Multiple minute digitate hyperkeratosis - a peculiar entity

Multiple minute digitate hyperkeratosis is a rare, non-follicular dermatosis, with fewer than 30 cases described worldwide. It can be either acquired or inherited in an autosomal dominant pattern. We describe the case of an 83-year old patient with life-long, multiple, digitate, milimetric lesions, and a positive family history for the same dermatosis.

Eritroqueratodermia simétrica progresiva: seguimiento prolongado de un paciente / Erythrokeratodermia progressive symmetrical: extended follow-up of a patient

La eritroqueratodermia simétrica progresiva (EQSP) es una genodermatosis rara que se manifiesta por placas queratósicas con base eritematosa, simétricas, policíclicas, localizadas principalmente en las extremidades. Se presenta un caso de un varón de 16 años, controlado desde el nacimiento, con un gran porcentaje de la superficie cutánea afectada.

The progressive symmetrical erythrokeratodermia (EQSP) is a rare genodermatosis manifested by symmetric erythematous and keratotic plaques, polycyclic, located mainly on the limbs. We report a case of a 16 years old boy, controlled from birth, with a large percentage of skin surface affected.

Parapsoriasis retiforme extensa o micosis fungoide: presentación de un caso / Extensive retiform parapsoriasis or mycosis fungoides: case report

La parapsoriasis retiforme es una variedad muy rara de parapsoriasis en grandes placas, también considerada como un estadio premicotico. Se caracteriza por presentarse como pápulas milimétricas que confluyen formando placas reticuladas eritemato violaceas con piel corrugada y finamente descamativa entre ellas con apariencia poiquilotermia. Se presenta el caso de un paciente anciano con compromiso extenso de la superficie corporal.

Retiform parapsoriasis is a very rare variety of large plaque parapsoriasis. It was also considerate by some authors like a premycotic state. This entity its characterized by present like milimetric papules that confluid to form eritemato-violaceous plaques that have between poikilodermatous skin. We present a case of an older man with wide compromise of body superface area.

Micosis fungoide hipopigmentada: reporte de un caso / Hypopigmented mycosis fungoides: a case report

La micosis fungoide es el linfoma cutáneo más frecuente. Tiene un curso usualmente crónico que pasa por estadios de macular, de placas y tumoral. También se han reportado muchas variantes poco frecuentes. La micosis fungoide hipopigmentada es una de ellas con solo un poco más de un centenar de casos reportados, la mayoría en personas de piel oscura y principalmente adolescentes y adultos jóvenes. Se presenta el caso de un paciente varón de mediana edad con largo tiempo de evolución.

Mycosis fungoides is the most frequent cutaneous lymphoma. Its curse is usually chronic and go through patches, plaques and tumoral stadies. Many variants less frequents has been reported. Hypopigmented mycosis fungoides is one ofthem and it have just a little more than a hundred of cases reported, especially in people of dark skin, young adults and teenagers. This occasion we present a case of a man in the middle age with long evolution.

Varón joven con lesiones eritemato-escamosas de larga evolución / Young male with long standing erythemato-squamous lesions

La parapsoriasis agrupa un número de estados patológicos caracterizados por lesiones eritemato-escamosas recalcitrantes en piel que no llenan los criterios de malignidad, y reflejan situaciones intermedias. Presentamos el caso de un varón joven con lesiones eritemato-escamosas de largo tiempo de evolución sin alteraciones del estado general y sin compromiso visceral. Hacemos una breve revisión clínica de la enfermedad y sus hallazgos histopatológicos.

Parapsoriasis brings together a number of pathological conditions characterized by erythemato-squamous skin lesions that do not meet the criteria for malignancy, and reflect intermediate situations. We present the case of a young male with erythemato-squamous lesions of long time of evolution, without alterations of the general statement and without visceral compromise. We make a brief review of the clinical disease and its pathological findings.

Dermatosis digitada: presentación de un caso / Digitate dermatosis: a case presentation

Se presenta el caso de un hombre de 43 años de edad con placas eczematosas de ocho años de evolución. Las placas, de color amarillo, se encontraban localizadas en el tronco. La biopsia muestra como hallazgo principal la presencia de linfocitos formando una fila india en la capa basal. Se diagnostica dermatosis digitada y se plantean las dos tendencias etiológicas: un proceso inflamatorio o uno neoplásico.

