Successful Treatment of Resistant Lichen Planus Pemphigoides with Cyclosporine: A New Hope.

Sir,

Lichen planus pemphigoides (LPP) is a rare bullous disorder and clinically appears to be a combination of lichen planus and bullous pemphigoid, characterized by development of bulla atop the classical lichen planus lesion or de novo in the uninvolved skin. LPP is first described by Kaposi as lichen ruber pemphigoides in 1892. Controversy regarding its nosology; whether immunologically a distinct entity or co-existence of LP and BP is yet to be solved. Though common antigen (180 kd) shared by BP has been identified, but involvement of a novel epitope of the same is a matter to be considered so also the participation of another 200 kd protein as a culprit one. The uniqueness of our case lies in the way that it failed to respond well to conventional systemic steroid and familiar immunosuppressive methotrexate. Excellent response to cyclosporine within 2 weeks brought new hope regarding treatment of resistant cases like ours.

A 35-year-old male presented with multiple pruritic, violaceous papule, plaque, and blisters distributed chiefly over distal extremities extending into the trunk for last 4 months. According to the patient, initial lesions were reddish elevated pruritic ones started 8 months back. He was provisionally diagnosed with lichen planus and treated with topical medications with frequent waxing and waning. Cutaneous examination revealed multiple, violaceous, fat-topped papules and erythematous patches present over the forearm, lower leg, and abdomen. He also had multiple tense blisters over preexisting lesions and normal skin predominantly over flexor aspect of forearm [Figure 1] and abdomen. Some of the blisters are filled with hemorrhagic fluid. There were no involvement of oral mucosa, hair, and nail. General and systemic examinations were within normal limit.

Figure 1

Blisters filled with hemorrhagic fluid over flexor aspect of forearm

Histopathology from bullae reveals subepidermal blister formation with moderate inflammatory infiltrate [Figure 2]. DIF from perilesional skin showed linear deposition of IgG and C3 along the BMZ without cytoid body staining. Other routine investigations were within normal limit. IIF could not be done considering the financial burden of the patient.

Figure 2

Histopathology showing sub-epidermal blister formation with moderate inflammatory infiltrate

In contrast to LPP, bullous LP is typified by appearance of bullae strictly on present or previous LP lesions, histopathology and DIF finding consistent with LP in excess of sub epidermal bullae that could possibly be explained by exaggerated BMZ destruction by band such as lymphohistocytic infiltrate and negative IIF.

Based on clinical and laboratory findings a final diagnosis of LPP was made, and the patient was put on oral prednisolone (40 mg/day). The initial response was dramatic, all lesions healed up within a period of 7 days. The dose of the steroid is gradually tapered over 2 months; but as it reached to 20 mg/alternate day, clinical relapse did take place. The dose of steroid again escalated to 40 mg/day with the addition of 15 mg/week methotrexate as an adjuvant one. But unfortunately, this time the result was frustrating. Though responded to some extent full regression could not be achieved even after 2 months. Now the patient has put on 200 mg cyclosporine in two divided doses for 2 weeks, and the result was dramatic; all the bullae subsided without the eruption of any fresh ones [Figure 3]. Cyclosporine was continued further with gradual tapering to 100 mg every alternate day [Figures 4 and 5] and then stopped after 3 months. Now the patient is on topical corticosteroid [Figure 6]. Since the discontinuation of cyclosporine for last 2 months, there was no relapse of bullae in this case.

Figure 3

Subsidence of bullae following treatment with cyclosporine

Figure 4

Lesions of Lichen planus and postinflammatory hyperpigmentation during therapy

Figure 5

Lesions of lichen planus and few postinflammatory hyperpigmentation during therapy

Figure 6

Healed lesions of lichen planus and postinflammatory hyper-pigmentation involving other body parts following therapy

In some cases of LPP, only topical steroid is sufficient.[1] In most of the cases, moderate doses of systemic steroid are effective.[2] Combination therapy with corticosteroid and dapsone was also used with success.[3] To the best of our knowledge, there is one case report of LPP successfully treated with a combination of cyclosporine and prednisolone.[4] Cyclosporine therapy may have some advantages. It inhibits itching by modulating the function of CD4+ T-cells. In long standing LP cases, particularly when LPP is present, basal keratinocytes damage by liquefaction may increase BP180 antigen exposure leading to antibody response and ultimately bullae formation.[5]

We could not conclude the reason for the failure of a systemic steroid and MTX controlling LPP lesions in our case. However, the fact that cyclosporine was highly effective in treating this case added an armamentarium in the treatment of LPP cases.

The possibility of LPP should be kept in mind if bullous lesion develops in the association of LP lesions and should be evaluated further to hit the target keeping in mind the close differentials. Though systemic steroid is reported to be highly effective in most of the literature, its failure and dramatic response to cyclosporine in our case could expand the horizon of treatment options in resistant cases if needed.

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Nil.

Conflicts of interest

There are no conflicts of interest.