, with a range of less than 1 year to 38 years.
What are the first-line treatments recommended for Lymphocutaneous Erythema Panniculitis (LEP)?
First-line treatments for LEP are antimalarials, which interfere with inflammatory cytokines and TLRs.
What is the significance of CD123 immunohistochemical staining in CHP prognosis?
CD123 staining is a prognostic indicator; a higher percentage of CD123 positive cells correlates with a better response to steroid therapy.
What are some treatment options for severe cases of CHP?
Severe cases may require treatments like rituximab or infliximab, with caution due to potential activation of Lymphocutaneous Erythema.
What are the clinical features associated with Cytophagic Histiocytic Panniculitis (CHP)?
Clinical features include subcutaneous erythematous to violaceous plaques and nodules, fever, hepatosplenomegaly, and hypertriglyceridemia.
What is the epidemiological significance of Cytophagic Histiocytic Panniculitis (CHP)?
The epidemiology of CHP is based on its causes, which are seen in adults, adolescents, and children.
What are the clinical features of Cytophagic Histiocytic Panniculitis (CHP)?
- Subcutaneous erythematous to violaceous plaques and nodules on extremities and trunk.
- Fever, hepatosplenomegaly, and hypertriglyceridemia.
What is the epidemiological significance of Cytophagic Histiocytic Panniculitis (CHP)?
The epidemiology of Cytophagic Histiocytic Panniculitis (CHP) is largely based on its causes, which are seen in adults, adolescents, and children.
What immunohistochemical marker can predict response to steroid therapy in lupus panniculitis?
CD123 immunohistochemical staining can predict response to steroid therapy.
What first-line treatment is recommended for lupus panniculitis and what is its mechanism of action?
First-line treatment is antimalarials, which interfere with inflammatory cytokines and TLRs.
What is the average disease duration for Cytophagic Histiocytic Panniculitis (CHP) and what does it indicate about the condition’s nature?
The average disease duration for Cytophagic Histiocytic Panniculitis (CHP) is 6 years, indicating that CHP is a chronic inflammatory disorder characterized by periodic flares or long remissions.
What are the first-line treatments for Lymphocutaneous Erythema Panniculitis (LEP) and how do they function?
The first-line treatments for Lymphocutaneous Erythema Panniculitis (LEP) are antimalarials, which interfere with inflammatory cytokines and Toll-like receptors (TLRs).
What are the potential risks associated with using rituximab or infliximab in severe cases of CHP?
The potential risks include the possibility of activating Lymphocutaneous Erythema Panniculitis (LE), which can exacerbate the condition.
How does the presence of CD123 positive cells serve as a prognostic indicator in CHP?
A higher percentage of CD123 positive cells is associated with a greater response to steroid therapy, indicating better treatment outcomes.
What is the significance of the duration of Cytophagic Histiocytic Panniculitis (CHP) in terms of treatment approach?
The duration varies from 3 months to 27 years, influencing treatment approaches such as chemotherapy for aggressive cases related to hemophagocytic lymphohistiocytosis (HLH).
What laboratory findings are associated with Cytophagic Histiocytic Panniculitis (CHP)?
- Erythrocyte sedimentation rate is often normal or low.
- Positive ANA.
- Hypertriglyceridemia due to inhibition of Lipoprotein lipase.
What is the etiology and pathogenesis of Cytophagic Histiocytic Panniculitis (CHP)?
- HLH is considered an autoinflammatory disease with impaired cytolytic T-cell and natural killer cell functions.
What are the diagnostic criteria for Cytophagic Histiocytic Panniculitis (CHP)?
- Multiple biopsies may be needed as cytophagocytosis on histopathology may be focal.
- Histopathology shows lobular panniculitis.
What is the clinical course and prognosis of Cytophagic Histiocytic Panniculitis (CHP)?
- CHP may have a rapidly fatal disease course or a longer disease course with intermittent remissions.
What management strategies are recommended for Cytophagic Histiocytic Panniculitis (CHP)?
