Indian Journal of Dermatology
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CASE REPORT
Year : 2005  |  Volume : 50  |  Issue : 4  |  Page : 224-226
Lichen planus pemphigoides in an indian female


Department of Dermatology & STD, LHMC and Associated SSKH and KSCH Hospitals New Delhi., India

Correspondence Address:
Vibhu Mendiratta
Department of Dermatology & STD, LHMC and Associated SSKH and KSCH Hospitals New Delhi.
India
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Source of Support: None, Conflict of Interest: None


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   Abstract 

Lichen planus pemphigoides is a rare, controversial disease which is thought by majority to be a concurrence of bullous pemphigoid (B P) and lichen planus (LP) with clinical, histological and immunological features suggestive of both. We describe LP pemphigoides in an Indian female who had classical LP with subsequent short lived bullous eruption over the lesions of LP and the normal skin. DIF of the bulla showed C3 and lgG along the basement membrane zone ( BMZ) linearly. She responded successfully to systemic steroids (short course of 6 wks prednisolone 40 mg/day for 6 weeks) and dapsone 100mg per day.


Keywords: Lichen planus pemphigoides , Treatment (corticosteroides/dapsone)


How to cite this article:
Mendiratta V, Asati DP, Koranne R V. Lichen planus pemphigoides in an indian female. Indian J Dermatol 2005;50:224-6

How to cite this URL:
Mendiratta V, Asati DP, Koranne R V. Lichen planus pemphigoides in an indian female. Indian J Dermatol [serial online] 2005 [cited 2020 Aug 5];50:224-6. Available from: http://www.e-ijd.org/text.asp?2005/50/4/224/19750



   Introduction Top


The earliest description of a combination of a case of typical lichen planus (LP) complicated by a widespread bullous eruption was by Kaposi, who coined the term "Lichen ruber pemphigoides" in 1892.[1] The nosological status of LP pemphigoides has been speculated to be as one of the coexistence of lichen planus and bullous pemphigoides (BP)[2], a variant of LP[3], a coincidental existence of LP and another autoimmune bullous dermatitis (i.e, dermatitis herpetiformis)[4] or an immunologically independent disease[5].

Clinically it is characterized by the presence of bullous lesions over the lesions of lichen planus as well as the normal skin (the later being more common). A typical case of LP is characterized by an initial phase of typical lichen planus lesions often having severe pruritus and there also may be patches of erythema with sudden eruption of tense bullae commonly on the extremities and over the normal skin ,erythematous patches, or pre-existing papules. Histopathologically the lesions of lichen planus show a band like lymphohistiocytic infiltrate, Civatte bodies in dermis, irregular acanthosis with saw toothing, orthokeratosis and hypergranulosis; with direct immunofluorescence showing predominantly IgM and C3 deposited on colloid bodies, fibrin and fibrinogen. The histopathology of the bullous lesion in LP shows a subepidermal bulla with variable infiltrate that may be diffuse or sparse lymphohistiocytic infiltrate with or without neutrophils and numerous eosinophils in upper dermis .Presence of C3 alone or with IgG along the dermoepidermal junction is confirmatory on DIF[6]. In the rare clinical presentations of this controversial disease the bullae may be seen only on the lesions of the LP.[7] There could be a lichenoid erythrodermic presentation of a bullous eruption.[8]

Controversy continues to shroud the true status of LP pemphigoides as regards it being a distinct disease entity[2],[8] or a coincidence of LP pemphigoides and BP. Majority of the patients of LP pemphigoides have been reported to have a 180 kd antigen identified by an immunoblotting technique. Similar to BP antigen but a novel 200kd antigen; not yet found in BP has also been identified in one patient,[9] which perhaps favours the distinct disease process of LP pemphigoides.

The mean age of LP pemphigoides has been described to be 35yrs (range14-60 yrs), with a variable course, tending to be less severe than bullous pemphigoid with short lived blistering which may abort spontaneously or with systemic steroids[2] and azathioprine[10] and the LP lesions may continue as such. A severe blistering with fatal outcome is rare. Since, LP permphigoides is a rare controversial disease entity and requires further immunological evaluation. We report an Indian female with typical LP who was successfully managed with short course of systemic steroid and dapsone.


