Disease characteristics, prognosis, and response to therapy in patients with large-cell transformed mycosis fungoides: A single-center retrospective study.

BACKGROUND: Mycosis fungoides with large-cell transformation (MF-LCT) is associated with an aggressive clinical course, yet data comparing treatment outcomes in MF-LCT are sparse. OBJECTIVE: To compare treatment outcomes and to determine disease prevalence and characteristics associated with survival in MF-LCT. METHODS: A retrospective review was conducted of mycosis fungoides patients from 2012 to 2020 treated at Thomas Jefferson University. Patients with histopathologic diagnosis of MF-LCT were included. Treatment outcomes were assessed by mean changes in the modified Severity Weighted Assessment Tool (mSWAT) and stage. RESULTS: Of 171 patients with mycosis fungoides, 23 (13.4%) had histologic diagnosis of MF-LCT. The overall 5-year survival rate for MF-LCT was 74% and was not significantly associated with sex, age, or initial stage at the time of MF-LCT diagnosis. Brentuximab vedotin showed the greatest mean decrease in mSWAT (-20.53) and stage progression (change in Δ stage: -0.4) in MF-LCT compared to oral bexarotene (ΔmSWAT: +4.51; Δstage: +0.27), skin-directed therapy (ΔmSWAT: -5.93; Δstage: -0.08), and chemotherapy (ΔmSWAT: +4.97; Δstage: +0.85). LIMITATIONS: Single-center retrospective design, and patients often on multiple treatment modalities. CONCLUSIONS: We report superior treatment outcomes for brentuximab vedotin compared to oral bexarotene, skin-directed therapy, and chemotherapy in MF-LCT in both early and advanced disease.

Response to topical corticosteroid monotherapy in mycosis fungoides.

BACKGROUND: Topical corticosteroids alone or in combination with other therapies are widely used to treat mycosis fungoides (MF), but data on response rates to their use as monotherapy in MF are limited. OBJECTIVE: To evaluate the efficacy of topical corticosteroid monotherapy in MF; compare sex, age, stage distributions, and histopathologic features between responders and nonresponders. METHODS: A retrospective cross-sectional review of patients with MF from 2013 to 2019 treated at Thomas Jefferson University was conducted. Patients with biopsy-proven MF, all stages, who received topical corticosteroid monotherapy were included. Response rates were determined by percent change in body surface area (BSA) involvement and modified Severity-Weighted Assessment Tool (mSWAT). RESULTS: Of the 163 patients with MF in our database, 23% (37/163) initially received topical steroid monotherapy. Of these, 73% (27/37) improved, with an average 65% decrease in BSA (67% in mSWAT); 27% (10/37) did not respond/progressed, with an average 51.6% increase in BSA (57% in mSWAT); and 33% (12/37) had a complete response (BSA, 0%) with prolonged topical steroid use. Early-stage MF and female sex were more represented in responders. LIMITATIONS: Single-center retrospective design. CONCLUSIONS: Topical steroid monotherapy in early-stage MF can produce measurable improvements in BSA and mSWAT scores and achieve complete remission in a limited subset of patients.

A prospective cohort study of condensed low-dose total skin electron beam therapy for mycosis fungoides: Reduction of disease burden and improvement in quality of life.

BACKGROUND: Low-dose total skin electron beam therapy (TSEBT) for mycosis fungoides is popular because of reduced toxicity with effective palliation. We condensed TSEBT, reducing visits by half and overall treatment length by one third. OBJECTIVE: To determine the efficacy and safety of a novel condensed low-dose TSEBT for mycosis fungoides. METHODS: We conducted a cohort study (2014-2018) with a median follow-up of 22.8 months. We delivered 12 Gy per 6 fractions with the modified Stanford technique, 3 fractions per week, with boosts to shadowed sites at risk between treatments, completing in 2 weeks. Primary outcomes included clinical response, duration of and time to response, and toxicity. Secondary outcomes included patient-reported quality of life (pain, pruritus, and Dermatology Life Quality Index) and physician-scored disease burden (body surface area involvement and Modified Skin Weighted Assessment Tool). RESULTS: Of 25 patients, stage IB was most common at the time of TSEBT (36%). The overall response rate was 88%. Most common was a near complete response (36%), and complete response was achieved in 6 (24%) patients. The median duration of response was 17.5 months (3.5-44.2), and the median time to response was 2 months (range, 0.9-4.1). No patients had toxicity of grade 3 or greater. QOL and disease burden showed significant benefit after TSEBT (P < .001). LIMITATIONS: Cohort study with limited sample size. CONCLUSIONS: Condensed, low-dose TSEBT has favorable outcomes and toxicity with logistical convenience.

