ABSTRACT
Lymphomatoid papulosis (LyP) is a benign condition, listed among primary cutaneous CD30+ lymphoproliferative disorders. Its typical picture consists of relapsing-remitting papular lesions and it can be encountered in the course of a hematologic disease, at times representing its first manifestation. Hypereosinophilic syndromes are a heterogeneous group of disorders characterized by persistent peripheral blood hypereosinophilia that may lead to life-threatening organ damage. Among eosinophilic disorders, the subtype identified as myeloid/lymphoid neoplasm with eosinophilia and tyrosine kinase gene fusions has aroused particular interest due to its excellent response to tyrosine kinase inhibitors, including imatinib. Here, we described the case of two 33-year-old men presenting with LyP and myeloid neoplasm with eosinophilia and FIP1L1::PDGFRA rearrangement who achieved complete clinical and molecular remission of both conditions a few months after starting imatinib.
Subject(s)
Hypereosinophilic Syndrome , Lymphomatoid Papulosis , Male , Humans , Imatinib Mesylate/therapeutic use , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/genetics , Receptor, Platelet-Derived Growth Factor alpha/genetics , Neoplasm Recurrence, Local/drug therapy , Hypereosinophilic Syndrome/diagnosis , Hypereosinophilic Syndrome/drug therapy , Hypereosinophilic Syndrome/genetics , Transcription Factors , Oncogene Proteins, Fusion/geneticsABSTRACT
The standard therapies for primary cutaneous anaplastic large cell lymphoma (pcALCL) in an advanced stage remain undefined. A 71-year-old man presented with multiple erythema and nodules. He was diagnosed with lymphomatoid papulosis (LyP) through a skin biopsy from the left postauricular area. All skin lesions achieved complete response by electron beam irradiation. However, nodular lesions appeared in both inner canthi 5 months later. Histopathological evaluation of the lesional biopsy revealed dominant infiltration of CD30-positive large cells. Positron emission tomography/computed tomography revealed fluorodeoxyglucose-positive cervical and inguinal lymph node swelling and right tonsillitis, followed by the diagnosis of pcALCL and TNM classification T3bN3M0. Since the patient had severe chronic obstructive pulmonary disease and recurrent pneumonia, he received low-dose methotrexate (MTX) (15 mg/week) therapy. Low-dose MTX effectively debulked the lymphadenopathies over time without particular adverse effects. Although the standard therapies for pcALCL are not established, low-dose MTX was effective and considered safe for patients with frailty and compromised respiratory function. Further study is warranted on the pathophysiology of pcALCL after the development of LyP and mechanisms of action of low-dose MTX against LyP and pcALCL.
Subject(s)
Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphomatoid Papulosis , Skin Neoplasms , Aged , Humans , Immunotherapy , Lymphoma, Primary Cutaneous Anaplastic Large Cell/drug therapy , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/pathology , Male , Methotrexate/therapeutic use , Skin Neoplasms/pathologyABSTRACT
ABSTRACT: Janus kinase (JAK) inhibitors are being prescribed with increasing regularity in dermatology. We report on a patient who initiated treatment with tofacitinib for refractory erythema elevatum diutinum and subsequently developed a novel cutaneous outbreak characterized by firm violaceous papules on the trunk and extremities along with conjunctival injection and periorbital inflammation. Biopsy of affected tissue from both the cutaneous and ophthalmologic sources demonstrated increased numbers of CD30+ large atypical cells amid a mixed inflammatory cell infiltrate, consistent with lymphomatoid papulosis. A review of the literature reveals a plausible mechanism for the induction of persistent JAK signaling in the presence of a JAK inhibitor. We discuss this mechanism in depth because it pertains to this patient and recommend continued vigilance with the use of these immunologic agents.
