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IJD FOCUS: LICHEN PLANUS
Year : 2015  |  Volume : 60  |  Issue : 5  |  Page : 457-460
Concurrent presentation of erythrodermic lichen planus and squamous cell carcinoma: Coincidence or malignant transformation?


Department of Dermatology, Fr. Muller Medical College, Mangalore, Karnataka, India

Date of Web Publication4-Sep-2015

Correspondence Address:
Neema M Ali
Flat no: 402, Sundari Apartments, Shivabagh, Kadri, Mangalore - 575 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.164355

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   Abstract 

Lichen planus is a common papulosquamous disorder affecting about 1-2% of the population, neoplastic transformation of cutaneous lichen planus lesions occurs very rarely. A 40 year old female patient presented with a 1 year history of developing multiple, itchy, pigmented lesions over both lower legs which gradually spread to involve the whole body. A few tense bullae were seen on the extremities. An erythematous fleshy lesion was seen on the upper aspect of the left buttock. Skin biopsy from a plaque on the right forearm showed features suggestive of lichen planus. Skin biopsy of a bullae showed a sub epidermal bulla filled with a mixed inflammatory infiltrate. Direct immunofluorescence revealed no immunoreactants along the basement membrane zone. A diagnosis of erythrodermic lichen planus with bullous lichen planus was made. Biopsy of fleshy lesion of left buttock revealed a moderately differentiated squamous cell carcinoma. Erythrodermic lichen planus with bullous lesions and secondary squamous cell carcinoma; these occurences in a single patient is extremely rare and has not been previously reported to the best of our knowledge.


Keywords: Lichen planus, erythroderma, squamous cell carcinoma


How to cite this article:
Ali NM, Bhat R, Rao SB. Concurrent presentation of erythrodermic lichen planus and squamous cell carcinoma: Coincidence or malignant transformation?. Indian J Dermatol 2015;60:457-60

How to cite this URL:
Ali NM, Bhat R, Rao SB. Concurrent presentation of erythrodermic lichen planus and squamous cell carcinoma: Coincidence or malignant transformation?. Indian J Dermatol [serial online] 2015 [cited 2019 Sep 19];60:457-60. Available from: http://www.e-ijd.org/text.asp?2015/60/5/457/164355

What was known?
Lichen planus is a common papulosquamous disorder which has several clinical types of which bullous lichen planus and erythrodermic lichen planus are rare. Neoplastic transformation of oral lichen planus is frequently documented but that of cutaneous lichen planus is rare.



   Introduction Top


Lichen planus is a benign disorder characterized by an itchy, noninfectious skin rash. Though lichen planus is a common papulosquamous disorder affecting about 1-2% of the population, neoplastic transformation of cutaneous lichen planus lesions occurs very rarely. [1] Various clinical variants of lichen planus include acute eruptive, hypertrophic, atrophic, bullous, actinic, and oral. Although lichen planus has been mentioned in the standard textbooks of dermatology as a cause of erythroderma, one hardly encounters such presentation in clinical practice. [2] Bullous lichen planus is a rare variant, which usually presents with blisters occurring over typical lesions of lichen planus. It is to be differentiated from lichen planus pemphigoides in which the blisters are more generalized and extensive and the course is prolonged. [3] This case is being reported because of the simultaneous occurrence of three rare phenomena in lichen planus, which are erythroderma, bullous lichen planus, and squamous cell carcinoma in the same patient. To the best of our knowledge, a similar case has not been reported previously, which makes it truly unique. If this is a case of malignant transformation of erythrodermic lichen planus, it makes it an even rarer case as a wide search of literature showed there was no previous case reports of similar nature.


