A 66-year-old man was referred to our dermatology clinic for a 6-year history of a progressive pruritic rash, refractory to prescribed treatments. On examination, he had widespread follicular papules with 90% body surface involvement, nontender inguinal lymphadenopathy, and leonine facies (Figure 1A). He had seen 2 dermatologists who had performed skin biopsies; these were nondiagnostic but suggestive of follicular dermatitis, spongiotic pityrosporum folliculitis, or arthropod reaction. Previous treatments included topical corticosteroids, antihistamines, antimicrobials, oral retinoids, and phototherapy, without benefit. Empiric ivermectin for scabies did not improve his pruritus. At our consultation, he was on methotrexate and low-dose prednisone for presumed severe dermatitis.
Figure 1:
(A) A 66-year-old man with leonine facies from advanced folliculotropic mycosis fungoides. (B) Three months later, the patient showed improvement after radiation and adjuvant treatment with brentuximab.
Our repeat biopsies revealed exocytosis of lymphocytes with nests of atypical lymphocytes at the dermal–epidermal junction and follicular mucin. Most of the atypical lymphoid cells were positive for CD4 and CD5 and negative for CD2 and CD7; around 10%–20% of atypical lymphoid cells expressed CD30. Blood flow cytometry confirmed an abnormal T-cell population. These findings were suggestive of T-cell lymphoma. Serologies for tuberculosis, hepatitis, HIV, human T-lymphotropic virus type 1 or 2, and strongyloidiasis were negative. Computed tomography showed cervical, mandibular, inguinal, and axillary lymphadenopathy. We diagnosed folliculotropic mycosis fungoides with early large-cell transformation (stage IIIB). We referred the patient to a radiation oncologist and a hematologic oncologist for urgent management. He underwent radiotherapy over 6 days and received adjuvant treatment with brentuximab every 3 weeks for around a year, after which his skin lesions and pruritis had improved (Figure 1B).
Leonine facies describes coarse facial skin with deep, prominent folds. It has a broad differential diagnosis, including leprosy, leishmaniasis, systemic amyloidosis, sarcoidosis, nodular mastocytosis, mucinoses, and chronic actinic dermatitis. Folliculotropic mycosis fungoides is a rare and aggressive cutaneous T-cell lymphoma; 0.45% of patients have leonine facies.1,2 Repeat investigations, including skin biopsies, may be required when the cause of leonine facies is unclear or refractory to treatment. For this patient, immunohistochemistry facilitated diagnosis and access to brentuximab treatment, an anti-CD30 chimeric monoclonal antibody with high response rates for refractory cutaneous lesions and extracutaneous disease in cutaneous T-cell lymphoma.3
Footnotes
Competing interests: None declared.
This article has been peer reviewed.
The authors have obtained patient consent.
References
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