   Case report Top


A 30 year old housewife presented in the OPD with multiple, itchy, violaceous, flat topped and raised lesions over legs (shins),back and arms bilaterally for last two months. She noticed multiple fluid filled lesions over the arms superimposed on the pre-existing lesions and over the normal skin of forearms, abdomen and shins. There was no preceding history of any drug intake/fever/jaundice/blood transfusion. She had received doxycycline/ metronidazole for her pelvic infection a couple of months prior to the development of the itchy violaceous lesions. Examination revealed presence of multiple polygonal, flat topped, scaly violaceous papules and plaques measuring 5cm by 5cm present over anterior aspect of legs, forearms, abdomen, back and the trunk; sparing the face, palms and soles. The mucosae, nails and the scalp were unaffected. Along with these lesions, there were multiple vesicles and tense bullae (15-20 in number)present over the preexisting lesions and the normal skin over flexor aspect of the forearms, abdomen; [Figure - 2] some with hemorrhagic fluid and the blister roof setteling down like a graft. Both the bulla spread sign and the Nikolsky sign were negative. Tzanck smear from the bulla showed mixed infiltrate of polymorphs and eosinophils with absence of giant cells/acantholytic cells. Histopathological examination from the violaceous plaque was consistent with lichen planus and from the intact bulla showed a supepidermal bulla with many eosinophils ,some polymorphs and a perivascular lymphocytic infiltrate in the dermis. Direct immunoflouresence revealed linear deposits of IgG with complement C3 at dermo-epidermal junction. Both indirect immunoflouresence and immunoblotting could not be performed because of non-availability.

A final diagnosis of lichen planus pemphigoides was made and the patient was given oral prednisolone (40mg/day). All the existing bullae healed within 10 days and there was no fresh bullae,then it was tapered over a month and dapsone was added in the dose of 100mg/day.In the follow up lasting six month the patient had no recurrence of the bullous lesions. The lichen planus lesions have also regressed with residual hyperpigmentation.


   Discussion Top


Lichen planus pemphigoides is accepted to be the co-existence of two separate diseases, lichen planus and bullous pemphoid by the majority of the workers all over the world but at the same time there are convincing reports which identify a separaste 200kd Ag by immunoblotting in a patient of LP pemphigoides.[9]

The case reported by us is a typical case of LP pemphigoides in all respects including the classical clinical features in the form of sudden eruption of tense bullae over the pre-existing lichen planus lesions and the normal skin in association with severe itching involving the extremities. There were no mucosal lesions. Histopathgologically a confirmation of both classical LP (from the LP papule) and the BP (from the tense bulla over the normal skin) was established which was finally corroborated by performing DIF on the bulla. Immunoblotting could not be undertaken due to non-availability.

The clinical course of the blistering was also short lived and all the bullae responded very well to systemic steroids (40mg/day tapered over 6 wks) with the residual lesions of LP being treated by dapsone (100mg/day).

Since LP preceded the bullous eruption in our case we feel that the basal cell damage due to the LP might have exposed or released BMZ antigens which resulted in autoantibodies formation against the BMZ, consequently causing a co-existent bullous pemphigoid (BP) like disease. With the application of immunoblotting / immunoprecipitation technique, the final status of LP pemphigoides would be clarified further by correlating it with the clinical, histopathological and immunological details of more cases[11].

 
   References Top

1.Kaposi M. Lichen ruber pemphigoides. Arch Derm Syph (Berlin) 1892; 343-6.   Back to cited text no. 1    
2.Mora RG, Nesbit LT, Brantly JB, Lichen planus pemphigoides: Clinical and immunofluorescent findings in four cases. J Am Acad Dermatol 1983;3 :331-6.   Back to cited text no. 2    
3.Souteyrond P,Pierini AM,Bussy RF, et al . Lichen planus pemphigoides :Entity or association? Dermatologica 1981; 1621 :414-6.   Back to cited text no. 3    
4.Stingel O,Holuber K.Coexistence of Lichen planus and Bullous pemphigoid.Br J Derm.1975;93: 313-20.  Back to cited text no. 4    
5.Sobels, MillerR, Shatin H.Lichen planus pemphigoides: Immunoflourescence.Arch Dermatol 1976;112:1280-3.   Back to cited text no. 5    
6.Okochi H, Nashiro K,Tsuchida T, et al .Lichen planus pemphigoides:Case reports and results of immunoflourescence and immunoelectron microscopic study. J Am Acad Dermatol 1990;22:626-31.   Back to cited text no. 6    
7.Archer CB, Cronin E, Smith NP. Lichen planus pemphigoides: case presentation at the Royal Society of Medicine .Dermatology Section;Nov I988.   Back to cited text no. 7    
8.Joly P, Tanasescu S,Wolkenstein P, Bocqet H, et al . Lichenoid erythrodermic bullous pemphigoid of the African patient. JAm Acad Dermatol 1998;39:691-7.   Back to cited text no. 8    
9.Davis A,Wojnarowsha F, Bhogal B, et al .Lichen planus pemphigoides and its relationship to bullous pemphigoid.Br J Dermatol 1989; 120: 296.   Back to cited text no. 9    
10.Long G Pearson, Maize C John, Charleston SC.Coexisting Lichen planus and Bullous pemphigoid or Lichen planus pemphigoides. J Am Acad Dermatol 1983;9: 133-40.   Back to cited text no. 10    
11.Mrphy GM, Cronin E. Lichen planus pemphigoides. Clin and Exp Dermatol 1989; 14;322-4.  Back to cited text no. 11    


Figures

[Figure - 1], [Figure - 2]



 

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