Case of Chilblain lupus erythematosus in 29-year-old
Chilblain lupus is a rare, chronic form of cutaneous SLE that is characterized by tender, pruritic, erythematous lesions that usually affect the acral surfaces. It is often referred to as “Hutchinson lupus” or perniotic lupus. Ears and nose are less commonly impacted. When affected, the palms and soles may present with necrosis or fissures.It frequently precipitates in cold temperatures. Its pathogenetic aspects include cold-induced vascular thrombosis, blood stasis, and impaired microcirculation. Chilblain lupus has been linked to anti-Ro antibodies. In addition, both the deep and superficial layers of skin exhibit lymphohistiocytic infiltration, according to histopathology. This article describes the case of a 29-year-old woman with a history of lupus erythematosus, presenting with a rash. Based on the clinical findings, she was diagnosed with chilbain lupus eryhtematosus.
Case report
The woman presented at the dermatology clinic complaining of a severe, itchy rash on her hands and nose that had been there for two weeks. According to the patient, the rash first developed when the weather was colder. The patient said they had not been in the sun. The physical examination revealed punched-out ulcers on the nose and erythematous macules and papules (Panel A). The palms had sporadic papules, and there was edematous erythrocyanosis of the fingertips (Panel B) and lateral aspect ulcerations (Panel C).
Antinuclear, anti-double-stranded DNA, anti-Ro, antiphospholipid, and rheumatoid factor antibodies were all positive in blood testing, and hypergammaglobulinemia was detected. Testing for cold agglutinin and cryoglobulin was negative. A biopsy sample from the right nasal sidewall underwent histopathological analysis, which identified perivascular lymphocytic infiltration and vacuolar interface dermatitis. A diagnosis of lupus erythematosus with chilblains was made. Chilblain lupus erythematosus is a rare kind of disease. Similarly, it is rare for the nose to be affected by the inflammatory skin lesions, which are usually brought on by exposure to cold. Glucocorticoids, azathioprine, nifedipine, and hydroxychloroquine were started as treatments. There was also counseling on avoiding the cold. Upon a follow-up visit after two weeks, the skin blemishes had subsided.
The Mayo Clinic diagnostic criteria
Vasculitis of the small vessels is a crucial differential diagnosis. ACLE rash and lupus-related vasculitis are typically linked to active illness. The interphalangeal regions of the fingers are impacted when an ACLE rash occurs on the hands. Chilblain lupus is treated with topical steroids, topical calcineurin inhibitors, calcium channel blockers to relieve vasoconstriction, and protection from the cold. The role of antimalarials is questionable. When topical therapy fails, patients may be referred for systemic immunosuppression.
Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. Furthermore, a number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). Raynaud’s phenomenon and photosensitivity are other prominent clinical features in patients with CL. Nailfold capillaroscopy often reveals pathological changes in every patient examined.
Characteristic findings on physical examination
It is easier to identify discoid lupus erythematosus (DLE) because most patients present with lesions elsewhere, particularly on the face and head. Usually, DLE develops before chilblains appear. Isolated lesions, however, make diagnosis challenging unless there is a high degree of suspicion.
Lupus pernio (Hutchinson) or lupus chilblain lupus is not the same as lupus pernio (Besnier), which is brought on by sarcoidosis. The latter is not especially brought on by cold, but rather manifests in a “pernio” distribution, mostly affecting the nose, ears, and cheeks. Under dermoscopy, these violaceous papule lesions have a “apple jelly” tint. They typically appear as beaded, violaceous papules surrounding the nasal ala on the nose. Sarcoidosis is the most helpful histopathological finding in lupus pernio (Besnier).
Pernio, sometimes known as “frostnip,” is an inflammatory illness that develops in cold, damp, non-freezing weather and is typically observed in the absence of central heating. A burning and stinging sensation is usually experienced in the distal fingers and toes. Clinically, macules and/or papules that are deep blue or violet are observed.
In conclusion, most chilblain lesions occur on the knee, calves, toes, fingers, and elbows, and they are often accompanied by hyperkeratosis and/or fissuring. Nasal tumors are not common. Fissuring is frequent when pulp lesions form in the fingers or toes. On the other hand, ulceration and atrophic papules are usually absent.
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