Blp is commonly associated with burning sensation and pain. Familial bullous lichen planus fblp is a rare condition. Childhood bullous lichen planus, pediatric dermatology. Bullous lichen planus is a form of lichen planus in which bullae blisters accompany the lichenoid papules and plaques and i occasionally encounter this version in my the woodlands dermatology and conroe dermatology offices. It is thought to be the result of an autoimmune process with an unknown initial trigger.
Lichen planus pemphigoides is a rare condition characterized by blisters arising on normal or erythematous skin in a patient with concurrent lichen planus. Clinical features white papules that usually coalesce, forming a network of lines wickmans striae, are the characteristic oral lesions of the disease. It must be distinguished from bullous lichen planus, in which, as a consequence of severe basal cell hydropic degeneration, blisters arise within lichenoid papules or plaques. Childhood bullous lichen planus, pediatric dermatology 10. Lichen planus pemphigoides lpp is a very rare autoimmune subepidermal blistering disease associated with lichenoid skin changes. No systemic abnormalities are revealed by physical. In cases of oral lichen planus, the mucous membranes inside the mouth are the only area affected. Several therapies have been tried, with varying results.
Bullous lichen planus in this bullse are situated on the voilaceous base resembling lichen planus. Compared with non familial bullous lichen planus, it has an earlier onset and wider disease distribution. We present the case of a 25yearold pregnant woman at 12 weeks gestation who developed an acute bullous eruption after 5 months of worsening lp. A child with previous lichenoid lesions had bullae refractory to antistaphylococcal antibiotics. Lichen planus lp is a chronic inflammatory and immune mediated disease that affects the skin, nails, hair, and mucous membranes. Like other forms of lichen planus, it is characterized by a skin rash shiny, flattopped, firm bumps that are a purple color and vary from pin point size to larger than a centimeter. There is no similar condition in the patients family. Autoantibodies in lichen planus pemphigoides react with a. Bullous lichen planus of the nails view large download a, before treatment, swelling and violaceous discoloration are apparent on the proximal fingernail folds along with oozing of blood, hemorrhagic crusting of the fingernails, and. Indirect immunofluorescence using the patients serum on a perilestional bulla biopsy showed positive staining for iga and igg in the stratum granulosum and stratum corneum, confirming the diagnosis. Most patients with blp are associated with multifocal involvement and skin lesions. We showed that oral hygiene measures and conventional periodontal treatment and strict maintenance were sufficient to control the gingival involvement of olp.
Lichen planus lesions are described using the six ps. Presence of bullae is seen in association with the white striae. Lpp patient 1 p1 showed lichenoid striae of the buccal. The cause of lichen planus is unknown but it is most likely a tcellmediated immunological. Lichen planus lp is derived from the greek leichen meaning tree moss and the latin planus meaning flat lichens are primitive plants composed of symbiotic algae and fungi planus in latin for flat. This chapters discusses the typical features of lp as well as the lp variants, it does not discuss follicular lp syn. Lichen planus pemphigoides is an autoimmune subepidermal blistering disease. Lichen planus bullous lichen planus perri dermatology. Definition lichen planus is a relatively common chronic inflammatory disease of the oral mucosa and skin etiology although the cause is not well known, t cellmediated autoimmune phenomena are involved in the pathogenesis.
It is characterized by vesicles or bullae, which usually develop in the context of preexisting lp lesions. The bullous form of lichen planus is rarely described with antipd1, with are only six published cases medline search conducted through pubmed. Diffuse lichen planuslike keratoses and clinical pseudo. The initial clinical presentation is either that of plaques subsequently becoming bullous 3, 7, as in our patient, or else presence of vesicles or bullae from the outset. To my knowledge, no similar cases have been presented in the american literature. Lichen planus pemphigoides genetic and rare diseases. The finding of immunoglobulin g antibodies directed against the basement membrane zone differentiates it from bullous lichen planus. Shah p, shah m, parikh k, khan f 2010 oral lichen planus. Lichen planus pemphigoides lpp is a rare crossover syndrome between lichen planus and bullous pemphigoid. We report the largest patient series of fblp and describe its clinical characteristics and inheritance pattern.
