Kaposi Sarcoma Associated with Tofacitinib Use in a Patient with Rheumatoid Arthritis.

Kaposi sarcoma is a rare vascular malignancy associated with HHV-8 infection. Four variants of Kaposi sarcoma have been described: Classic, African, HIV-associated, and iatrogenic. Iatrogenic Kaposi sarcoma is typically associated with immunosuppression and organ transplantation. We present a case of iatrogenic Kaposi sarcoma associated with tofacitinib therapy. A 69-year-old woman with rheumatoid arthritis receiving tofacitinib presented with multiple firm, purple-red nodules and brown plaques on the left lower extremity and a single lesion on the right medial calf. Clinicopathologic correlation confirmed a diagnosis of Kaposi sarcoma. Tofacitinib was discontinued and she was started on Alitretinoin 0.1% gel bid. The purple-red Kaposi sarcoma nodules decreased 50% in size after 4 months and resolved at 1 year off the tofacitinib and initiation of alitretinoin gel. As the use of immunomodulators and biologics continues to expand, awareness of this association is important for prompt diagnosis and management.

Cutaneous Sporothrix globosa infection mimicking cryptococcosis in a patient with history of kidney transplant.

Sporotrichosis is a subacute-to-chronic infection caused by Sporothrix species, a dimorphic fungus. Virulence varies by Sporothrix species and presentation can be region dependent. The patient had a history of immunosuppression as a result of a kidney transplant, and presented with a high disease burden on histopathological examination, but responded well to itraconazole. The case suggests considering Sporothrix speciation in immunocompromised patients to best determine treatment modality and duration.

A histo-immunopathologic and prognostic study of erythrodermic cutaneous T-cell lymphoma.

BACKGROUND: Sézary syndrome (SS) and erythrodermic mycosis fungoides (E-MF) represent two expressions of erythrodermic cutaneous T-cell lymphoma (E-CTCL). METHODS: Histopathologic features were compared on skin specimens from 41 patients with SS and 70 patients with E-MF. Immunopathologic findings were compared on 42 SS and 79 E-MF specimens. RESULTS: Specimens of SS usually showed band-like dermal infiltrates with intermediate-sized lymphoid cells and few plasma cells; on the other hand E-MF more often had a perivascular infiltrative pattern, predominance of small/mixed lymphoid cells and eosinophils. SS also had lower numbers of CD8+ cells and higher numbers of CD62L+ cells compared to E-MF. For E-MF patients, the presence of large Pautrier collections, infiltrates with intermediate-sized cells, increased number of mitotic figures and ≥50% CD62L+ cells in the dermal infiltrate correlated with a relatively poor disease-specific survival. However, only the presence of mitotic figures retained prognostic significance with clinical stage as a covariate. CONCLUSIONS: Clinical stage provides the most important prognostic information for patients with E-CTCL. However, mitotic activity for E-MF and CD8+ cells <20% for SS have additional value. We hypothesize that observed differences in plasma cell and eosinophil numbers may reflect the influence of CD62L+ central memory T-cells in the microenvironment.

Unilesional Follicular Mycosis Fungoides: Report of 6 Cases and Review of the Literature.

Unilesional follicular mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma characterized by selective involvement of the hair follicles by neoplastic T lymphocytes presenting initially as a solitary lesion occupying less than 5% of the body surface; there are 22 previous reported cases. We describe 6 patients with this rare variant of MF, all males (age range 6-64 years; mean age: 28 years) presenting with a solitary lesion primarily on the face and scalp except 1 patient who presented with a truncal lesion. All the patients had the lesions for at least a few months. The lesions were associated with follicular prominence and hair loss. In each, the biopsies showed an atypical folliculotropic lymphocytic infiltrate accompanied by follicular mucinosis in certain cases. The infiltrate involved the lower isthmic part of the follicle, and in 1 case, there was an alopecia areata-like neoplastic lymphomatoid bulbitis. There was no evidence of large cell transformation nor was there a significant degree of infiltration of the interfollicular dermis or epidermis. Higher magnification disclosed marked cerebriform atypia amidst the lymphocytes. Phenotypic studies demonstrated a high CD4 to CD8 ratio in excess of 5 with a significant loss of CD7. One patient developed additional similar lesions involving the thigh and buttock after a period of at least 3-4 years of untreated unilesional MF. Unilesional follicular MF is a potentially curable form of MF with a young male predilection. Early diagnosis and treatment intervention likely define a cornerstone for ensuring the best patient outcome and preventing clinical evolution to either tumor stage MF and or multilesional MF.

Trichophytum rubrum endonyx onychomycosis resistant to standard oral and topical therapies.

We present a 45 year-old man with an eight-year history of discoloration of the nail plate on his left hallux. He had been treated with two courses of oral terbinafine and topical 8% ciclopirox for presumed onychomycosis. On exam, his left great toenail contained a wide yellow-white longitudinal band involving a majority of the nail plate. No subungual debris, hyperkeratosis, or paronychial inflammation was present in the affected nail. Histopathology of the nail plate revealed numerous fungal elements arranged transversely and longitudinally, solely within the keratin layers of the nail plate; these were highlighted with periodic acid-Schiff (PAS) stain confirming endonyx onychomycosis. Cultures grew Trichophyton rubrum. All types of onychomycosis under the new classification system proposed by Hay et al. have now been associated with T. rubrum. Endonyx related to T. rubrum may be a particularly difficult infection to treat with oral or topical agents owing to the absence of robust local immune response and limited drug penetration to the interior nail plate. Physicians should be aware that this type of infection may require treatment with dual-agent therapy or alternative modalities including chemical or surgical plate avulsion or photodynamic therapy.