- Treatment includes immunosuppressive, immunomodulating, or cytotoxic agents targeting activated macrophages.
What are beanbag cells in the context of cytophagic histiocytic panniculitis (CHP)?
Beanbag cells are phagocytic histiocytes containing intact or fragmented cells and nuclear debris.
What is the underlying mechanism of hypertriglyceridemia in cytophagic histiocytic panniculitis (CHP)?
The mechanism is inhibition of lipoprotein lipase, which can also cause pancreatitis.
What laboratory findings would support the diagnosis of cytophagic histiocytic panniculitis (CHP) in a patient with hemophagocytic lymphohistiocytosis (HLH)?
Laboratory findings include elevated serum ferritin levels, hypertriglyceridemia, and soluble hemoglobin-haptoglobin scavenger receptor CD163 levels.
What treatment combination can reduce mortality in a patient with cytophagic histiocytic panniculitis (CHP) with a rapidly fatal disease course?
The combination of corticosteroids and cyclosporine can reduce the mortality rate from 70% to near 0%.
What are the clinical features associated with fulminant forms of cytophagic histiocytic panniculitis (CHP)?
- Fever
- Hepatosplenomegaly
- Lymphadenopathy
- Serosal effusions
- Pancytopenia
- Intravascular coagulation
- Liver failure
- Hemorrhagic diatheses
- Death
How does the pathogenesis of hemophagocytic lymphohistiocytosis (HLH) contribute to the clinical manifestations of cytophagic histiocytic panniculitis?
HLH is characterized by impairment of cytolytic T-cell and natural killer cell functions, leading to severe systemic symptoms.
What diagnostic procedures are necessary to confirm cytophagic histiocytic panniculitis (CHP)?
To confirm CHP, multiple biopsies may be necessary, along with histopathological examination.
What is the clinical significance of corticosteroids and cyclosporine in the management of cytophagic histiocytic panniculitis?
Corticosteroids and cyclosporine are significant because they reduce the mortality rate to near 0%.
What are the common clinical features of subcutaneous fat necrosis of the newborn?
- Sharply demarcated, erythematous to violaceous, firm, indurated nodules or plaques.
What is the etiology and pathogenesis of subcutaneous fat necrosis of the newborn?
- The etiology is unknown, but hypothermia and hypoxia are presumed to be involved.
What are the diagnostic methods for subcutaneous fat necrosis of the newborn?
- FNAB (Fine Needle Aspiration Biopsy) is more cost-effective and rapid for diagnosis.
What is the epidemiology of subcutaneous fat necrosis of the newborn?
- It presents in full-term or post-term newborns, with preterm cases also noted.
What is the most common location for lesions in subcutaneous fat necrosis of the newborn?
The most common locations are the back, shoulders, arms, buttocks, and thighs.
What treatment is effective for cyclosporine-resistant cases of cytophagic histiocytic panniculitis (CHP)?
Tacrolimus is effective for cyclosporine-resistant cases.
What is the presumed pathogenesis of hypercalcemia in subcutaneous fat necrosis of the newborn?
The presumed pathogenesis involves increased 25-hydroxyvitamin D3–1 hydroxylase within granulomatous infiltrates.
What is the most common metabolic complication in newborns with subcutaneous fat necrosis?
The most common metabolic complication is hypercalcemia, which should be monitored serially.
What alternative treatment options are available for a patient with cytophagic histiocytic panniculitis (CHP) resistant to cyclosporine?
Alternative treatments include tacrolimus, cyclophosphamide, and IVIG.
What are the common clinical features of cold panniculitis?
Cold panniculitis presents as indurated, erythematous plaques or nodules at sites of cold exposure.
What are the risk factors associated with cold panniculitis in equestrians?
Risk factors include moderate smoking, tight riding clothes, and riding for long periods.
What management strategies are recommended for hypercalcemia in cases of cold panniculitis?
Management strategies include hydration, use of furosemide, and low calcium formula.
