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5.3.1 Panniculitis
ICD-11
EF00
Synonyms
None.
Epidemiology
Rare depending on underlying cause.
Definition
A heterogeneous group of diseases showing inflammation of subcutaneous adipose tissue.
Aetiology & Pathogenesis
Various underlying causes of suppurative and non-suppurative panniculitis:
- As part of morphea or scleroderma
- Due to alpha1-antitrypsin deficiency
- Post trauma
- Post infection
- Subcutaneous panniculitis T-cell lymphoma
- Lupus profundus
In association with:
- Tuberculosis (erythema induratum)
- Pancreatic disease
- Perinatal asphyxia or hypothermia (sclerema neonatorum and subcutaneous fat necrosis of the newborn)
- Post-steroid therapy
- Autoimmune dermatoses (systemic lupus erythematosus; dermatomyositis)
- Post trauma including surgery
- Post infection
- Chronic venous insufficiency (lipodermatosclerosis).
There may be overlapping causes of panniculitis (e.g. lupus erythematosus and trauma).
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Various underlying causes of panniculitis exist:
Inflammatory and autoimmune
Infection and infestation
Trauma and physical agents (exogenous)
Malignancy (haemo-lymphoproliferative disorders including lymphomas.
Enzymatic (pancreatic, alpha-1-antitrypsin deficiency)
In association with:
Tuberculosis (erythema induratum).
Pancreatic disease.
Perinatal asphyxia or hypothermia (sclerema neonatorum and subcutaneous fat necrosis of the newborn).
Post-steroid therapy.
Autoimmune dermatoses (systemic lupus erythematosus; dermatomyositis).
Post trauma including surgery.
Post infection.
Chronic venous insufficiency (lipodermatosclerosis).
There may be overlapping causes of panniculitis (e.g. lupus erythematosus and trauma).
Signs & Symptoms
Can be asymptomatic or affected areas tender on pressure. Fever, joint pains, systemic upset and ulcers may be present. Signs on examination of other systems may be helpful to make a clinical diagnosis.
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Painful inflammatory nodules and plaques occur in all form of panniculitis but making a diagnosis of a specific type of panniculitis on clinical examination is difficult except erythema nodosum. The patient history and additional clinical findings such as ulceration with discharge, atrophy, sclerosis, livedo reticularis, fever, arthralgias may be useful.
Localisation
Varies according to underlying cause. Lupus panniculitis: upper torso and hips. Alpha1-antitrypsin deficiency-associated panniculitis: pelvic girdle. Erythema induratum and lipodermatosclerosis: lower legs.
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The location of lesions is also a diagnostic clue. Erythema nodosum: anterior lower legs. Lupus panniculitis: Face, upper arms, breasts, upper torso and hips. Alpha-1-antitrypsin deficiency-associated panniculitis: pelvic girdle. Erythema induratum/nodular vasculitis: posterior lower legs. Lipodermatosclerosis: medial lower legs.
Classification
Predominantly septal panniculitis:
- As part of morphea or scleroderma
- Due to alpha1-antitrypsin deficiency
- Erythema nodosum
Lobular and mixed septal/lobular panniculitis: all other types listed above.
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The classification of panniculitis is based on clinical grounds and histopathology. Accordingly, panniculitis can be divided into septal, lobular and mixed (see Dermatopathology). The panniculitides most likely to ulcerate were calciphylaxis (85.7% ulcerating), pancreatic panniculitis (66.6%), and α1-antitrypsin deficiency-associated panniculitis (100%). The panniculitides least likely to ulcerate were erythema nodosum and medication-induced and granulomatous panniculitis.
Laboratory & other workups
Depending on the underlying diagnosis.
Dermatopathology
Usually not necessary. Isolated pure forms of panniculitis are rare, and clinical and other laboratory features should be considered.
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Dermatopathology plays an integral role in the diagnosis of panniculitis. An elliptical biopsy is the recommended procedure. Panniculitides have been divided into septal (erythema nodosum, scleroderma) and lobular including most of the other forms. In addition to the localization of the inflammation, the composition of the infiltrate is also useful. Lymphocytic infiltrate predominates in lupus panniculitis, neutrophil-rich infiltrate in alpha-1-antitrypsin deficiency panniculitis, histiocyte-rich infiltrate in erythema nodosum, mixed-type in traumatic panniculitis. The presence or absence of vasculitis is also another clue.
Course
Depending on the underlying cause. Some forms will burn out leaving hard permanent scars. Longstanding and migrating subtypes exist.
Complications
Depending on the underlying cause.
Diagnosis
Clinical features. Occasionally biopsy may be required.
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Clinico-pathologic correlation is essential.
Differential Diagnosis
Polyarteritis nodosa, nodular vasculitis and, cellulitis, cutaneous T-cell lymphoma.
Read more
Polyarteritis nodosa
Cellulitis
Cutaneous T-cell lymphoma
Behçet disease
Eosinophilic panniculitis
Lipodermatosclerosis
Lupus erythematosus
Morphea profunda
Post-irradiation panniculitis
Sclerema neonatorum
Subcutaneous fat necrosis of the newborn
Prevention & Therapy
Depending on aetiology.
Special
None.
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