lichen planus atrophicus.

this is characterized by atrophic in the center of the papules so that ultimately, atrophic spots are present. in this type the papules are usually flat and difficult to find. close inspection will usually reveal their angular outlines,with often a prominent central umbilication. aggregation of these lession form small ivory or violet colored patches, around which there may be a zone of erythema. these may simulate morphea guttata and lichen sclerosus et atrophicus. when this atrophic variant occurs on skin with a black or olive complexion, the center may be hyperpigmented. there may be deep pigmentation centrally with only a rim of purple annular lesions. in rare cases the atrophy may be extensive, and ulcerations may develop (lichen planus atropicus et exulcerans) that persist for years, with carcinoma developing in the ulcers erosive and bullous oral lesions of lichen planus. these may be difficult to diagnose when the classic lacy network of white lessions is absent. the erosive lession occur with some frequency, especially on the sides or dorsum of the tongue and on the buccal mucosa, where they are extremely painful, but the diagnosis can be confirmed only by histologic examination. shklar describes the histologic features are hyperkeratosis or parakeratosis, infiltration of the upper corium with a broad band of lymphocytes, and liquefaction degeneration of the basal layer.in the differential diagnosis pemphigus vulgaris, lupus erythematosus, mucous membrane (cicatricial) pemphigoid and erythema multiforme must be considered. herpetic stomatitis and herpes zoster should also be considered. ulcerative lichen planus. cram and his associates described an unusual syndrome of lichen planus confined to the feet and toes and manifested by bullae and ulceration, permanent loss of the toe-nails and cicatricial alopecia of the scalp. there was also involvement of the buccal mucosa. the chronic ulceration on the feet were painful and disabling. in two of their patients skin grafting of the soles produced succesful results. they reviewed the literature and found nine similar cases, mostly in women. crotty et al reported two similar case from thr mayo clinic in 1980, and zijdenbos et al another in 1985. hepatitis - associated lichen planus. primary billiary cirrhosis and lichen planus may coexist, as reported by powell et al and graham-brown et al. patients with this liver abnormality have marked propensity to develop a lichenoid eruption while on d-peniciliamine therapy rebora et al have reported that nine of 11 patients with erosive lichen planus and 13,5 % of ther patients with nonerosive lichen planus had evidence of chronic active hepatitis with negative hepatitis b surface antigen studies. korkij et al found a similar incidence of documented liver disease, significantly higher than their control population. two other studies have not confirmed these findings. one should be receptive to signs or symptoms of liver disease when evaluating patients with lichen planus lichen planus - lupus erythematosus overlap. over 20 cases have been reported of discoid lesions with central atrophy and hypopigmentation outlined by a reddish purple periphery, which are fixed, have a predilection for acral areas, and are poorly responsive to conventional therapy. histologically and immunopathologically the diagnosis of lichen planus is supported. often there are coexistent findings to support the diagnosis of lupus erythematosus lichen planus erythematosus. this rare variant of lichen planus was originally described by crocker in 1891. the deep crimson-colored papules are soft to the touch and are more erythematous than the

ordinary lichen planus. the individual lesion vary from 5 to 10 mm in diameter and blanch on pressure; they are discrete and occur singly on the trunk. mucosal and nail changes are usually absent. tanenbaum et al reported one case, as did one of us (HLA) follicular lichen planus ( lichen planopilaris). follicular lichen planus produces a patchy eruption of spiny follicular papules, indistinguishable from those of lichen spinulosus. these may be mixed with flat papules. sometimes the projecting horny filament are extremely delicate. eruption is usually not pruritic. follicular lichen planus must be differentiated from darier's disease, keratosis pilaris, and lichen scrofulosorum. this type of lichen planus occurs mostly on the scalp, with scarring alopecia ensuing in the affected areas. this produces irregularly shaped bald patches that usually persist permanently graham little-piccardi-lassuer syndrome. this syndrome is characterized by patchy cicatricial alopecia of the scalp and by patches of follicular spinous papules involving the trunk, the upper part of the arms and legs and the scalp. there may be typical oral or cutaneous lichen planus. in addition, there may be patches of alopecia ( noncicatricial - appearing ) in the axillae and pubic areas. this syndrome with its many variants has received innumerable names, but there is agreement that is is a variant of follicular lichen planus. horn et al reported a case of typical lichen planopilaris and a case of graham little-piccardi-lassueur syndrome in which the clinical overlap and similarity of immunofluorescence finding supported the concept that both conditions are follicular variants of lichen planus annular lichen planus. most frequently annular configurations of lichen planus are located on the penis or lips, although they may be found scattered on the skin or more frequently in the mouth. the ringed edges are composed of small papules. the rings are often about 1 cm in diameter. they may coalesce to form polyciclic figures. less often annular lesions may result from central atrophy in a flat papule. lichen planus tropicus ( subtropicus ). this condition has been variously named lichen planus actinicus, actinic lichen planus, and summertime actinic lichenoid eruption. el-zawahry has called attention to this variety of lichen planus, which favors the sun exposed parts of the body such as face ( forehead, cheeks, and lips ), the V area of the chest, the backs of the hands, and the lower forearms. the extremely pruritic lesions are typical lichen planus papules, which frequently show annular configuration and the distinctive violance coloration. Lichen planus tropicus is elicite by long exposure to sun, particularly in the cases of adolescent and young adults who work all day in the fields. El-zawahry estimates that 40% of all cases of lichen planus in the united arab republic are of this variety. Constant exposure to heat also promotes the development of these lesions. 16 cases were reported by salman et al in1989 in young adult in Lebanon; two had deposits of mucin in the reticular dermis. They estimated these cases to account for 14 % of the cases they saw. Isaacson et al reported a case from the united states and van der schroeff documented one from the netherland. Both were succesfully induced by phototesting with UVB.

