ABSTRACT
The CD30-postive lymphoproliferative disorders, including lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, account for up to 30% of all cutaneous T-cell lymphomas (CTCLs) and are the second most common form of CTCLs after mycosis fungoides. Both conditions differ in their clinical presentations; however, they share the expression of the CD30 antigen as a common immunophenotypic hallmark. There is a wide spectrum of management options depending on factors such as extent of disease, staging and treatment tolerability. This Clinical Practice Statement is reflective of the current clinical practice in Australia.
Subject(s)
Lymphomatoid Papulosis , Lymphoproliferative Disorders , Skin Neoplasms , Humans , Australia , Ki-1 Antigen/metabolism , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/therapy , Lymphomatoid Papulosis/pathology , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Skin Neoplasms/pathologyABSTRACT
BACKGROUND AND PURPOSE: Primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder (PCSMLPD) is a benign behaving condition, typically manifesting as solitary head or neck papules, frequently creating cosmetic concerns. Optimal management of this rare disease is unclear. Herein, patterns of care and treatment outcomes are described, with particular focus on low-dose RT. MATERIALS AND METHODS: Eligibility required biopsy-proven PCSMLPD on central review, diagnosed between 2007-2022. Patterns of care, treatment responses and relapse patterns were assessed. Freedom-from-progression (FFP) was compared between RT and surgery. RESULTS: 41 patients were eligible. First-line treatments were: RT, 19 (46.3 %); surgery, 17 (41.5 %) (3 received adjuvant RT); watchful waiting, 5 (12.2 %). Median follow-up was 37.7 months. Overall, 24 patients received RT (19 definitive first-line, 3 adjuvant, 2 second-line). 10 (42 %) received 4 Gy in 2 fractions (with no acute toxicities); 14 (58 %) received 20-40 Gy. Complete response rate was 100 %. No post-RT relapses observed. After first-line surgery alone (n = 14, 3 with positive margins), 4 (28.5 %) experienced relapse (2 local, 2 distant). Watchful-waiting (n = 5) led to partial resolution post-biopsy in 4 patients; no complete resolution seen. 3-year FFP for RT alone was 100 % vs 61 % for surgery alone (p = 0.12). CONCLUSION: RT is a successful, non-invasive option for PCSMLPD: 100 % achieved complete response, with no relapses, and FFP appearing numerically superior to surgery in this cohort. In this first series of low-dose RT for PCSMLPD, 4 Gy in 2 fractions appears an excellent treatment option, offering durable disease control, no acute toxicities and convenient treatment time of only 2 days.
Subject(s)
CD4-Positive T-Lymphocytes , Humans , Follow-Up Studies , Treatment Outcome , Remission Induction , RecurrenceABSTRACT
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).
Subject(s)
Mycosis Fungoides/diagnosis , Mycosis Fungoides/therapy , Sezary Syndrome/diagnosis , Sezary Syndrome/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Biopsy , Hematologic Tests , Humans , Mycosis Fungoides/mortality , Neoplasm Staging , Prognosis , Sezary Syndrome/mortality , Skin/pathology , Skin Neoplasms/mortality , Survival RateSubject(s)
Anti-Inflammatory Agents/therapeutic use , Azathioprine/therapeutic use , Immunoglobulin G/blood , Immunosuppressive Agents/therapeutic use , Prednisolone/therapeutic use , Skin Diseases/drug therapy , Drug Therapy, Combination , Facial Dermatoses/drug therapy , Facial Dermatoses/immunology , Female , Humans , Middle Aged , Skin Diseases/immunologyABSTRACT
Multiple dermatofibromas is a rare entity consisting of more than fifteen lesions. Multiple clustered dermatofibroma is a distinct variant of multiple dermatofibromas and is defined as a well-demarcated plaque composed of individual dermatofibromas. We report a 16-year-old boy with multiple clustered dermatofibroma in a segmental distribution, which has previously not been reported in the literature.
Subject(s)
Histiocytoma, Benign Fibrous/pathology , Neoplasms, Multiple Primary/pathology , Skin Neoplasms/pathology , Adolescent , Histiocytoma, Benign Fibrous/diagnosis , Humans , Male , Neoplasms, Multiple Primary/diagnosis , Skin Neoplasms/diagnosisABSTRACT
Terminal osseous dysplasia with pigmentary defects (TOD) is an extremely rare X-linked dominant disorder, which is characterised by cutaneous digital fibromas, pigmentary skin defects and skeletal abnormalities. A single mutation in the last nucleotide of exon 31 of the filamin A gene (FLNA) has recently been identified as a cause of the disease. We describe a case of an 18-month-old girl with the clinical phenotype of TOD and the disease-specific FLNA mutation confirmed by genetic testing. This report highlights the importance of recognising this distinct phenotype that can present to a wide variety of health-care professionals, and reviews the spectrum of filamin A disorders.
Subject(s)
Dermatitis, Allergic Contact/etiology , Dermatitis, Atopic/chemically induced , Gloves, Protective/adverse effects , Hand Dermatoses/chemically induced , Latex Hypersensitivity/etiology , Rubber/adverse effects , Adult , Australia/epidemiology , Dermatitis, Allergic Contact/diagnosis , Dermatitis, Atopic/diagnosis , Dermatitis, Occupational/diagnosis , Dermatitis, Occupational/etiology , Female , Hand Dermatoses/diagnosis , Humans , Latex Hypersensitivity/diagnosis , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
A 24-year-old man with a long history of severe atopic eczema presented with a marked exacerbation requiring hospital admission. It emerged that his occupation as an animal house technician required him to work closely with laboratory animals, particularly mice and rats. Radioallergosorbent tests to mice allergens were markedly elevated. Avoidance of animal work, in conjunction with medical treatment, resulted in a marked improvement of his eczema.