Cutaneous B-Cell Lymphoblastic Lymphoma.

B-cell lymphoblastic lymphoma (B-LBL) is a malignant neoplasm of immature B cells that accounts for only 10% of all cases of lymphoblastic lymphoma. Most commonly, B-LBL presents as bony lesions, but in rare cases, the disease manifests cutaneously. We present a case of simultaneous cutaneous and systemic presentation of B-LBL in an otherwise healthy 28-year-old man in which the lymphoblastic infiltrate stained positive for CD79a, Tdt, CD10, and CD20. A diagnosis of cutaneous B-LBL was made, and systemic work-up revealed widespread involvement of the skin, bone, and lymph nodes. Review of all currently described cases of cutaneous B-LBL with or without systemic involvement revealed that the most frequently positive tumor markers were CD79a (92.3%), Tdt (90.6%), and CD10 (83.3%). Systemic involvement of B-LBL was found in nearly half of all cases with cutaneous presentation.

Koebner phenomenon: pemphigus vulgaris following Mohs micrographic surgery.

Koebnerization of pemphigus vulgaris (PV) is an infrequently reported reaction. We present a 65-year-old man with Koebnerized pemphigus vulgaris after Mohs microscopic surgery for a basal cell carcinoma. We present this case to heighten awareness of the phenomenon in the dermatological community.

Contiguous verrucous proliferations in syringocystadenoma papilliferum: A retrospective analysis with additional evaluation via mutation-specific BRAFV600E immunohistochemistry.

BACKGROUND: Syringocystadenoma papilliferum (SCAP) is an uncommon cutaneous adnexal proliferation. There have been several reports describing collision lesions of SCAP and verruca, although little is known about the frequency of this association. Molecular testing has revealed the BRAFV600E mutation in a large proportion of SCAP cases, although its expression pattern has not been previously evaluated. METHODS: In this retrospective analysis, we explored the potential histopathological association between verruca and SCAP. We also evaluated mutation-specific BRAFV600E expression in these lesions by immunohistochemistry. Cases of SCAP diagnosed over a 7-year period were closely reviewed for the presence of contiguous verrucous proliferations. Additional sections were cut and stained using the BRAFV600E-specific clone VE1 antibody. RESULTS: Contiguous verrucous proliferations were identified in 9 out of 12 identified cases. Furthermore, expression of the BRAFV600E mutation was identified in 7 out of 12 cases. Interestingly, in SCAP associated with endophytic verrucous proliferations (n = 4), expression of BRAFV600E was found in both the glandular and the contiguous hyperplastic squamous epithelium. CONCLUSION: Overall, these findings suggest that contiguous verrucous proliferations in SCAP are common. Both components of the neoplasm may express the BRAFV600E mutation, which is suggestive of a common origin.

Primary Cutaneous CD8+ T-cell Lymphoma, an Indolent and Locally Aggressive Form Mimicking Paronychia.

CD8 T-cell lymphomas comprise a wide spectrum of lymphomas, many which have yet to be formally classified. We present a case of a 43-year-old woman with an enlarging tumor distal to the distal interphalangeal joint of the fourth finger, compressing the underlying nail matrix. Magnetic resonance imaging showed bony involvement of the underlying distal phalanx. Histology showed a dense epidermotropic and pandermal infiltrate composed of medium-sized, uniformly pleomorphic lymphocytes with cleaved nuclei, which raised the possibility of primary cutaneous CD8 aggressive epidermotropic cytotoxic T-cell lymphoma. However, the patient's clinical photograph was inconsistent with this diagnosis. Other diagnoses, such as primary cutaneous acral CD8 T-cell lymphoma-a provisional entity, were also considered but did not capture all the features of this patient's lymphoma. We propose to classify this case as a primary cutaneous CD8 T-cell lymphoma, an indolent and locally aggressive form.

Clinical and histopathological features of cutaneous manifestations of adult-onset Still disease.

Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils. Later reports described a more persistent rash that tended to be photodistributed, hyperpigmented, often in a linear configuration, sometimes in a rippled pattern, referred to as the atypical eruption of AOSD. The presence of individual necrotic keratinocytes in the upper spinous layer has been the consistent histopathologic finding. The persistent rash may not represent an atypical presentation of AOSD as recent reports indicate a high prevalence of the rash. Emerging data also suggest that patients with persistent eruption have a worse prognosis. The recognition of the clinical and histopathological findings of skin eruptions of AOSD may facilitate an earlier diagnosis, potentially improving disease outcome. Herein, clinical and histopathological features of cutaneous manifestation of AOSD in 2 Asian women are highlighted accompanied by a relevant review of the disease.

Eruptive Milia Within a Tattoo: A Case Report and Review of the Literature.

Of the many tattoo reactions the most common are allergic, granulomatous, lichenoid, photosensitive, pseudolymphomatous, and infectious. Eruptive milia are a rare complication with only three prior reports in the English literature. A 19-year-old African American female presented with tiny, white papules confined within the margins of a tattoo. She denied trauma or associated symptoms at the site. Biopsy demonstrated deposits of black granular material within the dermis and a small infundibular cyst; a diagnosis of eruptive milia within tattoo was made. The milia responded to treatment with urea 40% cream and tretinoin 0.1% cream. Given its rarity, it is important to recognize the presentation of this disorder as other tattoo reactions require more aggressive management. While further research is necessary to determine the exact pathogenesis of this condition, the authors propose a mechanism along with a review of the literature to discuss management.

J Drugs Dermatol. 2017;16(6):621-624.

Evaluation of a 1540-nm and a 1410-nm Nonablative Fractionated Laser for the Treatment of Striae.

BACKGROUND: Aesthetically, striae distensae (SD) are a source of great concern. No treatment modality is currently considered the gold standard. However, studies of nonablative fractionated lasers (NAFLs) have been promising. OBJECTIVE: To evaluate and compare the clinical and histopathologic efficacy and safety of a 1540-nm NAFL and a 1410-nm NAFL for the treatment of SD. METHODS AND MATERIALS: Nine patients with abdominal striae were treated for 6 sessions-half of the abdomen was treated with a 1540-nm NAFL whereas the other half was treated with a 1410-nm NAFL. Photographs were taken at baseline and at the 3-month follow-up visit, when subjects were given a questionnaire. Two blinded dermatologists scored the photographs using a pre-established clinical scale. Biopsies were taken from 2 subjects and graded by 2 dermatopathologists using a pre-established pathology scale. RESULTS: All 9 subjects demonstrated clinical improvement bilaterally after treatment. Skin biopsies after treatment showed an increase in epidermal thickness, dermal thickness, and collagen and elastin density when compared with baseline. Clinical and histopathological differences between the 2 lasers were not statistically significant. CONCLUSION: Treatment with both the 1540-nm and the 1410-nm NAFL was shown to improve SD clinically and histopathologically. Further studies are needed to optimize treatment parameters.

Follicular Lymphomatoid Papulosis: An Eosinophilic-Rich Follicular Subtype Masquerading as Folliculitis Clinically and Histologically.

Lymphomatoid papulosis (LyP) is an uncommon CD30 lymphoproliferative disorder with a relatively excellent prognosis. Ten to twenty percent of cases, however, are associated with a lymphoma, typically systemic or cutaneous anaplastic large cell lymphoma, mycosis fungoides, or Hodgkin lymphoma. Subtypes divide LyP into infiltrate-descriptive categories along a spectrum of histological manifestation. Classically, LyP shows a patchy, wedge-shaped, perivascular dermal infiltrate of small- to intermediate-sized lymphoid cells, larger lymphoid, with one, 2, or multiple prominent nucleoli, and a variable admixture of neutrophils, eosinophils, and histiocytes. Follicular LyP shares these characteristics, although its infiltrate is folliculocentric. Variable folliculotropism, follicular dilation, rupture, and mucinosis can occur. This entity is commonly misdiagnosed and underreporting likely because its histopathologic features can masquerade as more common follicular-based entities. The authors present 2 cases of this rare variant to underscore the importance of clinicopathologic correlation in diagnosis. To the best of the authors' knowledge, this is the first report of the follicular LyP variant with concurrent mycosis fungoides. In the context of a literature review, diagnostic pitfalls and classification of this variant are discussed.

Adult Manifestation of Henoch-Schönlein Purpura.