What management strategies are recommended for hypercalcemia in cases of cold panniculitis?
Management strategies for hypercalcemia in cold panniculitis include:
1. Hydration
2. Use of furosemide
3. Low calcium formula
4. Bisphosphonates (alendronate, etidronate, pamidronate) to treat hypercalcemia
5. Systemic glucocorticoids to inhibit vitamin D production
6. Early monitoring and recognition of hypercalcemia to prevent dystrophic calcification.
What is the histopathological finding in cold panniculitis?
Histopathological findings in cold panniculitis typically show mostly lobular panniculitis with a lymphohistiocytic or mixed infiltrate, and perivascular lymphohistiocytic infiltrate involving blood vessels at the dermal-subcutaneous junction and within overlying dermis.
What pharmacologic treatments can be used to manage hypercalcemia in a newborn with subcutaneous fat necrosis?
Pharmacologic treatments include bisphosphonates (e.g., alendronate, etidronate, pamidronate) and systemic glucocorticoids.
What is the prognosis for cold panniculitis and how does it typically resolve?
Cold panniculitis is a benign process with an excellent prognosis, typically regressing within a few weeks to 6 months without the need for aggressive treatment.
What are the clinical features of cold panniculitis and how do they manifest after cold exposure?
Clinical features of cold panniculitis include:
- Indurated, erythematous plaques or nodules at sites of cold exposure.
- Onset ranges from 6 to 72 hours after exposure.
- Lesions appear at sites that have direct contact with cold, such as the lateral thighs in equestrians.
What are the risk factors associated with cold panniculitis in equestrians?
Risk factors for cold panniculitis in equestrians include:
- Moderate smoking
- Tight riding clothes
- Riding for long periods
- Age less than 35 years old.
What are the characteristics of scrotal cold panniculitis?
Scrotal cold panniculitis, also known as scrotal fat necrosis, presents as unilateral or bilateral tender masses located below the testes.
What is the etiology of equestrian panniculitis?
Equestrian panniculitis occurs hours after outdoor cold exposure while wearing tight-fitting, noninsulated pants, leading to pruritic, erythematous nodules and plaques that may ulcerate, crust, and drain.
What is the clinical diagnosis for scrotal cold panniculitis?
The clinical diagnosis involves identifying lobular panniculitis with a lymphohistiocytic or mixed inflammatory infiltrate within fat lobules, along with prominent inflammation of veins at the dermal-subcutaneous fat junction.
What is the management strategy for equestrian panniculitis?
Management includes avoidance of further cold exposure, wearing warm loose-fitting clothing, local topical corticosteroid application, and smoking cessation. Nifedipine is not effective.
What is the prognosis for patients with cold panniculitis?
The condition is self-limiting and resolves upon rewarming without continued exposure to cold, with full improvement typically seen over days to 3 months.
What histopathologic findings are expected in a patient with cold panniculitis and indurated plaques on the lateral thighs?
Histopathologic findings include lobular panniculitis with a lymphohistiocytic or mixed inflammatory infiltrate.
What diagnostic imaging should be performed for scrotal involvement in cold panniculitis?
Ultrasound should be performed for scrotal involvement.
What preventive measures should be taken for equestrians with cold panniculitis?
Preventive measures include wearing warm, loose-fitting clothing and avoiding prolonged cold exposure.
What is the likely diagnosis for a 35-year-old equestrian with erythematous nodules on the lateral thighs after prolonged cold exposure?
The likely diagnosis is equestrian panniculitis. Preventive measures include wearing warm, loose-fitting clothing and avoiding prolonged cold exposure.
What is the diagnosis for a child with erythematous plaques on the cheeks after eating a popsicle, and what is the expected clinical course?
The diagnosis is cold panniculitis (popsicle panniculitis). The clinical course is self-limiting and resolves upon rewarming without further cold exposure.
What are the clinical features of scrotal cold panniculitis and how does it differ from equestrian panniculitis?
