To our knowledge, the present case seems to be the first description of pagetoid reticulosis presenting at the site of a previously treated dermal anaplastic large cell lymphoma. [ncbi.nlm.nih.gov]

Most patients present with solitary or localized nodules, papules, or plaques. However, up to 20% of patients may have multiple lesions. Ulceration may be present or not. [ijpd.in]

• Soft Tissue Mass

  Computed tomography (CT) scan showed the presence of soft tissue mass with irregular outlines arising from the skin. There was no nodal or any other organ involvement in the body. [jcytol.org]

• Axillary Pain

  In this report, we present an unusual case of an 8-year-old boy with a noduloulcerativelesion present on the upper back with axillary painful lymphadenopathy, initially mimicking as scrofuloderma which was later histopathologically and immunohistochemically [ijpd.in]

• Axillary Lymphadenopathy

  There was no cervical and axillary lymphadenopathy and hepatosplenomegaly. The other systemic examinations were unremarkable. Routine investigations revealed a normal hemogram and urine examination. [e-ijd.org]

• Pruritic Rash

  The lesions began as eczema located in upper and lower limbs that have evolved to a widespread scaly and quite pruritic rash with papules and nodules which ulcerated and spontaneously regressed, leaving permanent hypochromic stains ( Figures 1 and 2 ) [scielo.br]

• Skin Plaque

  Large solitary or multiple slow-growing erythematous skin plaques, nodules or tumors develop, in either a localized or multifocal distribution. These lesions may ulcerate and/or itch. [orpha.net]

• Neck Swelling

  (a) Ulcerated swelling in the right side of neck lifting the ear lobe. [cancerjournal.net]

A systemic workup of the patient excluded other potential causes. The neonate was treated with wide excision, but chemotherapy or radiation therapy was not administered, as the patient's parents did not consent to such treatment. [ncbi.nlm.nih.gov]

Distinguishing primary cutaneous anaplastic lymphoma (PC-ALCL) from its systemic counterpart requires a complete clinical and laboratory workup. [cancerjournal.net]

Histologic examination is the first diagnostic step in the diagnostic workup of clinically suspected CD30 + LPDs. [bloodjournal.org]

• Human Papillomavirus

  Detection of human herpesvirus 6 and human papillomavirus 16 in cervical carcinoma. Am J Pathol. 1994;145:1509–16. [infectagentscancer.biomedcentral.com]

• Multiple Ulcerations

  C-ALCL is characterized by the presence of single or multiple ulcerated lesions on the skin's surface. [ncbi.nlm.nih.gov]

  Case Report An 83-year-old man presented with multiple ulcerated plaques over face, trunk, both upper and lower extremities for 6 months. [jdrntruhs.org]

There are several perils and pitfalls that exist regarding the differential diagnosis, the possible progression, and the treatment of PC-ALCL. [ncbi.nlm.nih.gov]

Management and treatment If lesions are solitary or localized to a single area, excision and/or radiation therapy is performed. [orpha.net]

Nevertheless, PC-ALCL must be distinguished from secondary skin lesions in systemic ALCL, which confer a poor prognosis, and other CD30+ PCLPDs, reactive conditions, or borderline cases. [ncbi.nlm.nih.gov]

Prognosis The prognosis is generally good, with a five-year survival rate of 90%. In 25% of cases, spontaneous regression is observed. Relapses, generally without any extracutaneous dissemination, may sometimes occur (approximately in 30% of cases). [orpha.net]

Usually favorable prognosis (10 year disease related survival of 90%) May show partial or complete spontaneous regression of skin lesions (10% - 40%) Frequent skin relapses Multifocality / nodal involvement have a similar prognosis as skin only lesions [pathologyoutlines.com]

Etiology Etiology is unknown. [orpha.net]

The systemic and primary cutaneous types of anaplastic large cell lymphomas have been differentiated on clinical and immunophenotypical findings, but further research is required to elucidate their exact etiologies and pathogeneses. [ncbi.nlm.nih.gov]

Background: The etiology of primary cutaneous anaplastic large-cell CD30+ lymphoma is largely unknown, and although an infectious involvement has been suspected, the implication of infectious agents in its pathogenesis is still unclear. [adolfosebastiani.it]

[…] cutaneous anaplastic large cell lymphoma (C-ALCL) Synonyms Primary cutaneous CD30(+) T-cell lymphoproliferative disorder This term also includes lymphomatoid papulosis Definitions Cutaneous lymphoma composed of large T cells that express CD30 (> 75%) ETIOLOGY [basicmedicalkey.com]

Summary Epidemiology The prevalence of primary C-ALCL is unknown but it accounts for approximately 9% of cutaneous lymphomas. The male/female ratio is of 3:2. [orpha.net]

Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma. [atlasgeneticsoncology.org]

[…] pleomorphic or immunoblastic cytomorphology that express the CD30 antigen by the majority (> 75%) of the tumor cells ( WHO 2008 ) Terminology Also called regressing atypical histiocytosis (European Organisation for Research and Treatment of Cancer) Epidemiology [pathologyoutlines.com]

Epidemiology and Mortality Age: 60 years median age (can occur in children, although rare) Race: female predominance Sources Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds): WHO Classification of Tumours of Haematopoietic [seer.cancer.gov]

[…] immunosuppressive drugs Gene expression profiling has failed to show genes that clearly distinguish C-ALCL from ALK(-) systemic ALCL Increased expression of skin-homing chemokine receptors may play a role in confining C-ALCL to skin CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Pathophysiology ALK positive and ALK negative ALCL have different gene expression profiles and seem to be biologically distinct entities. The pathophysiology of both subtypes of ALCL is incompletely understood. [cancertherapyadvisor.com]

As such histopathologic findings often represent close mimickers of neoplastic and infectious etiologies, knowledge of the potential for systemic manifestations of taxane therapy in patients treated topically may prevent potentially costly diagnostic [ncbi.nlm.nih.gov]

How can Primary Cutaneous Anaplastic Large Cell Lymphoma be Prevented? Presently, the cause of Primary Cutaneous Anaplastic Large Cell Lymphoma is unknown and there are no known methods to prevent its formation. [dovemed.com]

None of the available therapies for LYP appears unequivocally effective in preventing LYP-associated second lymphomas, although this point has not been systematically addressed in prospective studies. [bloodjournal.org]

Ruling out these conditions will prevent unnecessarily aggressive treatment for primary cutaneous CD30+ LCL, an indolent disease with a very favourable prognosis. [jpma.org.pk]

A good skincare regime with regular use of moisturisers helps to prevent dryness and irritation. If you have stage 1 disease, this may be all you need. [lymphomas.org.uk]