A 51-year-old man initially presented to the hospital with a 2-week history of constipation, intermittently diffuse abdominal pain, nausea, vomiting, and productive cough. Results from chest x-ray and obstruction series appreciated a right lower lobe opacity and small right pleural effusion. The patient met sepsis criteria and was empirically covered for community-acquired pneumonia with vancomycin, piperacillin-tazobactam, moxifloxacin, and tobramycin. His hospital course was complicated by oliguria, acute kidney injury with a normal renal ultrasound, and transaminitis with a negative hepatitis panel. After clinical improvement, he was discharged on 2 additional days of levofloxacin to complete a 7-day antibiotic course.

Asynchronous presentation of follicle center lymphoma and reactive lymphoid hyperplasia of the ocular adnexa.

Although ocular lymphoproliferative diseases may be rarely encountered by dermatopathologists, the frequency may increase particularly as more and more subspecialties rely on dermatopathology services. Emerging data suggest that there are some similarities between ocular and cutaneous lymphoproliferative diseases with respect to their clinical behavior, prognosis, and cytogenetics. In this case report, a patient with ocular follicle center lymphoma who subsequently developed ocular reactive lymphoid hyperplasia is presented with an accompanying review of the literature on the subjects. The encounter of both follicular center lymphoma and reactive lymphoid hyperplasia in the same patient provides a rare opportunity to compare and contrast the clinical, histological, and immunohistochemical findings of the respective lymphoproliferative diseases situated at opposite ends of the spectrum.

Tinea lucidum or dermatophytosis of the stratum lucidum: is the epidermal location of dermatophyte infection evolving?

Dermatophyte infections are traditionally localized to the stratum corneum. The advent of immunosuppressants and topical steroid/antifungal preparations, however, has created a new phenomenon: dermatophytosis in the stratum lucidum. This atypical presentation manifests in 3 clinical scenarios: oral immunosuppression and/or medical comorbidities, lesions/body sites with lichenification, and sites on/near acral skin. In each setting, superficial potassium hydroxide (KOH) preparations have proved ineffective in diagnosis, despite high indices of suspicion for dermatophytosis. It is only under histologic examination that florid hyphal elements are identified. The authors propose a modified KOH technique requiring a sample of deeper, pathological scale-containing fungal elements to be used in scenarios where tinea lucidum or dermatophyte infection of the stratum lucidum may be present.

Eosinophilic dermatosis of hematologic malignancy.

Cutaneous involvement by an eosinophil-rich process (eosinophilic dermatosis) may be encountered in the setting of various hematologic malignancies, including mantle cell lymphoma, acute monocytic leukemia, acute lymphoblastic leukemia, large cell lymphoma, myelofibrosis and chronic lymphocytic leukemia (CLL). Of the various hematologic malignancies, eosinophilic dermatosis has been most frequently described in association with CLL. Published previously as insect bite-like reaction and eosinophilic dermatosis of myeloproliferative disease, this rare dermatitis presents as a pruritic, papular and occasionally vesicular eruption associated with an eosinophil-rich infiltrate histopathologically. Although clinical and histopathologic features are similar to insect bites, affected patients frequently deny a history of insect bites. We report a case of eosinophilic dermatosis of hematologic malignancy in a patient with known history of CLL.

Pseudocyst of the auricle: an uncommon entity of the ear.

A 48-year-old white man presented with a 2-month history of a lump on his right ear following purported exposure to the cold. The patient denied any history of trauma, rubbing, insect bite, or inflammation at the site. The patient was otherwise healthy and was not taking any medications. The lesion was completely asymptomatic, as the patient denied any pain, tenderness, or change in size since the lesion appeared. Physical examination revealed a 1.1-cm x 1-cm skin-colored, slightly fluctuant, rubbery, nonmobile dermal nodule on the right scaphoid fossa (Figure 1). No erythema, warmth, tenderness, or drainage was noted. A review of systems was noncontributory. A punch biopsy performed at the site of the cyst yielded clear, serous drainage. The cystic lesion was drained completely and decompressed. Histological examination revealed the surface of an intracartilaginous cystic space lined by degenerated cartilage, consistent with a diagnosis of pseudocyst of the auricle (Figure 2 and Figure 3). The lesion, although much smaller than at the time of presentation, persisted at the 3-week follow-up visit. It remained asymptomatic, without pain or irritation. At that time, the patient declined re-excision with bolster dressing. At 7-month follow-up, he reported the lesion to be stable.