Occasionally, lichen planus lp, a common chronic inflammatory skin disorder of unknown etiology transforms into a bullous phenotype reminiscent of bullous pemphigoid bp and is associated with igg autoantibodies against bp180. Biopsy of the bullous lesion was diagnostic of bullous lichen planus. Full text pdf abstract lichen planus is a chronic mucocutaneous. Subsequently, a biopsy was performed from a bulla, and tissuefixed antibasement zone antibody was also demonstrated. Bullous lichen planusin this bullse are situated on the voilaceous base resembling lichen planus. Lichen planus lichen planus lp is a disease of the skin andor mucous membranes that resembles lichens. Childhood bullous lichen planus biopsy of a bulla showed histologic changes of bullous lichen planus. Bullous lichen planus blp is relatively a rare variant of lichen planus. Lichen planus is a chronic, inflammatory, autoimmune disease that affects the skin, oral mucosa, genital mucosa, scalp, and nails.
The worldwide prevalence of the disease is estimated to be 1%. Lichen planus pemphigoides lpp is a rare condition characterized by tense blisters that arise on lesions of lichen planus lp and on unaffected skin. Sep 10, 2015 lichen planus pemphigoides lpp is a rare crossover syndrome between lichen planus and bullous pemphigoid. Childhood bullous lichen planus childhood bullous lichen planus kwee, darlene j dufresne, raymond g ellis, darrel l. Lichen planus has been found to be associated with hepatitis c virus infection, chronic active hepatitis, and primary biliary cirrhosis. We are now following up a 64yearold black woman who was first seen in early july 1975 with a one months history of a pruritic. Bullous lichen planus is a rare variant of lichen planus.
Two weeks after the first avelumab infusion mcc lesions were inflamed and slightly enlarged, consistent with pseudoprogression of malignancy. Bullous lichen planus of the nails dermatology jama. In most patients lichen planus will heal within 18 months, and not return, although some patients. Lichen planus is a cellmediated immune response of unknown origin. In a recent article in the british journal of dermatology, 1 a patient was described with both lichen planus and bullous pemphigoid. Lichen planus is a chronic, inflammatory, autoimmune disease 1 occurring in 0. Otherwise its mostly an autoimmune disorder or a reaction to certain painkillers, antibiotics, or metal dental fillings. Aug 31, 2019 lichen planus lp is a pruritic, papular eruption characterised by its violaceous colour and polygonal shape, sometimes with a fine scale. Two months upon the completion of therapy, there were no signs of relapse. Jul 01, 2011 lichen planus is a chronic, inflammatory, autoimmune disease1 occurring in 0. Bullous lichen planus verma r, vasudevan b, kinra p. Limited information is available regarding the management of bullous oral lichen planus. There is one case report in which aloe vera av was used successfully in the treatment of lichen planus.
If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126. Bullous lichen planus accompanied by elevation of serum antibp180 autoantibody. Clinically, at times blp can be presented as erosive lichen. Bullous lichen planus may be confused with other subepidermal bullous dermatoses, especially if bullae arise on normalappearing skin. It is characterized by polygonal, flattopped, violaceous papules and plaques with overlying, reticulated, fine white scale wickhams striae, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. Where the trigger is known, a lesion is known as a lichenoid. The medical term lichen refers to small bumps on the skin and the term planus means flat, together they refer to the characteristic flat topped papules of lichen planus lp. Oral lichen planus is a chronic inflammatory mucocutaneous disorder that is tcell mediated. Etiology of periodontal diseases part 4 provides a more comprehensive background in periodontal anatomy, physiology, and pathogenesis. Request pdf bullous lichen planus of the nails nail involvement may occur in 1% to 10% cases of lichen planus lp and mostly in the setting of widespread cutaneous disease longitudinal. Anyone with a rash with red, purple or bluish lesions that itches and spreads should listen up.
Nivolumabinduced lichen planus pemphigoides mdedge. Indirect immunofluorescence using the patients serum on a. In most patients lichen planus will heal within 18 months, and not return, although some patients may have a second episode many years later. Lichen planus is a chronic systemic disease of established immunemediated pathogenesis. Clinical appearance of tumor and lichen planus like keratoses lplk in a patient with merkel cell carcinoma mcc. Lichen planus and bullous pemphigoid jama dermatology. It has been suggested that true lichen planus may respond to stress, where lesions may present on the mucosa or skin during times of stress in those with the disease. Hair growth is usually abnormala short and twisted, with longitudinal fissuring and fracturing, and only one knot located near the free end of the hair. Commonly seen variant of oral lichen planus is reticular type whereas erosive type of lichen planus is the.
We present two patients who were originally thought, on the basis of clinical and histopathologic criteria, to have bullous pemphigoid. Lichen planus pemphigoides associated with pregnancy. A rare form of the disease, familial bullous lichen planus, is inherited. Lichen planus pemphigoides lpp is characterized by tense blisters that arise not only on lichen planus lesions such as bullous lichen planus but also on skin unaffected by lichen planus.