CD7 expression in differentiating mycosis fungoides from benign cutaneous lymphocytic infiltrates

Diagnosis of the early phase of mycosis fungoides [MF] is sometimes difficult. Loss of CD7 expression is considered a distinguishing characteristic of MF. The aim of this study was to determine the range of CD7 expression in MF and compare the results with benign inflammatory dermatosis and equivocal cases of possible MF. During a period of 30 months, we examined 15 patients with MF, 12 patients suspicious for MF, and 15 patients with benign inflammatory dermatosis. The slides stained by H andE were reviewed by two pathologists. Immunostaining was done for CD43, CD3, CD5, CD7, and CD20 on paraffin embedded tissues. All the patients in MF group showed absence of CD7 expression in epidermotropic mycosis cells. Compared with benign inflammatory dermatosis, patients with MF had significantly lower CD7 expression in the dermal infiltrate [P< 0.0001]. In patients with MF, the mean CD7 was significantly lower than CD43, CD3, and CD5 [P=0.001]. The mean CD7 count of parapsoriasis was significantly higher than MF [P= 0.01]. The mean CD7 count of parapsoriasis was significantly lower than benign inflammatory dermatosis [P=0.016]. The lowest mean CD7 counts were found in patch stage of MF. Low CD7 expression < 10% lymphocytes had sensitivity and positive predictive values of 75% and 100% and specificity and negative predictive values of 100% and 83.3% for the diagnosis of patch stage of MF. Minimal expression of CD7 is a specific finding for patch stage of MF. Benign inflammatory dermatosis can also show low expression of this marker, but rarely matches that of patch stage of MF

Nevo epidérmico verrugoso inflamatorio lineal (NEVIL): a propósito de un caso / Linear inflamatory verrucous epidermal nevus (LIVEN): about a case

El nevo epidérmico verrugoso inflamatorio lineal, se caracteriza clínicamente por lesiones eritematoescamosas de aspecto psoriasiforme, pruriginosas. Su frecuencia es mayor en la primera infancia. Se presenta el caso de una paciente de 5 años de edad en la que aparece la lesión dérmica desde su nacimiento en extremidad superior izquierda.

Cutaneous manifestations in renal failure patients: a case series.

Cutaneous involvement in renal disease is due to a host of factors ranging from metabolic disturbances to immunosuppressive drugs. Herein we report a series of six cases of renal failure with varied cutaneous manifestations ranging from infections to neoplasms due to prolonged immunosuppression. Our first case had cutaneous cryptococcosis where skin lesions gave a clue to the diagnosis of altered sensorium and underlying meningitis. The second case initially presented with florid warts and was treated successfully but later presented with an explosive recurrence of skin lesions due to malignant transformation. Our third case had basal cell carcinoma over the presternal region that was successfully treated with liquid nitrogen cryotherapy. Our fourth case had diabetic nephropathy that presented with septicemia and purpura fulminans. The last case had cutaneous manifestations of drug therapy because of heparin infusion. To conclude, cutaneous manifestations in patients with renal failure are varied and a high degree of suspicion is needed for early diagnosis and aggressive treatment to effectively combat mortality and morbidity.

[Ichthyosiform parapsoriasis: report of a case]

Acquired ichthyosis is a known paraneoplastic sign of lymphoproliferative malignancies with nonspecific histopathologic findings revealing no implication of the underlying neoplasm. However, ichthyosiform eruption is considered as a specific manifestation of mycosis fungoides [MF], i.e., ichthyosiform MF. There are a few reports of ichthyotic manifestation of MF, but only one report of this presentation in parapsoriasis. We present a case of ichthyosiform parapsoriasis in a 22-year-old woman with hyperpigmented ichthyosiform scaly patches on her trunk and extremities. The histopathologic findings were hyperkeratosis, parakeratosis and acanthosis with scattered lymphocytic infiltration, also could be seen in parapsoriasis. The patient responded well to oral photochemotherapy

Chronic Plaque Psoriasis

Chronic plaque psoriasis; the most common form of psoriasis; is a papulosquamous disease defined by erythematous plaques with a silvery scale. The diagnosis usually is clinical; but occasionally a biopsy is necessary. Psoriasis affects 0.6 to 4.8 percent of the U.S. population; and about 30 percent of affected patients have a first-degree relative with the disease. Psoriasis is a T-cell-mediated autoimmune disease; but certain medications and infections are well-known risk factors. Management of psoriasis includes education about chronicity; realistic expectations; and use of medication. Steroids and vitamin D derivatives (e.g.; calcipotriene) are the mainstays of topical therapy. Topical steroids and calcipotriene together may work better than either agent alone. Patients with psoriasis involving more than 20 percent of their skin or those not responding to topical therapy are candidates for light therapy; traditional systemic therapy; or systemic treatment with immunomodulatory drugs such as alefacept; efalizumab; and etanercept

