Intense papillary dermal edema is present. A superficial and deep perivascular lymphocytic infiltrate is seen, with the described "fluffy edema" of vessel walls. Lymphocytic vasculitis may be present. [dermaamin.com]

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]

• Increased Susceptibility to Infections

  Apart from arthralgias, there is no evidence for internal-organ disease or an increased susceptibility to infection. [edoc.mdc-berlin.de]

• Cushingoid

  Osteoporosis, myopathy and cushingoid changes are additional adverse reactions that can be avoided with short therapeutic courses. Of note, osteonecrosis can occur with even short courses of prednisone. [psychiatryadvisor.com]

• Yellow Discoloration of the Skin

  It may cuase yellow discoloration of the skin. If the combination of hydroxychloroquine and quinacrine are ineffective, two options are (1) switch to chloroquine and quinacrine or (2) start dapsone 50mg daily. [psychiatryadvisor.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, reference range. [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]

PubMed View Article Google Scholar Swart JF, Wulffraat NM: Diagnostic workup for mixed connective tissue disease in childhood. Isr Med Assoc J 2008, 10: 650–2. [ped-rheum.biomedcentral.com]

• Hyperviscosity

  The chilblain lesions are the result of microvascular injury secondary to exposure to cold and possibly hyperviscosity from immunological abnormalities, Elevated serum gammaglobulins, positive latex factor and speckled pattern antinuclear factor are common [ncbi.nlm.nih.gov]

  Multiple erythematous plaques and reticulate telangiectasia on the face that produce large areas of disfigurement on confluence hyperviscosity from immunologic abnormalities may play a role (Mascaro et al. 1997, Yell et al. 1996). [mussenhealth.us]

  Vasoconstriction produced by cold along with the hyperviscosity due to immunological abnormalities like hypergamm-aglobulinemia and rheumatoid factor are thought to lead to chilblain like lesions. [7] ANA is of the speckled pattern and cryoglobulins and [ijdvl.com]

  […] in early childhood and is caused by a heterozygous mutation in the TREX1 gene that encodes a 3'- 5' DNA exonuclease. [3] The pathogenesis of sporadic CHLE remains unknown, though vasoconstriction or microvascular injury secondary to cold and possible hyperviscosity [idoj.in]

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]

Treatments for chilblain lupus such as antimalarial agents, prednisone, pentoxifylline, nifedipine, and dapsone are reported to be beneficial. Natural treatments should also be considered in the treatment of chilblain lupus. [drgaryslupustreatment.org]

The treatment of lupus pernio: results of 116 treatment courses in 54 patients. [kundoc.com]

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]

Cutaneous sarcoidosis occurs in up to one third of patients cutaneous lesions may have a bearing on prognosis of systemic sarcoidosis. Lesions of lupus pernio. [latestlupusupdate.review]

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]

] 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]

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]

Epidemiology  More common in African American’s and Puerto Rican populations  Female> Male  Usually with long- standing systemic, usually pulmonary disease 6. [de.slideshare.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]

Pathophysiology Pernio is due to an abnormal vascular response to cold exposure, [1] and it is most frequent when damp or humid conditions coincide. [emedicine.medscape.com]

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 is the mainstay in the treatment of pernio. [podiatrytoday.com]

Prevention of chilblains To prevent chilblains: Avoid or limit your exposure to cold. Dress in layers of loose clothing and wear mittens and warm, water-resistant footwear. [healthjade.com]