Cutaneous lupus erythematosus (CLE) is a chronic inflammatory autoimmune disease with a broad spectrum of clinical manifestations and a variable course. In numerous investigations, it has been shown that exogenous factors, such as UV-light and drugs, can induce this disease. However, not all clinical aspects can be explained and therefore, the pathogenesis of CLE is currently under extensive research. The various cutaneous manifestations of LE are divided into LE-nonspecific and LE-specific skin disease based on histologic criteria. LE-nonspecific manifestations are mostly associated with systemic LE but can also occur in other diseases and include particularly vascular skin lesions such as pe-riungual telangiectases. LE-specific skin disease includes the subtypes of CLE such as acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE), and intermittent CLE (ICLE). The subdivision of these subtypes with different prognosis and course is supported by genetic, clinical, histologic, and immunoserologic findings. The subtypes of CLE require a specific morphological and clinical analysis, which is described in the first part of this review. In the second part of this review, further diagnostic procedures and therapeutic strategies in patients with CLE are discussed.

/pdf/clinical-manifestations-of-cutaneous-lupus-erythematosus-5ccat5gife.pdf

Clinical Manifestations of Cutaneous Lupus Erythematosus

Chilblain lupus erythematosus (CHLE) is a rare, chronic form of cutaneous lupus erythematosus. Sporadic cases and two families with autosomal dominant-inherited CHLE have been reported. In familial CHLE, two missense mutations in TREX1 encoding the 3'-5' repair exonuclease 1 were described in affected individuals. The pathogenesis of sporadic CHLE remains unknown. Up to 20% of patients develop systemic lupus erythematosus (SLE). An association with anorexia is discussed. In many cases, there is good response to symptomatic therapy. SLE therapeutics have good effects on SLE-typical symptoms but not on chilblains themselves. This article reviews the clinical presentation, pathogenesis, diagnosis and treatment of CHLE. As an index patient with unique features, we report a 13-year-old boy developing CHLE after anorexia nervosa. Sequencing of TREX1 was normal. With psychotherapeutic support for anorexia and after antibiotic therapy, topical steroids, physical warming and calcium channel blockers, the patient experienced significant relief. Improvement of phalangeal perfusion was demonstrated by angio-MRI.

https://link.springer.com/content/pdf/10.1007%2Fs10067-008-0975-0.pdf

Chilblain lupus erythematosus—a review of literature

Chilblain lupus erythematosus Hutchinson: successful treatment with mycophenolate mofetil.

Cutaneous manifestations of lupus erythematosus (LE) are, usually, characteristic enough to permit an easy diagnosis. However, some patients may present less typical lesions, associated or not to the classic ones. Therefore, irrespectively of the variety of LE (acute, subacute and chronic), in absence of the typical butterfly rash, erythematosquamous papules or plaques, or any of the characteristic cutaneous alterations, it is important (even though not always easy) to recognize the uncommon and/or atypical changes of the skin.

Uncommon cutaneous manifestations of lupus erythematosus

Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity. The exact pathomechanisms and interactions resulting in the inflammatory and immunological processes of this heterogeneous disease remain elusive. Approaches in the understanding of the pathomechanisms revealed that the clinical expression of LE is predisposed by susceptibility genes and that various environmental factors are responsible for an abnormal immune response. Several studies demonstrated that ultraviolet (UV) light is one of the major factors in the pathogenesis of the disease. Standardized photoprovocation in patients with LE has been shown to be a safe and efficient model for evaluating the underlying pathomechanisms which lead to the production of autoantibodies and immune complexes. In particular, interferons were defined as important players in the early activation of the immune system and were observed to play a specific role in the immunological interface between the innate and the adaptive immune system. Abnormalities or disturbances in the different processes of cell death, such as apoptosis or necrosis, have also been recognized as crucial in the pathogenesis of LE. Although each process is different and characterized by unique features, the processes are interrelated and result in a complex disease.

Lupus erythematosus revisited

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. These symptoms and possible progression to systemic lupus erythematosus are discussed with regard to two cases. Patients with recurrent or persistent "chilblains" should be investigated histologically and serologically as well as by immunofluorescence.

Chilblain lupus erythematosus

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Chilblain lupus erythematosus