Academic journal article International Journal of Child Health and Human Development

Collagen Vascular Disorders

Academic journal article International Journal of Child Health and Human Development

Collagen Vascular Disorders

Article excerpt

Introduction

Lupus erythematosus (LE) (systemic lupus erythematosus; SLE) is a multisystemic spectrum of conditions characterized by chronic inflammation due to autoimmunity against ribonucleoproteins and nucleosomes (1). The underlying pathophysiology is complex and still not clear. Several underlying etiopathologic processes have been implicated in LE including immunogenetic, environmental, hormonal, and infectious factors (2).

Epidemiology

The exact prevalence of LE (SLE) is not known and a prevalence of 5-10/100,000 is estimated (3, 4). About 25% of LE starts in the first two decades of life. Sex distribution for males and females is about 3:4 until puberty; however, this ratio becomes 1:9 after puberty (5). Racial differences exist with much higher incidence in African-Americans, Hispanics, and Asians as compared to Caucasians (4, 6).

Clinical features

A wide spectrum of clinical presentation is seen in LE primarily because of multi-systemic involvement, variable onset, and unpredictable course of the disease. The typical presentation may be modified significantly with early institution of therapy. The general and systemic clinical presentations are listed in Table 1 (7). A high index of suspicion for LE should be maintained for females in child bearing age group presenting with fever, rash, and joint pains.

Cutaneous manifestations

There are three main dermatologic features in the diagnostic criteria as per the 1982 revised criteria for the classification of SLE (7) These include:

· Malar rash: This is an erythematous rash over the cheeks and bridge of the nose, spares the naso-labial folds, and has been typically referred to as the classic butterfly rash of SLE or acute cutaneous lupus erythematosus (ACLE) as well. These lesions may last for variable periods of time ranging from hours to weeks, sometimes even longer. Generally these lesions do not leave any scarring on healing.

· Photosensitivity: This is another prominent feature of SLE as well, so much so that sometimes the generalized form of ACLE is also referred to as photosensitive lupus dermatitis. This is typically precipitated or exacerbated by sun exposure. The malar rash and photosensitivity of SLE both portray waxing and waning during the course of the disease.

· Discoid rash: This is the cutaneous manifestation seen in the subacute cutaneous type of LE (SCLE), also referred to as disseminated lupus erythematosus (DLE). These lesions are also photosensitive, and therefore observed in generally the sun-exposed areas such as the neck, shoulders, arms, upper back, and also less commonly, the face. Initially these present as erythematous, maculopapular lesions which may transition to hyperkeratotic, papulos-quamous lesions, annular plaques, or polycyclic plaques. (1)

· Other nonspecific cutaneous lesions: Other cutaneous lesions may be seen which are otherwise not specific for LE. These include: 1) alopecia generally involving the temporal area of the scalp and exhibiting patchy hair loss; 2) Raynaud's phenomenon; 3) panniculitis, also referred to as lupus profundus; 4) livido reticularis; 5) bullous, erythema multiforme-like lesions; 6) vasculitic purpura or telangiectasia. Urticaria has also been reported with SLE. Lupus may be considered in cases of oral, vaginal, and even more specifically palatal ulcers (1).

Neuropsychiatric systemic lupus erythematoses (NPSLE)

SLE in children is more likely to present with early central nervous system involvement. Varied prevalence rates ranging from 20%-95% for NPSLE have been reported in the pediatric population. However, this may be more reflective of lack of a unified process of NPSLE characterization (8-11). Also, 70% of children may manifest features of NPSLE in the first year of illness (10).

The manifestations of NPSLE may be due to different pathophysiological processes including: 1) the primary autoimmune and inflammatory attributes of the underlying disease; 2) complications of the main disease process; 3) unintended side effects of the medications being used to treat the primary disease process; or 4) due to the psychosocial and emotional burden as well as stigma of the disease (8). …

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