A Clinical and Histopathological Study of Parapsoriasis / 대한피부과학회지

BACKGROUND: Parapsoriasis described a group of cutaneous disease that can be characterized by scaly patches or slightly elevated plaques that have a resemblance to psoriasis, hence the nomenclature. Up to now, a uniformly accepted definition of parapsoriasis remains lacking. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathological features of parapsoriasis in Korea. METHODS: The clinical charts, photographs and skin biopsy slides of patients who had been clinically diagnosed as parapsoriasis at the initial visit were reviewed. Then the evaluation of age at onset, sex distribution, disease duration, anatomical distribution, clinical features of the skin lesions, associated symptoms, family history, co-existing disease, and histopathologic features were conducted. Six cases from small plaque parapsoriasis group and four cases from large plaque parapsoriasis group were investigated for the presence of TCR gene rearrangement using a PCR technique. RESULTS: The male to female ratio was 1: 1.2. The average ages of onset in large plaque type and small plaque type were 44.7 and 32.9 years respectively. 1) According to the clinical classification, 7 (29%) of the patients had large plaque type and 17 (71%) had small plaque type. 2) Many lesions of large plaque parapsoriasis group exhibited varying degrees of epidermal atrophy, fine wrinkles and telangiectasia. 3) Large plaque parapsoriasis showed more histologic changes consisting of epidermal atrophy, exocytosis and atypical lymphocytes. 4) Monoclonal T-cell receptor gene rearrangement was not detected in all of 10 patients with large and small plaque parapsoriasis. CONCLUSION: In our study, some of our findings are in agreement with those already in the literature, whereas others, particularly the male to female ratio and constitutional symptoms, differ. Our results also indicate that there are many clinical and pathological differences between large plaque and small plaque parapsoriasis.

Polymerase Chain Reaction and Heteroduplex Analysis Based Detection of Clonal T Cell Receptor Gamma Gene Rearrangements in Paraffin-embedded Tissues of Cutaneous T Cell Proliferative Diseases

BACKGROUND: Recently, the molecular pathologic investigation for clonality in lymphomas has been introduced and has gained a role in the diagnosis of lymphomas. In fact, the clonality test using TCRGR phenomenon has been done by Southern blot analysis (SBA) and polymerase chain reaction (PCR) for molecular pathologic diagnosis of T cell lymphomas. However, it is difficult to perform SBA with paraffin embedded specimens or with samples of small skin biopsies. OBJECTIVE: We investigated the efficacy of PCR amplification of TCR gene in paraffin em-bedded cutaneous T cell lymphomas. METHODS: Iii this study, the clonality was assessed by polymerase chain reaction (PCR) analysis of T cell receptor gamma (TCR) gene from the DNA extracts obtained from paraffin em-bedded tissues (PET) of malignant T cells, B cell lymphomas, and benign cutaneous T cell proliferative disorders. Heteroduple-x-analyses were also performed to rule out the false positives. RESULTS: Among the total of 62 cases analyzed, monoclonality was observed in 4 out of 10 mycosis fungoides, 7 out of 9 cutaneous T cell lymphomas excluding mycosis fungoides, 1 out of 3 angiocentric lymphomas, 2 out of 2 lymphomatosis papulosis, 1 out of 7 large plaque parapsoriasis, and 1 out of 2 T cell lymphomas in other organs. No monoclonality was observed in 9 inflammatory cutaneous diseases, 5 small plaque parapsoriasis, 4 cutaneous B cell lymphomas, and 11 B cell lymphomas in lymph nodes. CONCLUSION: The results suggest that the PCR method and heteroduplex analysis used in this study were not only practical but also efficacious for the diagnosis of cutaneous T cell lymphomas using tissues embedded in paraffins.

Micose fungóide na infância: relato de um caso / Mycosis fungoides in childhood: a case report

A micose fungóide (MF) é a forma mais comum de linfoma cutâneo de células T. É rara na infância, pois afeta principalmente idosos. Os autores apresentam um caso de MF na infância, um dos primeiros relatados na literatura latino-americana, em um menino de 7 anos de idade. O diagnóstico foi realziado na fase inicial, estágio IB. O tratamento inicial com PUVA não teve boa respota. Com a substituição pela mostarda nitrogenada tópica, houve regressão das lesões

