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. [ijdvl.com]

Chilblain lupus erythematosus with depigmentation is a rare presentation of lupus erythematosus that may simulate vitiligo. A 52-year-old lady with such a manifestaiton is being reported. [ucdavis.pure.elsevier.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]

• Pain

  Type your tag names separated by a space and hit enter Abstract Chilblain lupus erythematosus is a chronic form of lupus erythematosus that is seen predominantly in women and first manifests itself during cold weather as painful purplish acral swellings [wwww.unboundmedicine.com]

  It may help ease the muscle pain associated with lupus. References [ + ] [healthjade.com]

  Perniosis Perniosis (or common chilblains) is characterized by: An itchy, burning, or painful sensation. [doctorhoogstra.com]

  CLE is one of the forms of skin manifestations of SLE (1), is characterized by painful papuloerythematous plaques over the fingers (2), (3), and can be mistakenly diagnosed as chilblain (3). [jmaj.jp]

  No personal or family history of oral ulcers, joint pains, photosensitivity, frothing of urine or any systemic complaints in the form of chest pain, abdominal pain, or breathlessness was found. [idoj.in]

• Fever

  (c) Raynaud phenomenon involving the fingers Click here to view Discussion Fever can be the presenting symptom in 30%–80% of active SLE. [ijms.in]

  She had no history of fever, joint pain, oral ulcers, malar erythema, alopecia, photosensitivity or any other systemic complaint. [go.gale.com]

  Typical findings include fever and fatigue, a malar rash (facial " butterfly rash "), myalgia, and arthritis. [amboss.com]

  Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis. [medical-dictionary.thefreedictionary.com]

• Underweight

  Being underweight. This condition is most common in people with a low body mass. Living in cold, humid areas. Your risk of chilblains is higher if you live in an area with high humidity and cold but not freezing temperatures. [mayoclinic.org]

  […] when exposed to cold: individuals with close family members who have/had chilblains patients with circulation problems patients with lupus people whose homes are draughty and cold (not well insulated) tobacco smokers women are more susceptible than men underweight [medicalnewstoday.com]

  The mean age of onset of perniosis symptoms was 39.6 ± 16.7 years, and the mean BMI of perniosis patients was 24.3±5.4. 65.8% of patients had a BMI classification of underweight or normal weight, while 34.2% of patients had a BMI classification of overweight [skin-diseases-and-skin-care.imedpub.com]

  People who are underweight (about 20 percent less than are expected for their height) have an increased risk of chilblains. Where a person lives. [lucianoschiazza.it]

• Nail Abnormality

  Ridged nails Ridges running either the length or width of the nail lichen planus or lupus can affect the nails. Abnormalities may Nails fingernail and. [lupusresearch.review]

  In addition, we performed nail fold capillaroscopy to determine the presence of microvascular abnormalities. [karger.com]

• Pleuritic Pain

  Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis. [medical-dictionary.thefreedictionary.com]

  pain or a pleuritic rub during auscultation OR Pericarditis documented by ECG, a rub during auscultation, or evidence of a pericardial effusion. [amboss.com]

  Subsequently she developed painful erythematous swellings on the feet and scaly plaques on the forearm and thigh consistent with subacute cutaneous lupus. [thedoctorsdoctor.com]

• Ulcer

  In all three cases, two biopsies were performed one from the ulcer center and the other from either the ulcer edge (Cases 4 and 6) or non-ulcerated papular lesion (Cases 5.) [ijdpdd.com]

  In such patients, this subtype of CCLE can last for several months and tends to recur annually, sometimes with hem-orrhagic blisters, erosions, or ulcers. [mussenhealth.us]

  [Medieval Latin, ulcerative skin disease, from Latin, wolf (the scarring caused by such diseases being likened to the marks left by wolf bites); see wl̥kwo- in Indo-European roots.] [thefreedictionary.com]

  D, Ulcerative, cold-induced infiltrates of the fingers of patient II-3. E, Oral palatinal erosion in patient II-3. [jamanetwork.com]

  Gold Medicine The British journal of dermatology 1960 It is apparent that for many years the term lupus was used somewhat indiscriminately for ulcerative and mutilating skin conditions, a great many of wliich must surely have been cancerous, and things [semanticscholar.org]

• Skin Lesion

  The two major criteria are skin lesions in acral locations induced by exposure to cold or a drop in temperature, and evidence of lupus erythematosus in the skin lesions by results of histopathologic examination or direct immunofluorescence study. [ncbi.nlm.nih.gov]

  DISCUSSION Gilliam and Sontheimer classified the skin lesions of LE into six types: discoid, profound, chilblain, annular-polycyclic, papulosqamous and acute skin lesions.[1] Chilblain or perniotic LE was first described by Hutchinson in 1888 as a tendency [ijdvl.com]

  (B) Similarily, his father showed erytheamtous skin lesions on dorsum of hands. Erythematous to purpuric swollen scaly patches were noted on the ear of the patient (A, B) and his father (C, D). [synapse.koreamed.org]

