12 - 73 - PANNICULITIS Flashcards
Erythema nodosum
Erythema induratum
Lipodermatosclerosis
(sclerosing panniculitis, hypodermitis, sclerodermiformis, chronic panniculitis with lipomembranous changes, sclerotic atrophic cellulitis, venous stasis panniculitis)
Infectious panniculitis
α1 -Antitrypsin panniculitis
Pancreatic panniculitis
Lupus panniculitis
Panniculitis with dermatomyositis
Cytophagic histiocytic panniculitis
Subcutaneous fat necrosis of the newborn
Cold panniculitis (Haxthausen disease)
Factitial panniculitis
differentiate septal and lobular panniculitis
the prototypic septal panniculitis
Erythema nodosum (EN)
most commonly involved area in erythema nodosum
anterior lower legs and ankles
most common etiology in pediatric cases of EN
streptococcal respiratory tract infections
EN has characteristic, but not sensitive or specific, groupings of histiocytes surrounding a central stellate cleft called
Miescher granuloma
mainstay of treatment of EN after removing or treating the provoking factors
Supportive care
- bed rest if severe, with lower-extremity elevation, and nonsteroidal antiinflammatory agents are recommended.
effective in Behçet-related EN
Colchicine
options for inflammatory bowel disease–associated EN
Etanercept and infliximab
Erythematous subcutaneous nodules and plaques of lower legs; common on calves, but also on anterolateral legs, feet, and thighs; rarely also on arms, forearms, and face
ERYTHEMA INDURATUM
Associated with venous insufficiency
ERYTHEMA INDURATUM
erythema induratum is most commonly associated with what infection?
MTB
most commonly presents with ulcerated nodules on the calves, and is associated with MTB infection
Erythema induratum (EI)
ulcerated nodules on the calves without MTB association
nodular vasculitis (NV)
most common diagnosis of a panniculitis with vasculitis
EI/NV grouping
lesions develop more frequently during winter, and are associated with obesity and venous insufficiency
erythema induratum
common finding in erythema induratum histopath
mostly lobular or mixed septal and lobular panniculitis
Indurated, firm plaques of wood-like consistency on the lower legs.
LIPODERMATOSCLEROSIS
the most common form of panniculitis
Lipodermatosclerosis (LDS)
best site for biopsy of lipodermatosclerosis
proximal edge of involvement for highest rates of healing
first line treatment for LDS
compression therapy
- contraindicated in patients with arterial compromise
- Higher compression gradient (30 to 40 mm Hg) may be more effective, but lower pressure (15 to 20 mm Hg or 20 to 30 mm Hg) encourages compliance
- Compression tightens vascular tight junctions, inhibiting permeability of fluid into the perivascular tissue
- must be worn all day; a few days without compression may lead to recurrence of edema and inflammation
most common sites of infection of infection-induced panniculitis
legs and feet
but upper extremities, trunk, and face also may be involved
most frequent association with infection-induced panniculitis
immunosuppression of varying etiology
histopath findings of infection-induced panniculitis
A mostly lobular panniculitis, IIP often reveals a **mixed septal and lobular pattern. **
The superficial dermis has more inflammation in infections acquired by direct inoculation, while infections secondary to hematogenous spread involve the deep reticular dermis and subcutaneous fat
most common phenotype to induce α1-Antitrypsin (α1AT) panniculitis
ZZ-phenotype
Homozygous MM represents 90% to 97% of the population
- Patients present with painful erythematous nodules and plaques, but early lesions may have a cellulitic or fluctuant abscess-type appearance
- Lesions may ulcerate with oily or serosanguinous discharge and resolve with atrophic scars
α1-Antitrypsin panniculitis
area of predilection of α1-Antitrypsin panniculitis
lower trunk (buttocks) and proximal extremities
but the lower legs and other sites may be affected
phenotype that is at highest risk for associated with **pulmonary and hepatic disease, **leading to chronic obstructive pulmonary disease, hepatic cirrhosis, or hepatocellular carcinoma
ZZ phenotype
early and distinctive diagnostic clue for α1-Antitrypsin panniculitis
splaying of neutrophils between collagen bundles throughout the overlying reticular dermis
**“floating” necrotic fat lobules ** from liquefactive necrosis and dissolution of dermal collagen may be accompanied by ulceration, and degeneration of elastic tissue may lead to septal destruction may be seen in what type of panniculitis
area of predilection of α1-Antitrypsin panniculitis
areas of predilection of pancreative panniculitis
lower legs, especially the periarticular areas
also on the arms, wrists, thighs, and trunk
potential markers of extrapancreatic fat necrosis
Resistin and leptin
ghost cells are seen in what panniculitis?
Pancreatic panniculitis
Adipocytes lose their nuclei but maintain peripheral outlines, forming characteristic “ghost cells”
important prognostic indicator in lupus panniculitis
CD123-positive staining
CD123 immunohistochemical staining can be a helpful prognostic indicator, as a higher percentage of CD123 positive cells has a** greater response to steroid therapy**
first-line treatment of lupus erythematosus panniculitis and may be the only medications needed
Antimalarials
- When monotherapy with hydroxychloroquine is ineffective, combination with quinacrine has been used successfully but is difficult to obtain
- interfere with both inflammatory cytokines and TLRs
- Antimalarials have a diminished effect in smokers
characteristic cell seen in Cytophagic histiocytic panniculitis (CHP)
Phagocytic histiocytes contain intact or fragmented cells and nuclear debris within their cytoplasm; this represents the characteristic “beanbag cell”
difference of beanbag cells from emperipolesis
- “beanbag cell” - phagocytic histiocytes contain** intact or fragmented cells** and nuclear debris within their cytoplasm
*emperipolesis - (the presence of an **intact cell within the cytoplasm of another cell) in which inflammatory cells pass through a histiocyte and no nuclear debris **is seen
medications that are often used and are often successful in treating Cytophagic histiocytic panniculitis (CHP)
cyclosporine and corticosteroids
■ Subcutaneous erythematous to violaceous plaques and nodules on extremities, trunk, and less frequently elsewhere; lesions may ulcerate.
■ Fever, hepatosplenomegaly, 2 or more cytopenias; hemocytophagocytosis in bone marrow, lymph nodes, liver, or CNS.
■ Hypertriglyceridemia, ferritin >500 mg/L; increased soluble CD25, CD163 levels.
Cytophagic histiocytic panniculitis (CHP)
histopath findings of Subcutaneous fat necrosis of the newborn (SCFN)
- Mostly lobular panniculitis without vasculitis.
- Dense inflammatory infiltrate of lymphocytes, histiocytes, lipophages, and multinucleated giant cells.
- Needle-shaped clefts, often in radial array, within cytoplasm of histiocytes and multinucleated giant cells.
The most common complication of Subcutaneous fat necrosis of the newborn (SCFN)
hypercalcemia
- The hypercalcemia may be related to increased 25-hydroxyvitamin D3 –1α hydroxylase within the granulomatous infiltrates.
- Rarely, hypertriglyceridemia, hypoglycemia, thrombocytopenia, anemia.
the most severe potential adverse outcome of histopath findings of Subcutaneous fat necrosis of the newborn (SCFN) and may cause nephrocalcinosis
Hypercalcemia
needle-shaped clefts can be seen in what disorders
- subcutaneous fat necrosis of the newborn
- sclerema neonatorum
- post-steroid panniculitis
