Sarcoidosis with small syringotropic granulomas presenting clinically as a pigmented purpuric dermatosis: Inconspicuous clinical and histopathological clues to systemic illness.

Sarcoidosis is a multisystem granulomatous disease with a myriad of clinical manifestations and a predilection to involve the lungs, eyes, lymph nodes, and skin. A 38-year-old man presented to dermatology with a history of progressive dyspnea, pulmonary consolidations on chest X-ray, and hilar adenopathy on computed tomography scan. Skin exam revealed asymptomatic, yellow to brown macules on the right lower extremity. Biopsy of a lesion showed diminutive syringotropic granulomas and perivascular hemosiderin; stains for bacteria, mycobacteria, and fungi were negative. Subsequent fine needle aspiration of a hilar mass revealed non-necrotizing epithelioid granulomas further supporting a diagnosis of sarcoidosis. The patient was placed on systemic steroids and had improvement of his pulmonary symptoms and stabilization of his hilar lymphadenopathy without resolution of his pigmented purpuric dermatosis (PPD) like lesions. Only three prior cases of syringotropic sarcoidosis have been reported; however, the biopsies had revealed conspicuously large granulomas in contrast with the small granulomas in our case, and none of the prior patients had clinical examination findings that mimicked PPD. Recognition of rare dermatologic and histopathological appearances of sarcoidosis is paramount as cutaneous sarcoidosis may be the harbinger of a systemic illness, which requires a timely diagnosis.

Tumor-stage mycosis fungoides in palmoplantar localization with large-cell transformation and partial CD30 expression shows complete response to brentuximab vedotin.

Mycosis fungoides in palmoplantar localization (MFPP) is a rare variant of MF that is confined to the hands and feet. Patients commonly receive treatment over many years for suspected palmoplantar dermatitis before the diagnosis is made. Most MFPP patients remain at patch or plaque stage, and often respond to treatment with radiotherapy. Herein, we describe a 77-year-old man who suffered 6 years of hand and foot dermatitis that failed multiple treatments, most notably TNF-α inhibitors and mycophenolate mofetil. He eventually developed a tumor on the hand, which was biopsied to reveal a dense dermal infiltrate of large lymphocytes (CD3+/CD4-/CD8-/TCR-BetaF1+/partial CD30+). A subsequent biopsy of an eczematous patch from his hand revealed an epidermotropic and syringotropic infiltrate comprised of smaller lymphocytes with a concordant immunophenotype and matching clonal peak with TCR gene rearrangement. He was diagnosed with MFPP and started on radiotherapy with a modest response; therefore, a decision was made to start brentuximab vedotin, which resulted in a complete response. MFPP is an exceedingly rare variant of MF that can show large-cell transformation and progress in stage. We highlight a possible association between disease progression and immunosuppressants and the potential role for treatment with brentuximab.