Superficial acral fibromyxoma and other slow-growing tumors in acral areas.

Superficial acral fibromyxoma (SAFM) is a rare fibromyxoid mesenchymal tumor with a predilection for the distal extremities and frequent nail bed involvement. Superficial acral fibromyxoma typically arises as a solitary, slow-growing nodule on a toe or finger, with the great toe being the most commonly affected site. Histopathologically, SAFM characteristically presents as a well-circumscribed but unencapsulated dermal tumor composed of spindle and stellate cells in a loose storiform or fascicular arrangement embedded in a myxoid, myxocollagenous, or collagenous stroma. The tumor often occupies the entire dermis and may extend into the subcutis and occasionally the underlying fascia and bone. The characteristic immunohistochemical profile of SAFM includes expression of CD34, epithelial membrane antigen (EMA), and CD99; it is notably negative for S-100 protein. We report 3 cases of SAFM and also provide a review of the literature on the clinical and histopathologic presentations of this unique entity as well as the differential diagnosis.

Prognostic factors and risk stratification in early mycosis fungoides.

Available demographic, clinical, histologic, immunohistochemical and laboratory findings, including serum cytokine/cytokine receptor levels, obtained at initial evaluation in a cohort of 33 patients with mycosis fungoides (MF) at stages I-IIA who had subsequent progression of disease were compared against 70 stage-matched cases of MF without observed progression. Significant factors that correlated with both disease progression and overall survival were: (1) presence of large Pautrier microabscesses (10 or more atypical lymphocytes), (2) presence of atypical lymphocytes with hyperchromatic or vesicular nuclei in the dermal infiltrate, (3) less than 20% CD8 + cells in the dermal infiltrate and (4) above normal (> 122 U/mL) serum immunoglobulin E (IgE) level. Combination of these factors was used to construct prognostic groupings which, if validated, might be useful to identify patients with clinically early MF at highest risk for disease progression and poor outcome.

Late-onset focal dermal elastosis: an uncommon mimicker of pseudoxanthoma elasticum.

Late-onset focal dermal elastosis is a rare disorder that presents clinically with the development of small white-to-yellow papules simulating pseudoxanthoma elasticum (PXE) in otherwise healthy adults in the seventh through ninth decades. It is characterized histopathologically by foci of increased normal-appearing elastic tissue in the reticular dermis. The disorder lacks any of the systemic complications of PXE and clinically resembles several other elastic tissue disorders that mimic PXE. We report two cases of late-onset focal dermal elastosis. The first is of a 75-year-old female who presented with symmetrically distributed, 2-5 mm white-to-yellow, discrete and coalescing, non-follicular papules on the posterolateral neck, anterior chest and axillae. The second case involves a 39-year-old female who presented with asymptomatic flesh-colored lesions on the posterior neck, back, antecubital and popliteal fossae, thighs, forearms and wrists. Skin biopsies in each case revealed aggregates of elastic fibers in the reticular dermis without calcification. The differential diagnosis of clinical and histopathologic imitators of PXE is discussed.

Verrucous carcinoma of the nail unit.

Verrucous carcinoma (VC) of the nail bed and matrix is a rare malignancy that is often misdiagnosed as a benign condition. We present a case of a 65-year-old woman with a 2-year history of a progressive right fourth fingernail verrucous plaque replacing and surrounding her entire fourth fingernail. Diagnosis of VC was confirmed by a longitudinal biopsy and patient underwent en bloc excision and skin graft placement. The patient remained disease free with acceptable function of the digit at 24 months of follow-up. Although uncommon, VC must be considered in all patients with a persistent and enlarging verrucous lesion involving the nail apparatus. Preoperative evaluation must include an adequate biopsy specimen to avoid misdiagnosis of verruca vulgaris. Digital radiographs are recommended to evaluate local invasion to bone.

Generalized purpuric drug exanthem with hemorrhagic plaques following bendamustine chemotherapy in a patient with B-prolymphocytic leukemia.

A 72-year-old woman presented with dyspnea and lower extremity edema. Extreme lymphocytosis, cytopenia, and splenomegaly were found, and she was diagnosed with B-prolymphocytic leukemia. Following the first dose of therapy with bendamustine, the patient developed severe generalized maculopapular rash, which subsequently progressed to exuberant, non-blanching palpable purpura with hemorrhagic plaques suspicious for leukocytoclastic vasculitis. These events coincided with severe chemotherapy-induced neutropenia and thrombocytopenia, but there were no clinical symptoms of infection. Skin punch biopsy revealed perivascular and diffuse upper dermal lymphocytic infiltrate with eosinophils and marked erythrocyte extravasation consistent with a purpuric drug exanthem. The patient was treated with steroids, with complete resolution of the findings. This new form of cutaneous toxicity of bendamustine is presented along with a review of previous experience with the drug.

Vestibular papillomatosis: a benign condition mimicking genital warts.

Vestibular papillomatosis (VP) is a benign condition of the female genitalia that may be mistaken for condyloma acuminatum (genital warts). In contrast to condylomata, lesions of VP each grow from a distinct mucosal insertion; match the color of the surrounding mucosa; and are symmetrically distributed, limited to the inner labia minora and vaginal introitus. Recognition of this entity will help to prevent unnecessary stress, testing, and discomfort.