Case Number : Case 1217 - 20 February Posted By: Guest

?Lichen planopilaris. ??Lupus.

I guess if the patient also has typical scalp lesions of lichen planopilaris and this is a case of Graham-Little-Piccardi-Lassueur syndrome.

Agree, if no scalp lesions, no hair loss in axillae and pubic area will call it just follicular lichen planus. If associated with cicatricial scalp alopecia, hair loss in axilae and pubic areas, will be as Romualdo said: Graham Little Piccardi Lassueur syndrome.

The clinical is exactly as I described. Any other thoughts or considerations?

follicular plugging, eccentric atrophy of hair follicles and perifollicular lymphocytic infiltrate. thinking of keratosis pilaris.

To Dr Richard Carr
Sir, i am from India. i recently completely my M.D Dermatology. i have been regularly reading the spot diagnosis cases and learned a lot. it's great to learn from so many great dermatopathologists.

Sir, we usually see a lot of children with follicular keratotic papule, both discrete and coalsced to form plaque. we usually put differentials of Keratosis pilaris, Phrynoderma, atypical PRP, Follicular psoriasis and sometime Follicular eczema and lichen spinulosus. It's not always possible to diagnose them clinically. but unfortunately manytimes the biopsy report also comes as nonspecific.

Sir, can you just put some light how to differentiate them histopathologically. Thank you

Keratin plug, follicular plugging, dilated infundibulum, heavy perifollicular infiltrate: lichen spinolosus.

Ok, let me put another hypothesis
Recent onset
Follicular hyperkeratosis together with Interface reaction in a folliculocenteric pattern with necrotic keratinocytes in the follicular epithelium.
If not follicular LP, then I will suggest that something is missing in the history, may be she was on BRAF kinase inhibitor or other protein kinase inhibitors for some sort of cancer therapy and this is the peculiar keratosis pilaris like folliculocenteric form of drug reactions related to kinase inhibitors .... May be :-))
It seems that every Friday we should think out of the box !!!!
I love that...
Hope I got it right ..

I think Mona's hypothesis will be correct!

Lichen spinolosus

Lichen spinulosus is a keratotic disorder. As such, the papules are not erythematous (contrary to the history of this case) and the inflammation is light and literally perifollicular. The pictures, to my eyes at least, show interface inflammation, wherein the basal layer is damaged. Lichen planopilaris continues to be my favoured option. Below is a description of 1 of 3 clinical variants of lichen planopilaris (14 of 33 patients), which I think fits the history of this case:

"A 54-year-old man had a generalized, itchy eruption of 4 months' duration. The trunk and extremities were diffusely involved with multiple but discrete, 1 to 2 mm, keratotic follicular papules (Fig. 1), some of which were violaceous. No evidence of scarring or alopecia was observed. The scalp and the mucous membranes were uninvolved. Findings of the remainder of the physical examination were normal."

Matta M, et al. Lichen planopilaris: A clinicopathologic study. J Am Acad Dermatol. 1990; 22:594-598.

Dr Abdel Halim's theory is superb, but I do no think that Dr Carr would expect us to make a diagnosis of an anti-BRAF drug reaction whilst withholding the history of such a drug!

If not follicular Lichen planus.Then mimickers such as Lupus , mycosis fungoids , and chronic graft versus host disease , ..etc should be kept in mind always.Neverthless in this scenario, we may think of keratin spicules or lichen plaris-like lesion that may occure in multiple myeloma patient!

First thank you to you all for another excellent discussion. Your combined experience far outweighs my own so I learned a lot again from the responses and I certainly cannot claim to be the master who can answer all questions. My reading of Weedon on keratosis pilaris led fairly quickly to [b]lichen spinulosus[/b] which seemed to fit the clinical (anatomic distribution of recent onset KP-like keratotic papules all though the age is normally younger) and histologial quite well (inflammatory variant of KP) but I did append my report that features were histologically close if not indistinguishable from lichen planopilaris - I had not personally seen a case of LPP with the nice KP-like spikes seen here but do wonder if they may be one and the same diagnosis. Sorry to dissappoint Mona as the hypothesis was really rather good. I will certainly mention it to the clinician as I am guessing other drugs might be relevent and new and varied drug reactions should always be considered for odd or novel dermatoses.