Subject(s)
Lymphomatoid Papulosis , Vasculitis, Leukocytoclastic, Cutaneous , Humans , Ki-1 Antigen , Lymphomatoid Papulosis/chemically induced , Lymphomatoid Papulosis/drug therapy , Piperidines/adverse effects , PyrimidinesSubject(s)
Dermatitis, Atopic , Lymphoma, Large-Cell, Anaplastic , Lymphomatoid Papulosis , Skin Neoplasms , Antibodies, Monoclonal, Humanized/adverse effects , Humans , Lymphoma, Large-Cell, Anaplastic/diagnosis , Lymphoma, Large-Cell, Anaplastic/drug therapy , Lymphoma, Large-Cell, Anaplastic/pathology , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/pathology , Skin Neoplasms/diagnosis , Skin Neoplasms/drug therapy , Skin Neoplasms/pathologySubject(s)
Hodgkin Disease/complications , Lymphomatoid Papulosis/diagnosis , Skin Neoplasms/diagnosis , Aged , Glucocorticoids/therapeutic use , Histamine Antagonists/therapeutic use , Humans , Lymphomatoid Papulosis/complications , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/pathology , Male , Neoplasm Recurrence, Local , Skin Neoplasms/complications , Skin Neoplasms/drug therapy , Skin Neoplasms/pathologyABSTRACT
Lymphomatoid papulosis (LyP) is a chronic skin condition, characterized by recurrent eruptions of papules and nodules with or without central necrosis that spontaneously resolve. This condition was originally described by Macaulay in 1968 as a self-healing rhythmical paradoxical eruption that was clinically benign yet histologically malignant.1 Clinically, it is defined by papules that wax and wane, are generally less than 1cm in diameter, and heal spontaneously after 68 weeks with subsequent scarring.2
Subject(s)
Lymphomatoid Papulosis/diagnosis , Skin Neoplasms/diagnosis , T-Lymphocyte Subsets , Administration, Cutaneous , Adult , Clobetasol/administration & dosage , Clobetasol/therapeutic use , Diagnosis, Differential , Female , Humans , Leg , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/pathology , Skin Neoplasms/drug therapy , Skin Neoplasms/pathologyABSTRACT
We report a case of a 13-year-old boy with extensive lymphomatoid papulosis (LyP) involving his elbows, forearms, proximal thighs, and right hip, with treatment-resistant nodules on his right forearm. He was treated with full-body narrowband ultraviolet B and targeted photodynamic therapy (PDT) with 20% aminolevulinic acid (ALA). After two months, there was complete resolution of the right forearm nodules. Due to its minimal toxicity, PDT offers unique advantages and may be considered for pediatric LyP patients with symptomatic, localized disease resistant to conventional treatments.
Subject(s)
Lymphomatoid Papulosis , Photochemotherapy , Skin Neoplasms , Adolescent , Child , Humans , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/drug therapy , Male , Skin Neoplasms/drug therapyABSTRACT
Lymphomatoid papulosis (LyP) is a benign skin condition that typically presents with grouped or scattered lesions on the body that self-resolve within weeks or months of onset. LyP belongs to the group of CD30-positive lymphoproliferative disorders. Several histological variants of LyP exist, and the histological features of LyP can overlap with other lymphoproliferative disorders; therefore, both histological and clinical correlations are needed for a proper diagnosis of LyP. We report an unusual case of LyP displaying a T follicular helper cell-like phenotype and histopathologically resembling the primary cutaneous CD4-positive small-sized to medium-sized T-cell lymphoproliferative disorder.
Subject(s)
Lymphomatoid Papulosis/pathology , Neoplasms, Multiple Primary/pathology , Skin Neoplasms/pathology , T Follicular Helper Cells/pathology , Clobetasol/therapeutic use , Glucocorticoids/therapeutic use , Humans , Lymphomatoid Papulosis/drug therapy , Male , Middle Aged , Neoplasms, Multiple Primary/drug therapy , Phenotype , Skin Neoplasms/drug therapyABSTRACT
Lymphomatoid papulosis (LyP) is a self-limiting cutaneous T-cell lymphoproliferative disorder that may progress into malignant lymphoma. Most of the previously reported associated lymphomas are primary cutaneous anaplastic large-cell lymphoma and mycosis fungoides with a low mortality rate. We report a case of primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS), associated with LyP after long-term follow up. The patient was a 79-year old Japanese man followed up for 9 years. He suddenly developed a 3-cm ulcerated lesion on his forehead, which was diagnosed as an exacerbation of LyP. The lesion regressed after conservative treatment, but the patient soon developed multifocal pcPTCL-NOS. Thereafter, the patient developed pneumonia and cerebral infarction and died within a few months of the onset of malignant lymphoma. Aggressive cutaneous lymphoma may develop in LyP patients. The present case re-emphasizes the need for careful follow up of patients with persistent LyP.
