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2.1
Chilblain Lupus Erythematosus Type 1
Chilblain lupus

Presentation

However, when patients present with isolated chilblain lesions, the diagnosis of LE may be difficult. We report a case of chilblain LE who presented with depigmented macules on the acral areas and face resembling acrofacial vitiligo. [go.gale.com]

In the context of the clinical presentation, histologic findings, and serologic markers, a diagnosis of chilblain lupus erythematosus (CHLE) was made. [mdedge.com]

Case presentation A 35-year-old Thai woman presented to her general practitioner in December with a 1-month history of an itchy and painful rash on the fingers of her right hand associated with finger swelling (figure 1). [ncbi.nlm.nih.gov]

Entire Body System

  • Sepsis

    Chilblains is a broad term used for a cold-related tissue injury and can be idiopathic or seen in anorexia (BMI <5% of the normal population), myelodysplasia and sepsis. [dermatologyadvisor.com]

Skin

  • Skin Patch

    Symptoms of Chilblain Lupus Chilblain lupus develops skin patches or lesions that are red or purple. These lesions usually occur on your fingers, heels, soles of your feet. Sores on the palms of your hands, knees, nose, and ears are less prevalent. [longdom.org]

    Depigmentation, patches of skin lightening or losing color. Heel fissures, deep cracks in the skin on your heels. Hyperkeratosis, skin thickening. Raynaud’s phenomenon, temporary blood restriction in your fingers and toes. [my.clevelandclinic.org]

Neurologic

  • Hyperesthesia

    Possibly hyperesthesia, central atrophy, also erosions or rhagades (over the dorsal parts of the finger joints) or also areal ulcers. [altmeyers.org]

Workup

The presence of other signs and symptoms suggestive of a hematologic disorder should prompt a systemic workup. [derm101.com]

Serologic workup revealed increased antinuclear antibody titers of 1:320 (reference range, <1:40) and anti-Ro/Sjögren syndrome antigen antibodies of 86 (reference range, <20). [mdedge.com]

Clinicians can distinguish between these lesions through histologic analysis and workup for systemic lupus. 7 Lupus pernio occurs secondary to sarcoidosis and is characterized by violaceous nodules and plaques on the central face. 8 Cryoglobulemia and [podiatrytoday.com]

(See Workup and Treatment.) SCLE lesions heal without scarring or atrophy, but they may result in dyspigmentation, which can be prominent. Severe systemic disease is unusual, but when it occurs, the patient may develop life-altering sequelae. [emedicine.medscape.com]

Treatment

Treatment aims are to reduced disease activity and minimise damage. The first-line treatment for mild and localised cutaneous lupus erythematosus is topical corticosteroids. [ncbi.nlm.nih.gov]

Treatment Options Trearment options are outlined in Table I. Table I. [dermatologyadvisor.com]

Therapy: Local treatment with medium strength glucocorticoid ointments temporarily under occlusion. Chloroquine (dosage see below antimalarials). [altmeyers.org]

Methods A literature search was conducted on PubMed using search terms “(dermatomyositis) AND (treatment)” and “(cutaneous lupus) AND (treatment)”. [dovepress.com]

Prognosis

Prognosis Prognosis is good. Recurrences may be observed annually with onset of cold weather. [7] Long-term follow-up of patients with chronic recurrent pernio is advised because this may reveal connective-tissue disease (lupus erythematosus). [emedicine.medscape.com]

Prognosis Special patient groups [amboss.com]

The prognosis is worse if associated with Raynaud’s phenomenon, chilblains and alopecia. [pcds.org.uk]

Etiology

The etiology of sarcoidosis is unknown. [ncbi.nlm.nih.gov]

Apart from rare monogenic deficiencies of complement factors, where lupuslike disease may occur in association with other autoimmune diseases or high susceptibility to bacterial infections, its etiology is multifactorial in nature. [edoc.mdc-berlin.de]

Figure 5: Etiology of primary and secondary perniosis. [skin-diseases-and-skin-care.imedpub.com]

Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. [msdmanuals.com]

Epidemiology

You might also be interested in Dermatology Occurrence/Epidemiology This section has been translated automatically. [altmeyers.org]

] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Nonfreezing Cold-Induced Injuries in Auerbach's Wilderness Medicine, 2-Volume Set, 7th Edition Links: epidemiology risk factors clinical features differential diagnosis management chapping cold injuries non freezing cold injuries (NFCI) [gpnotebook.co.uk]

Drug-induced pernio: Sulindac-induced cases have been reported. [12] Epidemiology Frequency United States The true incidence of pernio is unknown because pernio frequently is unrecognized or misdiagnosed. [emedicine.medscape.com]

SLE is relatively uncommon, is difficult to diagnose, and is not a reportable disease, so it is expensive to capture all diagnosed cases reliably for epidemiologic studies. [cdc.gov]

Pathophysiology

Its pathophysiological mechanism gives insight into the process of interferon-induced inflammation in auto-immune diseases. Keywords: Chilblain lupus; Interferon; JAK inhibitors; STING; hereditary. [pubmed.ncbi.nlm.nih.gov]

Causes of Chilblain Lupus The pathophysiology of chilblain Lupus in rare cases is uncertain. [longdom.org]

Pathophysiology of cutaneous lupus erythematosus. Clin Rev Allergy Immunol 2007;2013:85–106 [PubMed] [Google Scholar] 8. Weizel J, Brahler S, Baler R, et al. [ncbi.nlm.nih.gov]

Prevention

Prevention How can I prevent chilblain lupus symptoms? You can reduce chilblain lupus flare-ups by protecting yourself from cold temperatures. You may: Exercise regularly to improve blood flow and regulate your body temperature. [my.clevelandclinic.org]

Medical Care Prophylactic warming of acral areas, achieved by heat and appropriate clothing, best prevents pernio. [dermaamin.com]

None of the usual treatments for cutaneous LE is effective for the chilblain lesions, probably because none is directed towards prevention and treatment of the microvascular stasis. [ncbi.nlm.nih.gov]

Treatment of specific manifestations of SLE is aimed at prevention of complications. [medical-dictionary.thefreedictionary.com]

Prevention To prevent chilblains: Avoid or limit your exposure to cold. Dress in layers of loose clothing and wear mittens and warm, water-resistant footwear. Cover all exposed skin as completely as possible when going outside in cold weather. [mayoclinic.org]

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