Lichen planus pemphigoides. These patient usually have typical lichen planus lesions that, two to six weeks later, erupt with bullae, which also may arise on normal skin. These cases should be separated from those patients in whom blisters erupt only within lesions of lichen planus, as blisters occure at times in lesions with severe inflammation. The histopathology of the bullae may be that seen with bullous pemphigoid, and the immunopathology may reveal IgG and C3 in a linear pattern at the basement membrane zone. These deposits were seen to be present in the lamina lucida in a case reported by hinter et al. Circulating anti-BMZ IgG antibodies may be present. In one case investigated by immunoprecipitation, bullous pemphigoid antigen was not identified. Whether this disease is coexixtent lichen planus and bullous pemphigoid, or lichen planus and another as yet to be identified bullous disorder, or ( as is most likely ) is heterogenous in nature, remains to be elucidated. Erythema dyschromicum variants. Hermann pinkus suggested that erythema dyscromicum perstant (EDP) was one of gourgerots lichens atypiques ou invisible revealed by pigmentation, and Naidorf and Cohen reported the case of a woman who had EDP for two years before developing annular lichen planus with pigmentation Etiology. The cause of lichen planus remains unknown. Evidence points to the possibility that an alteration of epidermical cell antigens induces a cell mediated immune response similar to that seen in graft versus host disease. The type of infiltrate seen and its association with primary biliary cirrhosis and graft versus host disease support this. Several recent report document that lichen planus may be familial, and may occur in monozygotic twins. These familial cases occur at an early age and are chronic. Drugs may induce lichenoid reaction. Medications commonly associated with the development of this type of response are penicillamine, the antimalarials, arsenic, gold, quinidine, thiazide derivatives, methyldopa, clorpropamide, and penthothiazine derivatives. Propanolol, spironolactone, and naproxen are other drugs recently described to have induced this reaction. Incidence and course. Lichen planus is rare in children. Any that occurs is usually of the diffuse widespread variety. The familial cases are diffuse, chronic, and severe. The eruptive cases that occur in adults tend to invollute after several months to a year. Oral lesions, especially of the ulcerative variety, are chronic with little tendency for remission. They hypertrophic and linear varieties may persist indefinitely in spite of therapeutic measures. Histology. The histologic features of lichen planus are distinctive. There is a saw-tooth pattern of epidermal hyperplasia together with orthokeratosis and hypergranulosis. There is vacuolar alteration of the basal layer of the epidermis. Necrotic keratinocytes are present in the basal layer. In the superficial dermis there is a dense, bandlike infiltrate composed of lymphocytes, histiocytes, and melanophages. civatte bodies often present in the infiltrate, represent necrotic keratinocytes that have descended into the dermis. Drug induced lichenoid eruptions may have considerable number of eosinophils in the