   Case Report Top


A 40-year-old female patient presented with a 1 year history of developing multiple, itchy, pigmented lesions over both lower legs which gradually spread to involve the face, trunk and extremities over a period of 5 months. There was no history of remission of lesions during the past 1 year. She gave a history of exacerbation of lesions since the past 5 days with development of blisters on the existing lesions on the lower limbs and back. There was also a history of a fleshy lesion on her left buttock, which developed on a hyperpigmented plaque and was insidiously increasing in size since the past 3 months. No drugs were taken orally prior to the development of the eruption but there is a history of application of ayurvedic medicine. No similar illness was present in any of the family members. The patient was a known diabetic on irregular treatment. Cutaneous examination revealed erythroderma with hyperpigmented, violaceous, scaly, discrete and confluent plaques over extremities, trunk and back with relating sparing of the flexural areas [Figure 1]. There was also erythema and diffuse violaceous pigmentation of the face. A few tense bullae were seen on the extremities. A few of them had turbid fluid. Nicolski sign and bullae spread sign were negative. These bullae were present only on the existing violaceous lesions and not on the normal skin. An erythematous fleshy plaque measuring 5 × 4 was seen on the upper aspect of the left buttock [Figure 3]. It was not ulcerated or crusted and was non-tender on palpation. The palms and soles also had violaceous scaly plaques. The oral mucosa had diffuse pigmentation of inner aspect of both cheeks but had no whitish plaques. The genital mucosae, nails and hair were normal. Generalized non-tender lymphadenopathy was present. Systemic examination revealed no other abnormality. Routine blood and urine analysis revealed uncontrolled diabetes. A clinical differential diagnosis of erythroderma secondary to lichen planus, lichen planus pemphigoides or allergic contact dermatitis was considered.
Figure 1: Generalised involvement of trunk

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Figure 2: Biopsy from a bullous lesion showing subepidermal bullae and changes of lichen planus like orthokeratosis, basal cell degeneration and pigment incontinence. (H and E stain, ×40)

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Figure 3: Fleshy lesion seen on the left buttock

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A skin biopsy from a plaque on the right forearm showed eczematous changes and was inconclusive. A skin biopsy of a bulla along with adjacent perilesional skin showed sub-epidermal bulla filled with a mixed inflammatory infiltrate. The adjacent epidermis showed parakeratosis, irregular acanthosis, and basal vacuolar degeneration with other features of lichen planus [Figure 2]. A differential diagnosis of bullous lichen planus and lichen planus pemphigoides was considered. Direct immunofluorescence revealed no immunoreactants along the basement membrane zone. Hence, a diagnosis of erythrodermic lichen planus with bullous lichen planus was made. A biopsy of the fleshy lesion of the left buttock revealed a moderately differentiated squamous cell carcinoma [Figure 4]. FNAC of adjacent lymph nodes ruled out malignant changes.
Figure 4: Biopsy from a lesion on the left buttock shows tumor cells infiltrating the sub-epithelia in nests and sheets. (H and E stain, ×40)

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The patient could not be treated with corticosteroids due to her high blood sugar levels. She was treated with dapsone 100 mg, azathioprine 50 mg, antihistamines and topical steroids. The patient was started on insulin injections for diabetic control. She showed improvement of skin lesions on a 3-week follow up. A wide excision was advised for the squamous cell carcinoma, but the patient was unwilling for the procedure and was discharged against medical advice. She was then lost to follow up.


   Discussion Top


Lichen planus (LP) is a relatively common, chronic, papulosquamous disorder that is characterized by polygonal, pruritic papules and erosive lesions of the oral mucosa. Although the etiology of LP has yet to be fully elucidated, it is thought to be related to autoimmunity and is associated with HLA types-A3, -A5, -B8, -Bw35, and -DR1. Lichen planus is variable in its clinical presentation and natural history, with several well-characterized subtypes, such as eruptive, hypertrophic, atrophic, bullous, actinic, and oral. [4]

Erythrodermic lichen planus is an extreme rarity. In a case series of 80 cases of erythroderma, only one case of erythroderma due to lichen planus was reported. [5] This patient presented with erythroderma and a diagnosis of lichen planus was considered due to the violaceous appearance of the lesions which was confirmed by histopathological findings. This patient presented with tense bullae on the pre-existing skin lesions. The bullae were not widespread and were absent from normal skin. We considered a diagnosis of either bullous lichen planus or lichen planus pemphigoides. However, the biopsy and direct immunofluorescence findings were suggestive of bullous lichen planus. Bullous lichen planus is commonly seen on the legs. Blisters are tense and may be multilocular. Bulla formation may be due to the extensive liquefaction and vacuolation of the basal layer. [3] The blister of bullous lichen planus is a subepidermal bulla showing degeneration of the epidermal basal layer and other features of lichen planus, whereas in lichen planus pemphigoides the bulla is similar to that of bullous pemphigoid with rather more neutrophils than are usually seen. Direct immunofluorescence is positive in lichen planus pemphigoides and negative in bullous lichen planus. Lichen planus pemphigoides and bullous lichen planus are separate entities: The former is an auto-immune disease precipitated by lichen planus and not related to bullous pemphigoid, the latter is probably not auto-immune but represents the extreme consequence of the lymphoid infiltrate at the epidermal junction. [6]

