Mycosis fungoides and Sézary syndrome: Australian clinical practice statement.

Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T-cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following (http://www.australasianlymphomaalliance.org.au).

Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy.

Numerous systemic treatment options exist for patients with mycosis fungoides (MF) and Sézary syndrome (SS), but no large comparative studies are published. To study the efficacy of treatments, a retrospective analysis of our cutaneous lymphoma database was undertaken, with 198 MF/SS patients undergoing systemic therapies. The primary end point was time to next treatment (TTNT). Patients with advanced-stage disease made up 53%. The median follow-up time from diagnosis for all alive patients was 4.9 years (range 0.3-39.6), with a median survival of 11.4 years. Patients received a median of 3 lines of therapy (range 1-13), resulting in 709 treatment episodes. Twenty-eight treatment modalities were analyzed. The median TTNT for single- or multiagent chemotherapy was only 3.9 months (95% confidence interval [CI] 3.2-5.1), with few durable remissions. α-interferon gave a median TTNT of 8.7 months (95% CI 6.0-18.0), and histone deacetylase inhibitors (HDACi) gave a median TTNT of 4.5 months (95% CI 4.0-6.1). When compared directly with chemotherapy, interferon and HDACi both had greater TTNT (P < .00001 and P = .01, respectively). This study confirms that all chemotherapy regimens assessed have very modest efficacy; we recommend their use be restricted until other options are exhausted.

Radiation therapy-induced neuro-Sweet disease in a patient with oral squamous cell carcinoma.

Neurological involvement is a rare extracutanenous manifestation of Sweet's syndrome. We present a novel case of radiation therapy-induced neuro-Sweet disease in a patient receiving treatment for an oral squamous cell carcinoma.

Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers.

Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires thorough history taking and physical examination, in combination with skin biopsy. In some cases flow cytometry, molecular studies and imaging are also required in order to diagnose and stage the disease. Staging is derived from the tumour-node-metastasis-blood classification and is currently our best attempt to stratify prognosis and hence guide management in this complex disease. Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. Management remains heavily guided by staging, such that patients with early-stage disease generally begin treatment with skin-directed or local therapies and those with advanced-stage disease have many treatment options, including chemotherapy, the use of biological agents, local and total body radiotherapy, as well as haematopoietic stem cell transplantation. Besides staging, many other patient-related factors influence the treatment strategy, particularly where symptom relief is paramount. There are many challenges remaining in the study of Mycosis fungoides and Sézary syndrome and, given the rarity of the disease, concerted worldwide efforts are required to conduct efficient and effective research.

Cutaneous CD30 positive lymphoproliferative disorders with coexistent epithelial neoplasms: Report of two cases.

Primary cutaneous large cell anaplastic lymphoma (C-ALCL) and lymphomatoid papulosis (LyP) are cutaneous CD30+ lymphoproliferative disorders (CD30+ LPD). An association with CD30+ LPD and pseudoepitheliomatous hyperplasia has been recognized. Additionally, rare reports of epithelial neoplasms such as keratoacanthomas and squamous cell carcinomas (SCC) occurring in association with both C-ALCL and LyP have been reported. We report two cases of CD30+ LPD with associated epithelial neoplasms; one patient with a primary cutaneous CD30+ LPD and SCC identified within the same lesion, and the other with a keratoacanthoma arising in a lesion of LyP. The pathogenesis of this association is poorly understood although various hypotheses exist. Awareness of the coexistence of these entities will avoid misdiagnosis and incorrect treatment.

Cutaneous manifestations of peripheral T-cell lymphoma, not otherwise specified: a case series highlighting the diagnostic challenges for this heterogeneous group.

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a rare, heterogeneous group of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the defined T-cell entities, according to the World Health Organization classification. Most cases present with late stage nodal disease; however extranodal involvement is common. Skin and subcutaneous involvement is reported in approximately 20% of cases. Little attention has been given to the highly variable skin manifestations in the literature. It is our experience that lesions can present in ways other than previously described nodular or tumourous lesions that often ulcerate. We present a case series from a large tertiary institution of seven cases of PTCL, NOS with skin involvement, highlighting the variable presentations and diagnostic challenges for this heterogeneous group.

Food allergy testing in infantile eczema: a clinical approach and algorithm.

The complex relationship between food allergy and infantile eczema has prompted divergent approaches to investigating potential food triggers in eczematous patients. It is well recognised that a significant proportion of infants with eczema have immunoglobulin E-mediated food allergy, reported to range between 20-80%. Determining whether certain foods trigger an eczematous flare in individual infants with eczema is difficult. For all infants with eczema, good skin care is the mainstay of treatment but identifying and avoiding triggers (both allergic and non-allergic) is important in some infants. Given this, we have a developed an algorithm that can be used by dermatologists in the investigation and management of food allergies in infantile eczema. Issues such as patient selection, investigation and elimination diets are addressed, with reference to relevant evidence in the literature. Our aim is to provide dermatologists with a framework to manage food allergies in infantile eczema, allowing the problem to be addressed with confidence.

Success treatment of post-irradiation morphoea with acitretin and narrowband UVB.

Post-irradiation morphoea is a rare but under-recognised complication of radiotherapy treatment for breast cancer. Management of this condition is difficult, and many cases are recalcitrant to therapy. A 43-year-old woman with breast cancer received radiotherapy following a mastectomy and partial axillary lymph node dissection, shortly after which she developed a hot, tender, erythematous and indurated plaque at the mastectomy site. Subsequently the skin became retracted, depressed and hyperpigmented. The clinical features, along with histological findings, were consistent with post-irradiation morphoea. Treatment with narrowband ultraviolet B and acitretin 10 mg daily was commenced 5 years following radiotherapy. After 2 months of therapy the patient reported significant improvement in tenderness and range of left arm movement. Objectively the plaque was less indurated and softer to palpation. We propose that this treatment regimen is an option in the management of post-irradiation morphoea.