Differential diagnosis

Manifestations of BP may resemble those of a variety of dermatoses, including drug reactions, contact dermatitis, prurigo, fixed urticaria, vasculitis, arthropod reaction and scabies. Clinical history, pathologic features and negative immunofluorescence microscopy findings are essential to distinguish these disorders from BP. Diseases within the pemphigus group can be easily differentiated on the basis of distinctive immunopathological features. In dermatitis herpetiformis IF microscopy...

Info

Increased on an average of 3.6 per year over the whole period. The prevalence of morphea was 220 100,000 population. As for SLE a family history of SSc increases the risk of SSc. An estimated prevalence of approx. 1 of SSc in relatives was calculated in a recent study of Englert et al. (1999) based on 715 sclerosis patients and 371 controls. Possible risk factors for SSc were for a long time only in the focus of a few selected scientists. Everything changed when silicon implants were suspected...

Systemic Disease

Thirty to 63 of SCLE patients have four or more American College of Rheumatology (ACR) diagnostic criteria for SLE (Sontheimer 1989 Chlebus et al. 1998 Parodi et al. 2000 Black et al. 2002). Musculoskeletal symptoms such as arthritis and arthralgias are the most common systemic manifestations observed. Overall, most patients with SCLE tend to have mild systemic disease and it appears that isolated joint symptoms are a marker for milder disease. Some authors have reported musculoskeletal...

Therapy

The treatment of SSc is challenging due to the complex disease process and the difficulty to specifically treat distinct subgroups of this relatively rare disease. Thus in 1995, an American College of Rheumatology Comittee published guidelines for the conduct of clinical trials in SSc (White et al. 1995). To date, there is no proven effective disease modifying treatment of SSc. Nevertheless, there have been significant breakthroughs in the treatment of several individual end-organ...

References

Asboe-Hansen G (1985) Epidemiology of progressive systemic sclerosis in Denmark. In Black CM, Myers AR (eds) Systemic Sclerosis (Scleroderma). Gower, New York, p 78 Ball C, Sackett D, Phillips B, Haynes B, Straus S (1999) Levels of evidence and grades of recommendations Bastuji-Garin S, Joly P, Picard-Dahan C, Bernard P, Vaillant L, Pauwels C, Salagnac V, Lok C, Roujeau JC (1996) Drugs associated with bullous pemphigoid. A case-control study. Arch Dermatol 132 272-276 Bernard P, Vaillant J,...

Summary

Morphea is an uncommon but potentially disabling condition. Abnormalities in immune system parameters, endothelial activation and fibroblast metabolism have been described, but a unifying pathophysiologic model remains to be tested. The clinician is faced with considerable uncertainty when choosing a treatment modality for LS. Given the benign natural progression of plaque type morphea, treatment with topical modalities such as super-potent corticosteroids or calcipotriol is prudent. For more...

The Relationship between EBA and Other Systemic Diseases

In Roenigk's review of the EBA world literature Roenick et al. 1971 , it was noted that there were many anecdotal reports of EBA associated with sytemic diseases such as SLE, diabetes, inflammatory bowel disease, amyloidosis, autoimmune thyroiditis, multiple endocrinopathy syndrome, rheumatoid arthritis, pulmonary fibrosis, chronic lymphocytic leukemia, thymoma, diabetes, and others Woodley et al. 1998 . However, EBA is a relatively rare disease, and most of these are anecdotal reports. It...

Clinical Appearance Classification

Hemifacial Pediatric Violaceous

This commonest form of LS is defined by the presence of lesions gt 1cm in diameter, occurring in 1 or 2 anatomical sites Peterson et al. 1995 amp 1997 . The trunk is the most commonly involved site 41-74 patients , but plaques can occur anywhere, including the face and neck 12-13 of patients Christi-anson et al. 1956 Peterson et al. 1997 . Onset is usually slow and insidious. Circumscribed oval patches may be erythematous and oedematous in the earliest stages, becoming indurated,...

Diagnosis

In contrast to skin manifestations associated with overt SCLE and SLE, patients with the different subtypes of CCLE will primarily present to dermatologists as long as systemic manifestations are missing. Once internal organ involvement has occurred, these patients will be referred to general practitioners or rheumatologists for analysis of systemic disease. Alternatively, general practitioners or rheumatologists may ask dermatologists to search for typical skin manifestations of LE in a...