Biopsy during a punch biopsy test there is removal of small section of skin which is then examined under a microscope for cell patterns characterstics of lichen planus. The documents contained in this web site are presented for information purposes only. Lichen planus is a skin rash caused by an immune response. Of 303 patients diagnosed with oral lichen planus, 58 19.
It is often misdiagnosed and should be differentiated from other subepidermal bullous diseases especially lichen planus pemphigoides. This disease is triggered by the immune system in response to stress, allergens or a viral infection. We present a case of a 77yearold female who suffered from oral lichen planus olp involving her gingiva and bilateral buccal mucosa for over 6 months. Diagnosis and treatment of lichen planus american family. It is often misdiagnosed and should be differentiated from other subepidermal bullous diseases especially lichen. Lichen planus is a fairly common, itchy, noninfectious rash that usually occurs in adults. Rhodus nl, myers s, kaimal s 2003 diagnosis and management of oral lichen planus. It most commonly, protractedly and persistently, involves the mucosa of the oral cavity, but it can involve other sites, namely the skin, the scalp with inflammation around and affecting the hair follicles resulting in alopecia, the nails as well as the genital area the vulval and vaginal mucosa. In our case, short course of systemic corticosteroids and ultraviolet b phototherapy have been safe and effective. Indirect immunofluorescence using the patients serum on a perilestional bulla biopsy showed positive staining for iga and igg in the stratum granulosum and stratum corneum, confirming the. See reference oral lichen planus olp is a chronic inflammatory disease that can be painful especially in the atrophic and erosive forms. Lichen planus affects primarily middleaged adults, and the prevalence is greater among women. Sera from lichen planus pemphigoides patients n 4 stained the epidermal side of. Bullous and hemorrhagic lichen sclerosuscase report.
Bullous lichen planus and antiprogrammed cell death1. In lpp, the autoantibodies specifically target the mcw4 epitope of the nc16a4 domain of the bullous pemphigoid antigen bpag2, distinguishing it from other. It may affect the skin, scalp and nails, as well as the mouth and the genitals. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly bullous pemphigoid bp or lichen planus lp, a growing body of evidence suggests that it is a disease entity in its own right. Request pdf bullous lichen planus of the nails nail involvement may occur in 1% to 10% cases of lichen planus lp and mostly in the setting of widespread cutaneous disease.
Immunofluorescence testing in the diagnosis of autoimmune blistering diseases. Bullous lichen planus of the nails view large download a, before treatment, swelling and violaceous discoloration are apparent on the proximal fingernail folds along with oozing of blood, hemorrhagic crusting of the fingernails, and pus discharging from the lateral nail folds. Lichen planus new breakthrough treatment that works, award. American autoimmune related diseases association aarda. Term suggests flat fungal condition current evidence indicates immunologicaly mediated mucocutaneous disorder. Topical and oral corticosteroids form the mainstay of therapy and dapsone has been tried with variable results. Bullous form of lichen planus is seen in 116% of all children with lichen planus. Lichen planus lp is a chronic inflammatory dermatosis involving mucocutaneous surfaces and nails. It is most often found on the flexor surfaces of the upper extremities, genitalia and on the mucous membranes.
It may be inherited as an autosomal dominant condition with variable penetrance. Lichen planus has been reported as a complication of chronic hepatitis c virus infection and can be a sign of chronic graftversushost disease of the skin. We here studied three female patients with lpp with regard to peripheral blood t cell recognition of bp180. The aim of this study was to identify the target antigen of lichen planus pemphigoides autoantibodies. The mechanism of olp is complex and not yet fully understood. Ppt lichen planus lp powerpoint presentation free to. Prevalence of oral lichen planus in indian population is 2.
Familial bullous lichen planus is a chronic, progressive bullous eruption of the lower and upper extremities. Childhood bullous lichen planus kwee 1987 pediatric. Bullous lichen planus in childhood a case report bulozni. Biopsy of a bulla showed histologic changes of bullous lichen planus. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
The patients serum at that time was positive for circulating antibasement membrane antibody in a 1. Lichen planus new breakthrough treatment that works. It may be found with other diseases of altered immunity, such as ulcerative colitis, lichen sclerosis, myasthenia gravis etc. Women have higher predisposition to oral lichen planus and the condition is seen at the peak age of 3060 years.
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