I would also like to know what is blaschkoid LE. are both LP & BLE autoimmune diseases ? What are the remedies ? More detailed info can be found at: http://www.maxillofacialcenter.com/BondB...
Lichen planus is a lichenoid autoimmune mucositis with a clinically different but microscopically similar dermal counterpart. On the skin the disease is usually of shorter duration, approximately 3 years, and does not have the ulcerating and blistering effects seen frequently in oral lesions. In the mouth lichen planus has several clinical variants with considerable cross-over between variants, and with occasional shifting from one variant to another. Some of these variants are thought to represent an elevated cancer risk but there is ongoing debate as to the validity of this hypothesis.
Some cases have obvious etiologic associations, usually a systemic medication or mucosal contact with dental materials or certain spices, but the etiology in most cases remains unknown. There is no strong association between oral and dermal lesions and most persons with oral involvement never have skin involvement. Oral lichen planus can be found in 1/1,000 adults (Table 1).
Discoid and systemic lupus erythematosus may present with oral keratotic and ulcerative lesions which are clinically identical to lichen planus and show a strong histopathologic similarity as well. Elongated, thin rete ridges are more likely to be associated with lupus, as is deep extension of the subepithelial lymphocytic band, especially with lymphoid aggregates present. Rete hyperplasia in lupus may, in fact, be so extensive that dyskeratosis occurs and the epithelium takes on the localized appearance of pseudoepitheliomatous hyperplasia. Thickened or degenerated endothelium with perivascular infiltrates is, of course, very helpful for the identification of lupus vasculitis, but these changes are often missing in oral examples. Cutaneous lupus lesions usually show a positive IgG and IgA reactivity along the basement membrane, and a patchy band of complement reactivity may be seen on immunofluorescence. Circulating anti-nuclear antibodies may also be present in cases of systemic disease, but an extensive discussion of the extraoral characteristics of lupus is beyond the scope of the present chapter.
Lichen sclerosus et atrophicus is the final lesion to differentiate from oral lichen planus. Extremely rare in the mouth, this typically genital mucositis may be clinically indistinguishable from oral lichen planus. The epithelium is uniformly atrophic, often extremely so, and only a thin layer of surface keratin is seen. There is typically extensive subepithelial fibrosis or hyalinization and a lesser inflammatory infiltrate is noticed; the infiltrate is often separated from the epithelium by a hyalinized band. Subepithelial hyalinization is also a feature of systemic sclerosis or scleroderma, amyloidosis and oral submucous fibrosis. Congo red birefringence and thioflavin T fluorescence can help to rule out amyloidosis, but differences in clinical features may be needed to rule out the other disorders.
There is no cure for this disease and therapy is only palliative. Fortunately, oral lichen planus lesions wax and wane, and are typically asymptomatic. For those patients suffering from tenderness and sensitivity to acidic foods, topical or systemic prednisolone is usually effective but should be used sparingly because of the potential systemic side effects. Persons affected with oral lesions seldom develop skin lesions, although the clinician should be on the lookout for evidence of lupus erythematosus during follow-up examinations, especially in patients with arthritic joint pains.
For patients with atrophic or ulcerative or bullous forms of the disease, an examination for early oral cancer should be performed every 4-6 months. This follow-up may entail repeat biopsies of areas of unhealing ulceration, induration or deep erythema. The estimated risk of malignant transformation, if real, is less than 2% over a 10 year period. Lichen sclerosus et atrophicus of the mouth carries no malignant potential, as it does in the genital region.
2nd part of your question:
Widespread Blaschkoid lichen planus by
Heather A Klein MD, Richard A Krathen MD, Sylvia Hsu MD
Dermatology Online Journal 12 (7): 17:
this info can be found here: http://dermatology.cdlib.org/127/case_pr...
Lichen planus is a cutaneous and mucous-membrane disorder of unknown etiology characterized by pruritic, planar, polygonal, purple papules that upon close examination have a white lacy reticular surface. Several variants have been described, including linear lichen planus sometimes following Blaschko lines. Blaschko lines, distinct from Voight lines, Langer lines, and the lines of innervation of the spinal nerves, follow a V-shape on the back, an S-shape on the abdomen, an inverted U-shape on the upper chest, and a linear pattern down the front and back of the lower extremities [1]. Long et al. reported linear lichen planus following Blaschko lines, as in our patient [2]. This patient's lesions were not confined to one side of the body, but rather began on the right side of the chest and spread to the trunk, arms, left thigh, left foot, and third finger of both hands.
This patient was given a 3-week course of prednisone 40 mg daily with clinical and symptomatic improvement, at which time the patient was tapered off prednisone over the next few weeks.
Blaschko's lines, also called the Lines of Blaschko, are an extremely rare and unexplained phenomenon of human anatomy first presented in 1901 by German dermatologist Alfred Blaschko. Neither a specific disease nor a predictable symptom of a disease, Blaschko's lines are an invisible pattern built into human DNA[citation needed]. Many inherited and acquired diseases of the skin or mucosa manifest themselves according to these patterns, creating the visual appearance of stripes.
The cause of the stripes is thought to result from mosaicism; they do not correspond to nervous, muscular, or lymphatic systems. What makes them more remarkable is that they correspond quite closely from patient to patient, usually forming a "V" shape over the spine and "S" shapes over the chest, stomach, and sides. |