A Clinicopathological Study of Early Mycosis Fungoides / 대한피부과학회지

BACKGROUND: Mycosis fungoides(MF) is a representative of cutaneous T-cell lymphoma and progresses through clinical stages, such as initial pre-mycotic(macule or patch), plaque, and tumor stage. Although lesions of plaque and tumor stage show typical features, those of pre-mycotic stage are commonly non-specific that precise diagnosis of early MF remains elusive. OBJECTIVES: This study attempted to elucidate the clinical and histopathologic features of early MF. METARIALS AND METHODS: We investigated 11 cases of MF stage Ia(based on TNM staging system) that visited the department of dermatology at the Seoul National University Hospital for the past decade year(1988-1998). Medical records and biopsy slides were reviewed. RESULTS: The results were as follows : 1. Clinical characteristics: Lesions were relatively well-defined erythematous scaly patches or plaques on lower abdomen, buttocks, proximal extremities or whole body. Clinically initial diagnoses were large plaque parapsoriasis in nine cases and in the remainders, poikiloderma atrophicans vasculare and MF, respectively. Duration of disease ranged from one month to 41 years, averaging 15.2 years. 2. Treatments: Follow-up of 8 patients was continued and all showed partial responses to application of topical steroids or BCNU(carmustine) and PUVA therapy. 3. Histopathologic findings: Epidermotropism was found in all cases and can be the most important pathologic finding, but typical Pautriers microabscesses were found in a few cases. Mild spongiosis was seen in epidermis and superficial perivascular infiltration was the most common finding in dermis. Lichenoid infiltration with epidermal psoriasiform change was found in two cases. Atypism of infiltrating cells was found in all cases and more prominent in epidermis. CONCLUSION: Lesions of early mycosis fungoides were relatively non-specific erythematous patches/plaques but characteristically, patients had relatively long duration of stationary disease status and showed good responses to treatments. Histopathologically, variable degree of epidermotropism was the most constant finding.

bcl-2 Expression in Cutaneous T Cell Lymphoma / 대한피부과학회지

BACKGROUND: The bcl-2 is an oncogene involved in tumorigenesis by blocking apoptosis, or programmed cell death and over-expression of bcl-2 protein has been reported in several malignant tumors such as lung cancer, basal cell carcinoma, breast cancer and malignant melanoma. However, there have been only a few studies about bcl-2 expression of cutaneous T cell lymphoma. OBJECTIVE: The purpose of this study was to examine whether there is any difference in expression of bcl-2 between mycosis fungoides(MF), angiocentric T cell lymphoma, angioimmunoblastic T cell lymphoma, subcutaneous T cell lymphoma and anaplastic large cell lymphoma. We also evaluated the statistical significance between expression of bcl-2 and the prognosis of the diseases. METHODS: Routine paraffin sections of formalin-fixed 36 tissues (14 MF, 7 angiocentric T cell lymphoma, 5 subcutaneous panniculitic T cell lymphoma, 2 anaplastic large cell lymphoma, 1 angioimmunoblastic T cell lymphoma, 1 unspecified peripheral T cell lymphoma, 2 small plaque parapsoriasis, 2 psoriasis and 2 lichen planus) were labelled with anti-bcl-2 monoclonal antibody using an avidin- biotin-peroxidase complex. Normal skin for bcl-2 served as negative controls. RESULTS: The results were as follows. l. All cases of benign inflammatory diseases, small plaque parapsoriasis and patch stages of MF showed positive staining for bcl-2. Therefore, there were no differences in expression of bcl-2 among these diseases. 2. In the plaque and tumor stages of mycosis fungoides, statistically significancant differences in bcl-2 expression were not found during disease progression. 3. bcl-2 expression in peripheral T cell lymphoma (five in seven cases of angiocentric T cell lymphoma showed positive staining but all other peripheral T cell lymphoma was negative) decreased significantly (p0.05). CONCLUSION: These results suggest that the loss of bcl-2 expression may play a significant role in progression of cutaneous T cell lymphoma except in MF and angiocentric T cell lymphoma.

Rapid reversal of manifestations of acquired zinc deficiency in alcoholic cirrhosis with diet therapy.

Features of acquired zinc deficiency syndrome occurred in an alcoholic cirrhotic during hospital stay while he was on parenteral nutrition. Rapid reversal of symptoms occurred with resumption of normal diet without additional zinc supplementation.

[ puvatherapy]

The definition PUVA therapy formed by the originals of psoralen [P] and ultraviolet "light therapy" A [UVA] indicate a photochemotherapeutic technique in big improvment. The main indications of PUVA therapy are the generalised and complicated psoriasis, plaque parapsoriasis and mycosis fungoides. Considering the early side effects, usually mild [digestiv intolerance, allergy, phototoxicity], eye lesions and increased incidence of skin cancer we have to be very careful in order to prescribe this technique

Tratamiento de Linfoma T cutáneo ( Micosis fungoide ) con Interferon Alfa intralesional / Cutaneous T-cell Lymphoma (Mycosis fungoides): treatment with Interferon Alpha intralesional

Se presenta el caso de un paciente de 63 años de edad, portador de Linfoma T (Micosis Fungoide) de dos años de evolución, en todos sus estadíos (máculas, parches, placas y tumores); quien luego de haber recibido todas las opciones terapéuticas (algunas, por diversas razones, sólo parcialmente) sin resultado, inicia Interferon alfa intralesional, en dosis de 1.000.000 de U. por centímetro cuadrado de superficie cutánea que provocó remisión completa de las lesiones en seis meses, sin recaída en nueve meses de control. Presentamos una nueva modalidad terapéutica, en corto lapso de tiempo muy efectiva para una enfermedad de curso inexorable, en la que todos los resultados terapéuticos son muy poco efectivos.