Scrotal cold panniculitis presents as unilateral or bilateral tender masses below the testes, while equestrian panniculitis occurs hours after outdoor cold exposure in tight-fitting pants, leading to pruritic, erythematous nodules and plaques that may ulcerate, crust, and drain.
What is the etiology behind scrotal cold panniculitis in younger males?
Scrotal cold panniculitis may be related to the greater concentration of scrotal fat and the increased cold sensitivity of the scrotal adipose tissue (AT) in younger males, along with a higher proportion of saturated fat to unsaturated fat in infants.
What are the recommended management strategies for equestrian panniculitis?
Management strategies for equestrian panniculitis include:
1. Avoidance of further cold exposure.
2. Wearing warm, loose-fitting clothing.
3. Application of local topical corticosteroids.
4. Smoking cessation.
Nifedipine is not effective for this condition.
How does the clinical course of cold panniculitis typically progress?
Cold panniculitis is generally self-limiting and resolves upon rewarming without continued exposure to cold. Full improvement may be observed over days to 3 months, with a positive history of prior hypothermia (PIH) noted in some cases.
What are the clinical features of factitial panniculitis?
Factitial panniculitis is characterized by:
- Self-induced trauma causing subcutaneous inflammation.
- Erythematous geometric papules and nodules with erosions or ulcerations.
- Disparate location or appearance of lesions.
- It can occur in patients with personality disorders and may involve cosmetic fillers, oils, and food.
What is the histopathological finding in factitial panniculitis?
Histopathology of factitial panniculitis typically shows:
- Nonspecific lobular panniculitis without vasculitis.
- Suppurative granuloma involving fat lobules, requiring cultures for organisms.
- Features like foreign body granuloma and necrosis may also be present.
What are the treatment options for traumatic panniculitis?
Treatment for traumatic panniculitis includes:
1. Psychiatric treatment for self-inflicted injuries.
2. Supportive care and interference with injection of responsible agents.
3. Surgical excision of implanted materials if necessary.
4. Prevention of ongoing trauma and supportive care as it is self-limiting without further trauma.
What are the clinical features associated with connective tissue disease-associated panniculitis?
Clinical features include:
- Association with dermatomyositis, often preceding or occurring with disease flares.
- Erythematous, tender, painful nodules and plaques on the arms, buttocks, thighs, and abdomen.
- Complications may include ulceration and lipodystrophy.
What histopathological changes are observed in connective tissue disease-associated panniculitis?
Histopathology typically shows:
- Lobular panniculitis with lymphocytic and plasma cell infiltrate.
- Dermal mucin and vacuolar interface change, similar to lupus panniculitis.
- Possible calcification and membranocytic changes in late-stage lesions, indicating worse prognosis.
What are the treatment strategies for connective tissue disease-associated panniculitis?
Treatment strategies include:
- Corticosteroids and immunosuppressants (e.g., azathioprine, mycophenolate mofetil, methotrexate).
- IVIG may benefit patients with dermatomyositis-associated panniculitis.
- Antimalarials are not effective in this condition.
What are the clinical features of factitial panniculitis and how do they differ from traumatic panniculitis?
Factitial panniculitis is characterized by self-induced inflammation due to mechanical, physical, or chemical trauma, presenting as erythematous geometric papules and nodules with possible erosions or ulcerations. It often occurs in patients with personality disorders and may involve cosmetic fillers or food waste. In contrast, traumatic panniculitis results from external injuries, such as cupping or acupuncture, and is not self-inflicted.
What histopathological features are associated with connective tissue disease-associated panniculitis?
Histopathological examination of connective tissue disease-associated panniculitis typically reveals:
- Lobular panniculitis with lymphocytic and plasma cell infiltration.
- Dermal mucin and vascular interface changes, which are similar to those seen in lupus panniculitis.
- Possible calcification in the tissue, indicating a worse prognosis in late-stage lesions.
What treatment strategies are recommended for patients with factitial panniculitis?
