The 2016 revision of the World Health Organization classification of lymphoid neoplasms

The relative frequencies of cutaneous metastases are similar to those of the primary cancers; breast, colon, and melanoma are the most frequent in women and lung, colon, and melanoma are the most common in men. Cutaneous metastases represent an opportunity to detect a potentially treatable cancer before other evidence of it is present, to modify therapy as appropriate to the tumor stage, or possibly to use the cutaneous lesion as a source of easily accessible tumor cells for specific therapy. Cutaneous metastatic disease as the first sign of internal cancer is most commonly seen with cancer of the lung, kidney, and ovary.

Cutaneous metastatic disease.

The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and α 1 -antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Panniculitis. Part II. Mostly lobular panniculitis.

/pdf/the-international-consensus-classification-of-mature-13upp7d3.pdf

The International Consensus Classification of Mature Lymphoid Neoplasms: A Report from the Clinical Advisory Committee.

Skin metastases occur in 0.6%-10.4% of all patients with cancer and represent 2% of all skin tumors. Skin metastases from visceral malignancies are important for dermatologists and dermatopathologists because of their variable clinical appearance and presentation, frequent delay and failure in their diagnosis, relative proportion of different internal malignancies metastasizing to the skin, and impact on morbidity, prognosis, and treatment. Another factor to take into account is that cutaneous metastasis may be the first sign of clinically silent visceral cancer. The relative frequencies of metastatic skin disease tend to correlate with the frequency of the different types of primary cancer in each sex. Thus, women with skin metastases have the following distribution in decreasing order of frequency of primary malignancies: breast, ovary, oral cavity, lung, and large intestine. In men, the distribution is as follows: lung, large intestine, oral cavity, kidney, breast, esophagus, pancreas, stomach, and liver. A wide morphologic spectrum of clinical appearances has been described in cutaneous metastases. This variable clinical morphology included nodules, papules, plaques, tumors, and ulcers. From a histopathologic point of view, there are 4 main morphologic patterns of cutaneous metastases involving the dermis, namely, nodular, infiltrative, diffuse, and intravascular. Generally, cutaneous metastases herald a poor prognosis. The average survival time of patients with skin metastases is a few months. In this article, we review the clinicopathologic and immunohistochemical characteristics of cutaneous metastases from internal malignancies, classify the most common cutaneous metastases, and identify studies that may assist in diagnosing the origin of a cutaneous metastasis.

Cutaneous metastases from internal malignancies: a clinicopathologic and immunohistochemical review.

Hydroa Vacciniforme-Like Lymphoma: A Chronic EBV+ Lymphoproliferative Disorder With Risk to Develop a Systemic Lymphoma

Background : Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis. Objective : Our purpose was to characterize and differentiate our patients' disease from HV. Methods : We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV". Results : The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV. Conclusion : Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.

Edematous, scarring vasculitic panniculitis: A new multisystemic disease with malignant potential

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.

Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital.

Background Silvery hair and severe dysfunction of the central nervous system (neuroectodermal melanolysosomal disease or Elejalde syndrome) characterize this rare autosomal recessive disease. Main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement (seizures, severe hypotonia, and mental retardation). Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. Differential diagnosis with Chediak-Higashi syndrome and Griscelli syndrome must be done. Observations We studied pediatric patients with silvery hair and profound neurologic dysfunction. Immune impairment was absent. Age of onset of neurologic signs ranged from 1 month to 11 years; the signs included severe muscular hypotonia, ocular alterations, and seizures. Mental retardation since the first months of life was noted in 4 cases. Psychomotor development was normal in 3 cases, but suddenly the patients presented with a regressive neurologic process. Four patients died between 6 months and 3 years after the onset of neurologic dysfunction. One patient showed characteristic ultrastructural findings of Elejalde syndrome. Conclusions Elejalde syndrome is different from Chediak-Higashi and Griscelli syndrome and is characterized by silvery hair and frequent occurrence of fatal neurologic alterations. Psychomotor impairment may have 2 forms of presentation: congenital or infantile. Although Elejalde syndrome and Griscelli syndrome are similar, the possibility that they are 2 different diseases, although probably allelic related, is suggested.

https://jamanetwork.com/journals/jamadermatology/articlepdf/477721/dob8029.pdf

Elejalde syndrome--a melanolysosomal neurocutaneous syndrome: clinical and morphological findings in 7 patients.

The case is presented of a 3-year-old girl with urticaria and pressure dermographism. The condition began the age of one year. A skin biopsy confirmed the suspected diagnosis of diffuse mastocytosis. The identification of the form of mastocytosis without skin lesions and with dermographism and pressure urticaria as its only signs is important. It should be considered in any chronic urticaria appearing at birth or in an early age which is unresponsive to the usual symptomatic medication.

Cecilia Ridaura

Papers

Hydroa Vacciniforme-Like Lymphoma: A Chronic EBV+ Lymphoproliferative Disorder With Risk to Develop a Systemic Lymphoma

Edematous, scarring vasculitic panniculitis: A new multisystemic disease with malignant potential

Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital.

Elejalde syndrome--a melanolysosomal neurocutaneous syndrome: clinical and morphological findings in 7 patients.

Diffuse dermographic mastocytosis without visible skin lesions