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.

Objectives To determine the pathogenic association of latent Epstein-Barr virus (EBV) infections with both typical hydroa vacciniforme (HV) and severe HV-like eruptions, and to survey the complications and outcomes of patients. Design Case series. Patients Twenty-nine patients with HV or severe HV-like eruptions. Interventions In situ hybridization and immunostaining of biopsy specimens; extraction of DNA samples from cutaneous lesions and/or peripheral blood mononuclear cells for EBV DNA assay. Main Outcome Measures Clinicopathologic manifestations, hematologic findings, complications, and outcomes; presence of latent EBV infection. Results T cells positive for EBV-encoded small nuclear RNA (EBER) were detected, to various degrees, in cutaneous infiltrates in 28 (97%) of 29 patients, including all 6 patients with definite HV with a positive phototest reaction, 11 of 12 patients with probable HV without evidence of photosensitivity, and all 11 patients with severe HV associated with systemic symptoms. In addition to EBER-positive T cells, many cytotoxic T lymphocytes expressing T-cell intracellular antigen 1 and granzyme B were present in the cutaneous lesions. Natural killer (NK) cells were absent or at a background level. The UV-induced cutaneous lesions showed histopathologic findings consistent with those of HV, containing many EBER-positive cells. Although no hematologic abnormalities were found in the definite and probable HV groups, the amounts of EBV DNA were increased in the peripheral blood mononuclear cells. By contrast, the severe HV group had markedly increased levels of EBV DNA associated with NK-cell lymphocytosis, and complications including chronic active EBV infection, hypersensitivity to mosquito bites, and hemophagocytic syndrome. Five patients with severe disease died of EBV-associated NK/T-cell lymphomas or hemophagocytic syndrome 2 to 14 years after onset. Conclusion Both typical and severe HV are included within the spectrum of cutaneous disorders mediated by EBV-infected T cells, and the severe HV group may have overt EBV-associated NK/T-cell lymphoproliferative disorders with a frequently fatal outcome.

https://jamanetwork.com/journals/jamadermatology/articlepdf/405178/DST50031.pdf

Pathogenic link between hydroa vacciniforme and Epstein-Barr virus-associated hematologic disorders.

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

Disorders of Connective Tissue

Background: Angiocentric cutaneous T-cell lymphomas of childhood (ACTCLC) are an unusual type of T-cell lymphomas that present with a vesiculopapular eruption mimicking hydroa vacciniforme. Most patients have been children from Asia and Latin America. Objective: The purpose of this study was to describe four cases of ACTCLC; to discuss its clinical, histopathologic, and immunohistochemical features; to consider its possible relationship to the Epstein-Barr virus (EBV); and to clarify its classification within the spectrum of angiocentric lymphomas. Methods: The clinical, histopathologic, and immunohistochemical features of four cases of ACTCLC were identified and analyzed. In addition in situ hybridization for EBV was performed in all cases. Results: The clinical features were similar to previous cases reported under different names, such as hydroa-like lymphomas, edematous, scarring vasculitic panniculitis. Histologically, all showed angiocentric infiltrates composed mainly of T cells. In all cases there were variable numbers of CD30+ cells. The EBV was present in three of the cases. Conclusion: ACTCLC is a distinct type of T-cell lymphoma. It affects mainly children, and the EBV appears to play a role in the pathogenesis of this disease. (J Am Acad Dermatol 1998;38:574-9.)

https://www.jaad.org/article/S0190-9622(98)70120-3/pdf

Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): A distinctive type of cutaneous T-cell lymphoma

Ten years after the onset of hydroa vacciniforme (HV), a 16-year-old boy developed edema and red induration of the face, ears, and dorsa of the hands. Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase of several serum enzymes. The vesicle was situated intraepidermally with thrombosis and hemorrhage underneath. This confirmed the diagnosis of HV. In addition, dense cell infiltrate was seen in deep dermis and subcutaneous tissue. Histologic and immunohistochemical studies of the dermal cell infiltrate and lymph node showed an infiltrate of helper T lymphocytes with an atypia and histiocytic cells (S100[-], alpha-subunit[+]). Hence, we concluded HV and malignant lymphoma coexisted in this patient.