The management of factitial panniculitis includes:
1. Psychiatric treatment for underlying psychological issues related to self-inflicted injuries.
2. Supportive care to manage symptoms and prevent further trauma.
3. Surgical excision of any implanted materials that may be causing the condition.
4. Prevention of ongoing trauma is crucial, as the condition is self-limiting without further injury.
How does the clinical presentation of connective tissue disease-associated panniculitis differ from that of factitial panniculitis?
Connective tissue disease-associated panniculitis often presents with:
- Erythematous, tender, and painful nodules and plaques on the arms, buttocks, thighs, and abdomen, which may correlate with disease flares.
- Complications such as ulceration and lipodystrophy are more common.
- It may also be the primary presentation of conditions like dermatomyositis, whereas factitial panniculitis is primarily self-induced and linked to psychological factors.
What are the clinical features of gouty panniculitis?
- Subcutaneous urate crystals are a rare manifestation of gout.
- Commonly found as indurated nodules or plaques on lower extremities.
- Possible ulceration and massive AT hypertrophy may occur.
- Associated with hyperuricemia and arthralgias.
- Lesions may be diagnosed as chronic gout but are often a consequence of it.
What is the histopathological finding in gouty panniculitis?
- Fine, radially oriented, needle-shaped urate crystals surrounded by granulomatous inflammation.
- Crystals are refractive and negatively birefringent.
- Alcohol fixation enhances crystal visualization.
- No evidence of vasculitis is present.
What are the treatment options for gouty panniculitis?
- Allopurinol (high doses) and probenecid are standard treatments for gout.
- Anti-inflammatory medications, including colchicine, can reduce uric acid levels.
- Corticosteroids may benefit acute panniculitis.
- Surgery may be necessary for ulcerated lesions that do not heal.
What are the clinical features of post-steroid panniculitis?
- Post-steroid panniculitis is often seen in children after high doses of systemic corticosteroid treatment.
- Erythematous, tender nodules and plaques appear on cheeks, trunk, arms, and legs.
- Lesions resolve within weeks or months without scarring, but may leave residual hyperpigmentation.
What is the histopathological finding in post-steroid panniculitis?
- Lobular panniculitis with a lymphocytic and granulomatous infiltrate.
- Lipophages and crystals may be present.
- Multinucleated giant cells may contain needle-shaped clefts similar to those seen in sclerema.
What is the treatment approach for post-steroid panniculitis?
- Lesions typically resolve within weeks or months without treatment, leaving residual hyperpigmentation.
- Gradual tapering of steroids may eliminate the risk of poststeroid panniculitis.
What are the clinical features of gouty panniculitis and how do they differ from post-steroid panniculitis?
Gouty Panniculitis:
- Subcutaneous urate crystals are rare.
- Commonly presents as indurated nodules or plaques, especially on lower extremities.
- May involve massive AT hypertrophy requiring surgical resection.
- Associated with hyperuricemia and arthralgias.
Post-Steroid Panniculitis:
- Often seen in children after high doses of corticosteroids.
- Erythematous, tender nodules on cheeks, trunk, arms, and legs.
- Resolves without scarring, but may leave residual hyperpigmentation.
What is the histopathological difference between gouty panniculitis and post-steroid panniculitis?
Gouty Panniculitis:
- Characterized by fine, needle-shaped urate crystals surrounded by granulomatous inflammation.
- Crystals are refractile and negatively birefringent.
Post-Steroid Panniculitis:
- Features lobular panniculitis with a lymphocytic and granulomatous infiltrate.
- May contain lipophages and crystals, but lacks vasculitis.
What treatment options are available for gouty panniculitis and how do they compare to those for post-steroid panniculitis?
Gouty Panniculitis Treatment:
- Allopurinol (high doses), probenecid, and anti-inflammatory medications.
- Acute cases may benefit from corticosteroids.
- Surgery for non-healing ulcers.
Post-Steroid Panniculitis Treatment:
- Lesions typically resolve within weeks to months without treatment.
- Gradual tapering of steroids may reduce the risk of recurrence.
