Department of Rheumatology, General University Hospital of Ciudad Real, Ciudad Real, SpainSpain
Department of Dermatology, General University Hospital of Ciudad Real, Ciudad Real, SpainSpain
Department of Pathology, General University Hospital of Ciudad Real, Ciudad Real, SpainSpain
Department of Family and Communitary, General University Hospital of Ciudad Real, Ciudad Real, SpainSpain
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Perniosis or chilblain pernio is an inflammatory skin disease, typically located on acral skin surfaces secondary to prolonged expo-sure to cold temperatures and damp conditions. This disorder can appear as an idiopathic dermatosis, usually in young women, but occasionally it is associated with diseases such as anorexia, intestinal lymphoma, chronic myelomonocytic leukemia, monoclonal gammopathies, cryoproteinaemia, viral infections, as well as connective tissue diseases, especially lupus, picture that is known as chilblain lupus. We present a case of perniosis associated with Systemic Lupus Erythematosus and review the literature.
Keywords: Chilblain lupus; Perniosis; Chilblain pernio; Rare cutaneous; Myelomonocytic leukemia; Systemic Lupus Erythematosus; Chilblain lupus; Perniosis; Chilblain pernio; Rare cutaneous; Myelomonocytic leukemia; Systemic Lupus Erythematosus
matology and Dermatology departments made the diagnosis of SLE according to systemic lupus international collaborating clin- Chilblain is generally a relatively benign disease, but it can someics (SLICC) 2012 criteria with pulmonary involvement (interstitial times predict a more serious underlying disease, such as SLE, lung disease), with ANAs and anti-DNA positive antibodies and even to present severe pulmonary manifestations, as has been our chilblain lupus was reached, so the patient began treatment with case, so that treatment with antimalarials and other immune suphydroxychloroquine 200 mg twice daily, prednisone 10 mg daily pressants is recommended.4 In patients with the chronic-onset and azathioprine 100 mg daily with partial improvement of skin disease, a careful evaluation is warranted for screening for undermanifestations and lung disease.
lying disease and subsequent clinical follow-up.
Chilblain pernio or perniosis is a rare cutaneous manifestation of SLE that, despite being uncommon, should be followed up when we diagnose erythema pernio. It may go on to develop systemic manifestations of SLE and transform into a systemic disease (chilblain lupus).
From the clinical point of view, the presence of skin lesions compatible with chilblain that persist for a long time and persist beyond the cold seasons obliges us to carry out screening for an associated autoimmune disease. Therefore, complementary tests are needed. It includes the search of laboratory data of SLE,5 as it is in our case, making a definitive diagnosis for presenting tenderness in 2 or more joints with morning stiffness (arthritis), positive ANAs, positive anti-DNA and skin involvement (chilblain lupus), according to SLICC 2012 criteria. In our case, the diagnosis of sarcoidosis was rejected, by the Rheumatology and Dermatology Departments, due to the lack of meeting classification criteria according to the international consensus A Case Control Etiologic Study of Sarcoidosis (ACCESS): granulomas were not observed in the skin biopsy, despite the typical pulmonary involvement of sarcoidosis, and that there was no consistent evidence of the involvement of at least 2 systems or organs for diagnosis.
The skin biopsy, generally, does not offer any help to distinguish chilblain lupus from the idiopathic form. However, at least half of the biopsies of patients with chilblain lupus show some typical histological characteristics of chronic cutaneous lupus erythematosus: interface dermatitis with vacuolation of the basal layer and necrotic keratinocytes with a perivascular dermal infiltrate. On the contrary, the periecrine distribution of the infiltrate is more typical of the idiopathic form.6,7
With the final diagnosis of SLE according to SLICC 2012 (skin involvement, arthritis, positive ANAs, positive anti- DNA), it was decided to establish treatment with immunosuppressants for the control of systemic disease.
The authors have received written informed consent from the patient.
The authors declare that they have no conflicts of interest.