infiltrate. Hypertrophic lichen planus shows the histologic features of lichen simplex chronicus superimposed upon those of lichen planus. Pinkus (1973) has described the lichenoid tissue reaction in detail, and his classic article is worth studying. On direct immunofluorescence, clumps of IgM and less frequently IgA, IgG and C3 are commonly present subepidermally, corresponding to the colloid bodies. These do not occur in oral lesions, but instead oral biopsies show a fibrillar band of fibrinogen at the basement membrane zone. Lichen planopilaris and graham little-piccardi-lassueur syndrome show finding similar to those of lichen planus, except that involvement in the superficial follicular epithelium. Duschet et al reported that 86 % of cases of lichen planus biopsied on the upper trunk had positive immunofluorescent findings, while only 50% of lesions on the lower extremities were positives. differential diagnosis. lichen planus has so many manifestations that it must be differentiated from a number of disease. scabies, pediculosis corporis, pityriasis rosea, psoriasis guttata, the small papular or lichenoid syphilid, and pityriasis lichenoides et varioliformisacuta are dermatoses that may resemble generalized lichen planus. mucous membrane lesions may be confuse with leukoplakia, lupus erithematosus, mucous patches of syphilis, candidiasis, cancer and traumatic stomatitis. on the scalp atrophic lesions may be mistaken for lupus erythematosus, folliculitis decalvans, and cicatrices. the follicular type must be differentiated from lichen spinulosus, which usually occurs only in children, so that the diagnosis of " lichen spinulosus " in an adult generally means lichen planus. in lichen spinulosus, horny filiform papules apear in crops in symmetrically arrange patches over the trunk and limbs. in keratosis pilaris, the onset is insidious, with horny follicular papules the color of normal skin or slightly gray occuring chiefly on the lateral aspects of the arm and thighs, without itching

the atrophyc type of lichen planus may be difficult to distinguish from lichen sclerosus et atrophicus and morphea guttata the hypertrophic type may simulate psoriasis, kaposi's multile hemorraghic sarcoma, and lichen amyloidosis. isolated patches of lichen planus may resemble neurodermatitis circumscripta or if heavily pigmented, may suggest a fixed drug eruption there is striking similarity between lichen planus and lichenoid dermatitis due to contact with color film developer chemicals. annular lichen planus, especially on the extremities may resemble granuloma annulare treatment. treatment is symptomatic and depend mostly upon the administration of corticosteroids. they should always be us intralesionally when feasible but of course in most cases lesions are either too larger or to numerous. topical steroids are rarely effective and though the newer high potency ones such as temovate might be effective, their potential for side effects is heightened too, and it would be safer to use intramuscularly injected triamcinolone, 60 mg in most situations. the potent steroids in

orabase may be useful for oral lesions. generally if lichen planus is eruptive or generalized, systemic steroids are needed. oral prednisone should be avoided if possible, because of side effects, but if unavoidable, a dose of 40 to 60 mg daily is suggested, tappering of over 2 to 3 months snyder et al reported a man with generalized lichen planus in whom low to intermediate dose oral and intramuscular steroids as well as griseofulvin and methotrexate had failed, but who responded well to intermitten megadose IV ("pulse") therapy with methyprednisolone, 1 gr daily for three days, each month for three months dapsone induced complete healing of oral and palmoplantar ulcerations in a case reported by falk et al, and isotrenitoin 10 mg a day, cleared a case of erosive oral lichen planus of 14 years duration reported by staus and bergfeld. woo also reported succes with oral isotrenitoin in two patients with erosive oral lesions. glutina and associates reported improvement from topical isotretinoin gel in 20 cases. mahrle and associates reported their experience with etretinate. twenty six patients wer given a starting dose of 1 mg/kg ( 75-100 mg) a day, tappered to a maintenance dose of 25 to 50 mg every other day after 2 to 5 weeks. all of four exanthematous eruptive patients responded. the overall response in classic lichen planus was 46 % " good to excellent "; it was less effective in cases with oral lesions. gonzalez et al have reported on the use of PUVA for lichen planus. 5 of 10 patients cleared completely and needed no maintenance treatment; three other improved at least 50%. two patients were made worse. paslin reported succes in three cases with cyclophospamide, 50 mg a day, after failure or transitory success with a variety of agents, including grenz radiation, chlorambucil, amitriptyline and others. resolution of lesions was complete in a few weeks and lasting. Metronidazole (flagyl) has been reported effective in 29 cases of erosive oral lichen planus, and shelley et al saw an initial eruption and two relapses of lichen planus respond to metronidazole ( given for resistant cystitis ) after seccessive two weeks trials of ampicilin, erythromycin, ketoconazole and cholestyramine had failed to help. They also saw it succeed dramatically in a nine years old boy who was given it for giardiasis; his hitherto unresponsive lichen planus cleared in two weeks. Tranquilizers and antipruritic drugs such as the antihistamines and cyproheptadine ( periactin ) may be helpfull in selected cases. Levy et al in israel reported complete healing in 80 to 90 % of 26 cases of lichen planus treated with griseofulvin, 125 mg twice daily for three to six months.