Our patient had developed cutaneous squamous cell carcinoma on her left buttock. Although the incidence of cancer in oral LP is about 1.3%, neoplastic transformation in cutaneous LP is very rare. [7] Cutaneous lichen planus does not carry an increased risk of malignant transformation and is not considered as a premalignant lesion unlike its oral counterpart. [1] Neoplastic transformation of lichen planus is a rare event. The underlying mechanisms of neoplastic changes in lichen planus are unknown. Chronic inflammatory processes with oncogenic growth factors may facilitate the development of cellular clones in the epidermis. [8] This patient had erythrodermic lichen planus, which is an extremely rare presentation. The presence of squamous cell carcinoma in a patient with erythrodermic lichen planus adds to the rarity of this case. The chronicity of lichen planus in this patient suggests a malignant transformation to squamous cell carcinoma, but a possibility of a concurrent occurrence of malignancy cannot be ruled out as we had no evidence of lichen planus and squamous cell carcinoma in the same slide. Most cutaneous squamous cell carcinomas are associated with risk factors like arsenic exposure, radiation exposure, chronic tar application, ultraviolet rays, burn scars, varicose ulcers, and human papilloma virus. Here in our case the associated risk factors are chronic irritation in the form of itching and long-standing non-healing lesions of lichen planus. [4] The incidence of squamous cell carcinomas complicating cutaneous lichen planus is 0.4% and most of the reported cases are of hypertrophic type. [9] Our case presented with erythrodermic lichen planus with bullous lesions and squamous cell carcinoma. These occurrences in a single patient are extremely rare and have not been previously reported to the best of our knowledge. Squamous cell carcinoma in lichen planus in this case could be either a coincidence or a malignant transformation. In either scenario, squamous cell carcinoma in erythrodermic lichen planus has not been reported so far.

 
   References Top

1.
Ghosh S, Kotne S, Ananda Rao PB, Turlapati SP, Kumar Soren D. Squamous cell carcinoma developing in a cutaneous lichen planus lesion: A rare case. Case Rep Dermatol Med 2014;2014:205638.  Back to cited text no. 1
    
2.
Gupta LK, Garg A, Khare AK, Mittal A. Lichen planus presenting as erythroderma. Indian J Dermatol Venereol Leprol 2012;78:409.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.
Verma R, Vasudevan B, Kinra P, Vijendran P, Badad A, Singh V. Bullous lichen planus. Indian J Dermatol Venereol Leprol 2014;80:279.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Rose AE, Patel U, Chu J, Patel R, Meehan S, Latkowski JA. Erythrodermic lichen planus. Dermatol Online J 2011;17:26.  Back to cited text no. 4
    
5.
Rym BM, Mourad M, Bechir Z, Dalendo E, Faika C, Ladh AM, et al. Erythroderma in adults: A report of 80 cases. Int J Dermatol 2005;44:731-5.  Back to cited text no. 5
    
6.
Gawkrodger DJ, Stavropoulos PG, McLaren KM, Buxton PK. Bullous lichen planus and lichen planus pemphigoides-clinico-pathological comparisons. Clin Exp Dermatol 1989;14:150-3.  Back to cited text no. 6
[PUBMED]    
7.
Sengupta S, Das JK, Gangopadhyay A. Malignant transformation of hypertrophic lichen planus. Indian J Dermatol Venereol Leprol 2006;72:470.  Back to cited text no. 7
  Medknow Journal  
8.
Okiyama N, Takahiro S, Hiroo Y, Kiyoshi N. Squamous cell carcinoma arising from lichen planus of nail matrix and nail bed. J Am Acad Dermatol, 2005;53:908-9.  Back to cited text no. 8
    
9.
Singh SK, Saikia UN, Ajith C, Kumar B. Squamous cell carcinoma arising from hypertrophic lichen planus. J Eur Acad Dermatol Venereol 2006;20:745-7.  Back to cited text no. 9
[PUBMED]    

What is new?
Erythrodermic lichen planus with bullous lesions and concomitant squamous cell carcinoma. in a single patient is extremely rare and has not been previously reported to the best of our knowledge.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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