PMC5985601_01
Case 1: Diffuse tumoral thickening of the face with cystic areas.
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On examination, she presented generalized infiltrated erythematous-violaceous plaques and diffuse facial tumoral thickening with cystic areas, giving an overall leonine appearance (Figure 1).
Case 1: Microscopic examination (200x magnification) of the initial biopsy demonstrates a prominent mixed lymphohistiocytic and eosinophilic, perifollicular and dermal, inflammatory infiltrate. The infiltrate is polymorphous and dominated by reactive cells obscuring the atypical lymphoid population.
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Suspecting an underlying lymphoproliferative etiology, biopsies including immunophenotyping and gene rearrangement studies were done and revealed a dense heavy dermal lymphoeosinophilic infiltrate, with no evidence of atypical lymphocytes (Figure 2).
Case 1: Microscopic examination (200x magnification) of the follow-up (post-treatment) biopsy demonstrates more moderate inflammation with less reactive background cells including fewer eosinophils. The atypical folliculotropic infiltrate is more conspicuous and permitted more reliable ancillary pathological testing.
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Only 4 months later while on continuous prednisone therapy did a biopsy show a greatly diminished eosinophilic infiltrate with underlying findings finally consistent with FMF (Figure 3).
PMC5985601_02
PMC5985601_03
Case 3: Multiple comedo-like papules of the face with alopecia of the scalp and eyebrows.
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A 72 year-old female was referred to our clinic for a 6-year history of widespread comedogenic rash (Figure 4).