Coexistence of Hydroa Vacciniforme and Malignant Lymphoma

To the Editor.— The most important dermatologic problem of prolidase deficiency1is chronic recurrent leg ulcers that are recalcitrant in healing. We used 5% glycine-5% proline ointment on leg ulcers with excellent results. Report of a Case.— The patient, now a 22-year-old woman, is the same patient as described previously.1She had never been free of recalcitrant leg ulcers despite various conventional therapies including topical application of antibiotics (gentamicin sulfate, colistin sulfate, polymyxin B sulfate, etc), leg bath in 0.01% potassium permanganate solution, oral administration of zinc sulfate (180 mg daily), and/or ascorbic acid (3 g daily). Oral L-proline (1 g daily) was also tried for two months with no apparent benefit. We decided to use topical proline and then a combination of topical proline and glycine on her leg ulcers. L-proline and/or L-glycine ground with a mortar and pestle was mixed in liquid paraffin (30 g) and

Effect of Topical Application of Glycine and Proline on Recalcitrant Leg Ulcers of Prolidase Deficiency

A rare case of congenital alveolar rhabdomyosarcoma revealing multiple skin metastases in a female neonate is reported. At birth, a ping-pong ball-sized tumor on the neck and a tumor the size of a little finger end on the chin were noticed. Then, multiple skin tumors over the whole body occurred soon after birth. A biopsied small skin tumor was at first interpreted as being compatible with metastatic congenital neuroblastoma. However, immunohisto-chemical and electron microscopic findings revealed positive immunoreactivity for myoglobin in a few tumor cells and the presence of a few rhabdomyoblasts among poorly differentiated tumor cells, resulting in a final diagnosis of alveolar rhabdomyosarcoma. Therefore, it should be emphasized that in cases of round cell tumor, immunohistochemical and ultrastructural studies are imperative in order to identify the tumor and differentiate it from other forms, including rhabdomyoblastoma, neuroblastoma, Ewing's sarcoma, malignant lymphoma, and small cell carcinoma. ACTA PATHOL JPN 38: 241–248, 1988.

Congenital alveolar rhabdomyosarcoma with multiple skin metastases. Report of a case.

To the Editor.— Bloom's syndrome is a rare autosomal recessive disorder characterized by photosensitive telangiectatic erythema of the face, stunted growth, a predisposition to malignancy, and increased sister chromatid exchanges (SCEs).1,2We describe a 34-year-old man with Bloom's syndrome whose phytohemagglutinin-stimulated lymphocytes are composed of two populations,5,6one with normal SCE frequency (30%) and the other with increased SCE frequency (70%). Report of a Case.— A man, aged 34 years, was referred to Kochi (Japan) Medical School Hospital for evaluation of anemia and diabetes mellitus. The three generations down to his parents are densely consanguineous. He was born of a full-term delivery, and his birth weight was 1500 g. Four months after birth, erythema of the face appeared. He had recurrent episodes of otitis media and otitis externa during his childhood. When he was 19 years old, he was found to have diabetes mellitus. Physical examination showed diffuse

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=DERM&articleId=548473&pdfFileName=archderm_123_8_003.pdf

Jirô Arata

Papers

Coexistence of Hydroa Vacciniforme and Malignant Lymphoma

Effect of Topical Application of Glycine and Proline on Recalcitrant Leg Ulcers of Prolidase Deficiency

Congenital alveolar rhabdomyosarcoma with multiple skin metastases. Report of a case.

Bloom's syndrome with dimorphism of sister chromatid exchanges in phytohemagglutinin-stimulated lymphocytes.

Corticosteroid treatment effective for 17 patients with high titers of antiRNP antibody.