Jacqueline M. Junkins-Hopkins

Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

Sézary syndrome (SS) has a poor prognosis and few guidelines for optimizing therapy. The US Cutaneous Lymphoma Consortium, to improve clinical care of patients with SS and encourage controlled clinical trials of promising treatments, undertook a review of the published literature on therapeutic options for SS. An overview of the immunopathogenesis and standardized review of potential current treatment options for SS including metabolism, mechanism of action, overall efficacy in mycosis fungoides and SS, and common or concerning adverse effects is first discussed. The specific efficacy of each treatment for SS, both as monotherapy and combination therapy, is then reported using standardized criteria for both SS and response to therapy with the type of study defined by a modification of the US Preventive Services guidelines for evidence-based medicine. Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted.

Cutaneous γδ T-cell lymphomas: a spectrum of presentations with overlap with other cytotoxic lymphomas.

We reviewed our multicenter experience with gamma-delta (&ggr;&dgr;) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3+/CD4−/CD5−/CD8−/BF1−/&ggr;-M1+/TIA-1+/granzyme-B+/CD45RA−/CD7−, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous &ggr;&dgr; T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.

Percentage of γδ T Cells in Panniculitis by Paraffin Immunohistochemical Analysis

Cutaneous T-cell lymphomas with panniculitis-like histologic features have different clinical courses depending on whether they are composed of alphabeta T cells or gammadelta T cells, necessitating their distinction for proper prognostication. However, unlike alphabeta T cells, gammadelta T cells cannot be reliably detected in formalin-fixed, paraffin-embedded sections. We demonstrated that a commercially available antibody can detect gammadelta T cells and examined 2 cases of flow cytometry-proven gammadelta T-cell lymphomas and 15 control cases of nonneoplastic panniculitis. In both lymphomas, the atypical lymphocytes were gammadelta T cells, whereas the reactive lymphocytes were alphabeta T cells. In contrast, nonneoplastic panniculitis had predominantly alphabeta T cells with many fewer and individually scattered gammadelta T cells. The detection of gammadelta T cells in paraffin sections provides a powerful new tool to characterize T cells in lymphomas and inflammation.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Cutaneous T-cell lymphomas most commonly have a CD4(+) memory T-cell phenotype with relatively indolent course, but may in rare cases present with a CD8(+) cytotoxic phenotype exhibiting strikingly more aggressive clinical behavior. We present two cases of the clinically aggressive subtype of primary cutaneous epidermotropic CD8(+) cutaneous T-cell lymphoma and review the current literature, clinical behavior, and recommendations for treatment distinct from that of more common CD4(+) variants of cutaneous T-cell lymphoma.

Imiquimod use in the treatment of lentigo maligna

Dialogues in Dermatology, a monthly audio program from the American Academy of Dermatology, contains discussions between dermatologists on timely topics. Commentaries from Dialogues Editor-in-Chief Jacqueline M. Junkins-Hopkins, MD, are provided after each discussion as a topic summary and are provided here as a special service to readers of the Journal of the American Academy of Dermatology.

Dermatitis herpetiformis: Pearls and pitfalls in diagnosis and management

526 Dialogues in Dermatology, a monthly audio program from the American Academy of Dermatology, contains discussions between dermatologists on timely topics. Commentaries from Dialogues Editorin-Chief Jacqueline M. Junkins-Hopkins, MD, are provided after each discussion as a topic summary and are provided here as a special service to readers of the Journal of the American Academy of Dermatology. ( J Am Acad Dermatol 2010;63:526-8.)

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas

Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19–89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αβ T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αβ/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.

Hormone therapy for acne.

486 Dialogues in Dermatology, a monthly audio program from the American Academy of Dermatology, contains discussions between dermatologists on timely topics. Commentaries from Dialogues Editorin-Chief Jacqueline M. Junkins-Hopkins, MD, are provided after each discussion as a topic summary and are provided here as a special service to readers of the Journal of the American Academy of Dermatology. ( J Am Acad Dermatol 2010;62:486-8.)

Malignant melanoma: Molecular cytogenetics and their implications in clinical medicine

From D tio The th Fund Conf Repr D D St 0190 a 20 doi:1 Dialogues in Dermatology, a monthly audio program from the American Academy of Dermatology, contains discussions between dermatologists on timely topics. Commentaries from Dialogues Editor-inChief Jacqueline M. Junkins-Hopkins, MD, are provided after each discussion as a topic summary and are provided here as a special service to readers of the Journal of the American Academy of Dermatology. ( J Am Acad Dermatol 2010;63:329-32.)

Myometrial myxoidosis: a report of 2 cases of a distinctive type of secondary myometrial hypertrophy in patients with lupus erythematosus.

Myxoid mesenchymal lesions of the uterus are generally restricted to tumors, but non-neoplastic myxoid mesenchymal lesions of the uterus have not received much attention in the literature. We analyzed the clinicopathologic features of 2 patients with lupus erythematosus (ages 43 and 52 yr, respectively) in whom myometrial myxoidosis produced a markedly enlarged uterus with myometrial thickening (“secondary myometrial hypertrophy”). Both patients underwent a hysterectomy for presumed leiomyomas, and intraoperatively an enlarged uterus was noted. On gross examination, the uteri measured 13.5×13.5×11.5 cm and 14.5×11.5×9.5 cm, respectively. The significantly thickened myometrium was due to marked expansion of the interstitial compartment of the myometrium, in which non-neoplastic smooth muscle fascicles were widely separated by abundant extracellular mucin producing a striking myxoid appearance (“myxoidosis”). These histologic findings are akin to the pattern of dermal mucin deposition seen in lupus erythematosus. The lesion in each case diffusely involved the entire myometrium. Histochemical stains were performed and showed the following results: mucicarmine—diffusely but weakly positive; periodic acid-schiff (PAS)—negative; colloidal iron—diffuse positive; alcian blue, pH 2.5 (without hyaluronidase digestion)—diffuse positive, and alcian blue, pH 2.5 (with hyaluronidase digestion)—negative. These histochemical findings are consistent with hyaluronic acid. Follow-up in 1 case was not available. In the other case, the patient presented to clinical attention 5 weeks after surgery because of ascites, which after an extensive clinical evaluation was interpreted as being of unknown etiology. To the best of our knowledge, this rare and unusual non-neoplastic myometrial lesion has not been previously described. Pathologists should be aware of its existence because of the distinctive appearance and as it may prompt consideration of various myxoid neoplasms of the uterus in the differential diagnosis. Patients with myometrial myxoidosis should be evaluated for lupus erythematosus.