  […] lupus [ loo´pus ] a name originally given to a destructive type of skin lesion, implying a local degeneration rather than systemic involvement. [medical-dictionary.thefreedictionary.com]

• Papule

  She developed photosensitivity and erythema of face with periorbital heliotrope, discrete, erythematous, flat smooth surfaced papules on face and extensors of forearms since 15 days. [ijdvl.com]

  Lesions are chronic, indurated papules or plaques that affect the mid-face, particularly the alar rim of the nose. [ghorayeb.com]

  Clinically, CHLE presents as erythematous/purplish papules or plaques on the acral sites, both superior and inferior. [karger.com]

  The most common types of specific lesions are papules. 3 They commonly occur on the face but may occur anywhere on the body. While only a few papules may be noted ( Figure 3 ), multiple scattered or confluent lesions are more common. [aafp.org]

• Skin Disease

  [Medieval Latin, ulcerative skin disease, from Latin, wolf (the scarring caused by such diseases being likened to the marks left by wolf bites); see wl̥kwo- in Indo-European roots.] [thefreedictionary.com]

  Piaggio-Muente ARTICLE REFERENCES Abstract Perniosis or chilblain pernio is an inflammatory skin disease, typically located on acral skin surfaces secondary to prolonged exposure to cold temperatures and damp conditions. [openventio.org]

  Lasers Surg.Med 1992 On DermNet NZ: Cutaneous sarcoidosis Löfgren syndrome Darier-Roussy disease Other websites: Medscape: Lupus Pernio Sarcoidosis Dermatologic Manifestations of Sarcoidosis Lupus - American Academy of Dermatology Books about skin diseases [dermnetnz.org]

  This type of lupus is a skin disease that can affect people with or without SLE. “Cutaneous” means “skin.” Symptoms may include rashes, hair loss, swelling of the blood vessels, ulcers, and sun sensitivity. [cdc.gov]

  • patients with LE skin disease who smoke are less responsive to antimalarial treatment. 84. [slideshare.net]

• Hyperkeratosis

  A biopsy from the dorsum of the index finger revealed hyperkeratosis, irregular acanthosis, liquefaction degeneration of the basal layer and aggregation of lymphocytes around blood vessels. [ijdvl.com]

  Hematoxylin and eosin (H&E) staining from lesional skin. a Thin epidermis with hyperkeratosis, orthokeratosis, and mixed infiltrate in the dermis. [karger.com]

• Suggestibility

  These results suggest that surgical removal of local factors reduces the rash in these cases. [ncbi.nlm.nih.gov]

  On initial presentation the patient reported the lesions developed after wearing a new pair of tight-fitting shoes, suggesting a diagnosis of trauma-induced bullae. [mdedge.com]

  Some of these lesions are nonspecific, but others are highly suggestive of sarcoidosis. Cutaneous anergy is common. [meddean.luc.edu]

• Neglect

  […] child Child & Adolescent Mental Health Services child abuse child abuse accommodation syndrome child abuser child grooming child guidance child health surveillance child labor Child Labour child life specialist child maltreatment Child Murder child neglect [medical-dictionary.thefreedictionary.com]

  None Shall Look Back (1937), which had the misfortune to appear shortly after Margaret Mitchell's Gone with the Wind, is a distinguished but neglected novel with a theme similar to her first. [books.google.de]

  Excessive cold exposure and parental neglect have also resulted in pernio. Among adolescents, pernio has been seen in association with anorexia nervosa. [lucianoschiazza.it]

  PubMed View Article Google Scholar Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P: Lupus erythematosus tumidus–a neglected subset of cutaneous Lupus erythematosus: report of 40 cases. Arch Dermatol 2000, 136: 1033–41. [ped-rheum.biomedcentral.com]

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

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]

(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]

• Leukocytosis

  Laboratory tests revealed leukocytosis, elevation of the erythrocyte sedimentation rate (> 40), decreased level of albumin, and increase of α1-, α2-, and γ-globulins. [karger.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]

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]

PubMed Google Scholar In any case, it is essential to make a correct diagnosis; although clinically the two entities are similar and have in common their association with systemic disease (lupus erythematosus or sarcoidosis), the prognosis and treatment [journal.chestnet.org]

Although the two entities are clinically similar and both are associated with systemic disease (sarcoidosis and systemic lupus erythematosus, respectively), the prognosis and treatment regimens are distinct [7]. [dermnetnz.org]

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

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]

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

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

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]

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]

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]

CLE EPIDEMIOLOGY • ACLE All races are affected much more common in women than men (8:1). the malar rash in 20-60% of patients in LE Age – the malar rash is associated with a younger age of disease onset 9. [slideshare.net]

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

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]

The pathophysiology of the lesion is still not clearly known. [ijdvl.com]

Assessment of CLL's pathophysiology compared to SC showed both involved lymphocytic infiltration around superficial and deep dermal blood vessels. [news-medical.net]

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]

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 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]

Prevention To prevent chilblains: Avoid or limit your exposure to the cold. When you come in from the cold, rewarm the skin gradually. Dress in layers of loose clothing and wear mittens, a scarf and a hat, and warm, water-resistant footwear. [mayoclinic.org]