Case Number : Case 1815 - 12 May - Dr Richard A Carr Posted By: Guest

We wrap up our week with a fine case from Dr Richard A Carr

Geoff Cross - DermpathPRO Projects

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My first thought is LICHEN PLANUS PEMPHIGOIDES, because:

there are too eosinophils in a subepidermal distribution, almost linear, as waited in BP

you can see hypergranulosis acanthosis and hyperkeratosis as waited in LP

annular and almost blistering lesions fit well!

Agree with LP pemphigoides.

Lichenoid dermatitis with eosinophils. Drugs would be my first thought.

Incidentally, I was just reading up on LP Pemphigoides on derm101 a few minutes ago in relation to one of my own cases and came across this interesting write up by Dr. Ackerman. Comments?

'Lichen planus pemphigoides is a condition reputed to be true lichen planus linked in the very same lesion to bullous pemphigoid. In our experience, what are purported to be combinations of different diseases in the same lesion usually turns out to be not a collisions or concurrence of them, but one or the other of them alone. Time was, in the not-too-distant past, when some reputable workers averred that pemphigus erythematosus really was coexistence of pemphigus vulgaris and lupus erythematosus in a single lesion. That assertion is heard no more. In our estimation, pemphigus erythematosus is unrelated to lupus erythematosus. Even now, some serious students contend that an overlap sometimes occurs between discoid lupus erythematosus and lichen planus, the so-called overlap syndrome. In our view, the condition is either lichen planus or lichenoid discoid lupus erythematosus. In sum, although cutaneous inflammatory diseases that are common do collide with one another rarely, e.g., seborrhiasis (sebopsoriasis) as a Koebner phenomenon induced by seborrheic dermatitis in a patient with a psoriatic diathesis, in the vast majority of instances they are separate, unrelated conditions, i.e., seborrheic dermatitis and psoriasis. Readers should be wary of diagnoses lichen planus pemphigoides and lichen planus actinicus!'

Drug reaction

Lichen planus-like bullous pemphigoid, to be confirmed by direct immunofluorescence. Drug reaction must be ruled out.

Commenting upon the text brought by Dr. Attili, I think I was particularly influenced by Dr. Ackerman's opinion since I am disciple of a distinguished alumnus of the Ackerman Academy of Dermatopathology, and I've already read his complete works (actually, I still read his works very often). So I do not believe in a coexistence of both conditions in one same lesion. I prefer to sign out lichen planus-like bullous pemphigoid when I interpret the lesion as bullous pemphigoid with lichen planus-like features, or bullous pemphigoid-like lichen planus when I interpret the lesion as lichen planus with subepidermal blister resembling bullous pemphigoid.

Yes, a lichenoid reaction pattern with eosinophils. Needs DIMF. Not inconsistent with lichen planus pemphigoides. Can never exclude a drug reaction. However, favour LPP. 

I had a recent case that was similar with hypertrophic lichen planus in a patient on a pd 1inhibitor for metastatic melanoma. I see the eosinophils which can are reported in hypertrophic lp  especially drug induced. I see the max joesph space separation. I was thinking lichen planus pemphigodies but never seen if. Drug related something. Would be keen to hear bout the dif. More Keen to learn from the stains? Thanks

Lichenoid toxicodermia VS LP pemphigoides 

I initially reported this as possibly drug induced lichen planus but the clinical colleague indicated to me that IMF was positive with IgG and C3 at the BMZ. Indirect IMF / ELIZA also positive supporting pemphigoid. Clinically it was favoured to be pre-bullous pemphigoid. I felt somewhat embarrassed to have missed the possibility of bullous pemphigoid completely!  To my eye the striking lichenoid reaction is limited to the follicles i.e. the pattern of lichen planopilaris. I read the discussion above with great interest. I think this patient does have bullous or pre-bullous pemphigoid. I don't know if the lichen planopilaris pattern is merely an expression of pemphigoid or whether this is a case of lichen planopilaris pemphigoides?

Nice case Richard. I guess the diagnosis then would be Lichen Planus like Bullous Pemphigoid?

1 hour ago, Dr. Richard Carr said:

I initially reported this as possibly drug induced lichen planus but the clinical colleague indicated to me that IMF was positive with IgG and C3 at the BMZ. Indirect IMF / ELIZA also positive supporting pemphigoid. Clinically it was favoured to be pre-bullous pemphigoid. I felt somewhat embarrassed to have missed the possibility of bullous pemphigoid completely!  To my eye the striking lichenoid reaction is limited to the follicles i.e. the pattern of lichen planopilaris. I read the discussion above with great interest. I think this patient does have bullous or pre-bullous pemphigoid. I don't know if the lichen planopilaris pattern is merely an expression of pemphigoid or whether this is a case of lichen planopilaris pemphigoides?

Dear Richard, the keratin staining was to highlight the colloid bodies?

Yes. I'm sure you all saw them in numerous quantities on the H&E's but I felt it would be nice to show the clinical colleague I had not imagined the lichen planus-like features and was not a total idiot!

Thanks for this case, Richard. I don't know if it's more correct to define a similar case as lichen planus-like BP or LPP or BP, and I feel a bit confused about the appropriate differential diagnosis, but I'm sure that I've learned a lot about this pathological chapter!!!

Thankx for the case Richard but ....Don't you think that pre- bullous pemphigoides( urticarial and eccematous plaques) fit more in spongiotic dermatitis than lichenoid?