What is chilblain lupus erythematosus?
Elder lupus erythematosus (LE) is a rare variant of chronic cutaneous LE and was originally described by Jonathan Hutchinson in 1888 .
The term "chilblains" is derived from the Anglo-Saxon terms "cold" which refer to "cold" and "blegen", which is synonymous with "pain".
Chilbains from lupus
Who gets measles, lupus erythematosus?
Chilblain LE is a little recognized entity. In a 2008 review, the authors stated that 70 cases have been reported in the literature, with a limited number of adolescents. .
Chilblain LE occurs as a sporadic and hereditary condition. Sporadic cases are the most common and occur predominantly in middle-aged women. . Family Measles LE presents in early childhood .
Associations with intestinal anorexia nervosa lymphoma, and pregnancy have been described in the literature.
What Causes Chilblains Lupus Erythematosus?
the Pathogenesis of the sporadic form of the savanna LE remains unknown .
Cold stimulus is believed to cause vasoconstriction or microvascular injury with occlusion of the capillary bed and decreased circulation, leading to hyperviscosity and skin stasis, which is exacerbated by reduced blood temperature [2,3].
These pathophysiological changes in flow and microvasculature can also be aggravated by immune abnormalities (eg, rheumatoid factor or autoantibodies)
In those with a family history, or onset of sabacolin LE in childhood, the condition may be the result of a mutation at TREX1 or SAMHD1 genes .
- TREX1 is a gene Located in chromosome 3 encoding a nuclear protein with a role in DNA Repair and correction of DNA polymerase tests.
- SAMHD1 is a gene located on chromosome 20 that encodes a host Restriction nuclease with a role in the innate immune response.
These mutations in TREX1 and SAMHD1 are inherited through a autosomal dominant mode of inheritance [4,6]. Heterozygous mutation (a mutation of one allele) in these genes lead to the accumulation of nucleic acids (e.g., dsDNA, RNA: DNA duplex).
TREX1 Mutations have also been described in other microvascular-related conditions endothelial dysfunction, like the dominant retina vasculopathy and cerebral leukodystrophy.
What are the clinical features of measles lupus erythematosus?
Chilblain LE starts out as dark red or purple patches, papulesand plates that are initiated or exacerbated by exposure to cold and humidity in a cold climate. Lesions are often pruritus and painful
The common sites involved are the fingers and toes, heels, and soles. Less commonly, injuries may also be present on the nose, ears, palms, knuckles, elbows, knees, and legs.
Other features may include:
- Hyperkeratotic cracked injuries
- Depigmentation reminiscent of vitiligo
- Raynaud's phenomenon.
People who live in regions with warmer and drier climates may not develop typical chilblains.
In some circumstances, chilblains may represent lupus pernio with pre-existing LE rather than chilblains LE . In these cases, although the two entities are clinically similar and both are associated with systemic disease (sarcoidosis and systemic lupus erythematosus, respectively), the forecast and treatment regimens are different .
What are the complications of chilblain lupus erythematosus?
Complications of savanna LE include:
- Secondary bacterial skin infection
- Progression to systemic lupus erythematosus (SLE).
Monitoring is recommended for those with SLE characteristics (for example, malar eruption)
How is chilblain lupus erythematosus diagnosed?
The Mayo Clinic Diagnostic Criteria for the diagnosis of Savannah LE is made up of two major criteria and four minor criteria. . In order to diagnose LE measles, the main criteria and at least one minor criteria must be demonstrated.
- Injuries to acral Places induced by exposure to cold or any decrease in temperature.
- Evidence of LE in skin lesions due to histopathological exam or indirect immunofluorescence study
- A shallow and deep lymphocytic vascular reaction
- Fibrin statement in lattice, dermal-settled down blood vessels
- Interface dermatitis with degeneration of the dermoepidermal junction with an accumulation of IgM, IgA and C3.
- Coexistence of SLE or other forms of cutaneous LE
- Response to anti-LE therapy
- Negative results of cryoglobulin and cold agglutinin studies.
Chilblain LE is often associated with various immune manufacturers including:
- Anti-Ro / SS-A antibodies
- Rheumatoid factor
- Antinuclear antibodies
- Antiphospholipid antibodies
- Positive latex agglutination tests. .
Raynaud's phenomenon can also coexist .
Lesions that persist beyond the colder months, positive antinuclear antibodyand concomitant The characteristics of the American College of Rheumatology (ACR) criteria for SLE distinguish the sabaquin LE from idiopathic chilblains .
Which is the differential diagnosis for measles lupus erythematosus?
Other skin conditions that can be confused with elderberry lupus include perniosis (ordinary chilblains), acrocyanosis, Raynaud's phenomenon, lupus pernio, livedo reticularis, dermatomyositis, cold panniculitis, and cold urticaria.
Perniosis (or common chilblains) is characterized by:
- An itchy, burning, or painful sensation.
- Symmetrical erythrocyanotic discoloration of acral skin
- Not specific histology dermal compound edema with shallow and deep lymphohistiocytic infiltrate and perieccrine accentuation.
Acrocyanosis is characterized by:
- Painless lesions, in most cases.
- Red to dark purple discoloration
- Acral distribution.
Raynaud's phenomenon is characterized by:
- The sequence of welldemarcated pallor, followed by blue, then red discoloration
- Absence of ulceration (with the idiopathic form).
Livedo reticularis is characterized by:
- Bluish, broad, lattice patches
- Association with:
- Hematological disorders (eg, antiphospholipids syndrome)
Cutaneous vasculitis (eg, polyarteritis nodosa)
- Infections (eg, Syphilis)
- Medication (eg, Minocycline).
Dermatomyositis can cause nail fold erythema, telangiectasia and nail fold inflammation similar to chilblain lupus erythematosus Other clinical features of dermatomyositis include:
- Muscular weakness (myositis)
- Facial rash including heliotrope (mauve) eyelids
- Gottron's papules (dry red plaques on the knuckles).
Cold panniculitis is characterized by:
- Erythematous, indurated plaques that develop at sites of cold exposure
- Location on the cheeks and chin.
- Occurrence in children and young women.
- Resolution within two weeks.
Cold urticaria is a form of chronic disease. inducible urticaria characterized by:
- Cold-induced cure and angioedema
- Systemic symptoms in some patients.
What is the treatment for chilblain lupus erythematosus?
Protection against cold should be emphasized through various physical measures.
Examples of these measures include:
- Avoid cold and humid environments, when possible.
- Ensuring that the home and workplace are well insulated and provide heating
- Wear gloves, thick wool socks and comfortable protective shoes.
- Soak your hands in warm water to warm them throughout the day
- Participate in physical activity to maintain core body temperature.
Of smoking cessation It should also be encouraged, due to the vasoconstrictive effects of nicotine.
Medication may be necessary for patients with recalcitrant lesions and for secondary bacterial infections of the skin. A therapeutic An evidence-based approach to therapies in LE measles has been proposed .
These include the following:
Antibiotics (for secondary bacterial skin infections)
- Current steroids
- Systemic calcium channel blockers
- Systemic steroids
- Mycophenolate mofetil
Antimalarial agents (eg, Hydroxychloroquine)
Patients have been treated successfully in certain circumstances for excision and repair using a full thickness graft derived from an unaffected area.
What is the outcome for chilblain lupus erythematosus?
Most patients respond well to symptomatic treatment.
In those with the sporadic form, people's 18% will eventually develop SLE; there is no evidence of progression to SLE in LE familial measles.