Leonine facies: A unique presentation of T-prolymphocytic leukemia.

We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as "Leonine facies". He had impaired sensation over the face and was initially diagnosed to have lepromatous leprosy and was treated with antileprosy drugs. Investigations showed a total Leukocyte count of 550 X 109/l with 90% atypical lymphoid cells with prominent central nucleolus suggestive of prolymphocytes. On flow cytometry, these cells were positive for cytoplasmic CD3, CD2, CD5, CD7, CD4, and CD38 (dim) and were negative for CD1a and TdT and diagnosis of T-prolymphocytic leukemia was made.

Eritrodermia y Linfomas Cutáneo de Células T / Erythroderma and Cutanous T - Cell Lymphoma

La eritrodermia es una erupción eritematosa más o menos escamosa que afecta a más del 90 por ciento de la superficie corporal. Se trata de una enfermedad capaz de comprometer el pronóstico vital y que puede complicarse con desequilibrios hidroelectrolíticos, trastornos de la termorregulación, infecciones, así como con fallo cardiovascular. En la presente publicación referimos el caso de un paciente masculino de 72 años con antecedentes de hipertensión arterial y de Lepra que recibió poliquimioterapia, es ingresado en el servicio de dermatología en el período de observación, pues hace alrededor de un año comenzó a presentar enrojecimiento de la piel y múltiples escamas. Al momento del examen se observa paciente eritrodérmico, ectropión, distrofia ungueal importante, con adenopatías palpables axilares e inguinales, asociado a un prurito intenso generalizado. Se realizan estudios para el diagnóstico incluyendo biopsia de ganglios inguinal y de piel, esta última compatible con Micosis Fungoide. Actualmente se reconocen tres variantes eritrodérmicas en el linfoma cutáneo de células T: el síndrome de Sézary, la Eritrodermia en micosis fungoide y la Eritrodermia en linfomas cutáneos de células T: No Especificada. Si bien se entiende al síndrome de Sézary como una expresión leucémica del linfoma cutáneo de células T eritrodérmico, con numerosas células de Sézary en piel, sangre y otros tejidos, la Eritrodermia en micosis fungoide se determinaría ante la ausencia de estos mismos hallazgos hematológicos e histológico ante un cuadro clínico eritrodérmico. Se hace necesaria la publicación del artículo por la forma de presentación de la micosis fungoide, teniendo en cuenta que esta dermatosis tiene un sin número de diagnósticos diferenciales que la hace la gran simuladora en la Dermatología(AU)

Erythroderma is a more or less scaly erythematous rash that affects more than 90 percent of the body surface area. It is a disease capable of compromising the vital prognosis and that can be complicated by hydroelectrolytic imbalances, thermoregulation disorders, infections, as well as cardiovascular failure. In this publication we refer to the case of a 72-year-old male patient with a history of high blood pressure and leprosy who received polychemotherapy, is admitted to the dermatology service in the observation period, as about a year ago he began to develop redness of the skin and multiple scales. At the time of the examination, an erythrothermal patient, ectropion, important ungueal dystrophy, with axillary and inguinal palpable adenopathies, associated with a widespread intense itching, is observed. Studies are conducted for diagnosis including inguinal and skin node biopsy, the latter compatible with Mycosis Fungoide. Three erythrodermal variants are currently recognized in skin T-cell lymphoma: Sézary syndrome, Erythroderma in fungoid mycosis, and Erythroderma in skin T-cell lymphomas: Undepede. While Sézary syndrome is understood as a leukemia expression of erythrodermal T-cell skin lymphoma, with numerous Sézary cells in skin, blood and other tissues, Erythroderma in fungoid mycosis would be determined in the absence of these same haematological and histological findings before an erythrodermal clinical picture. It is necessary to publish the article by the form of presentation of fungoid mycosis, taking into account that this dermatosis has a number of differential diagnoses that makes it the great simulator in Dermatology(EU)

Erythroderma as a manifestation of leprosy.

Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.

Erythroderma as a manifestation of leprosy

Abstract: Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.

Erythroderma in children.

Although erythroderma is a well-recognized entity in the adult age groups and has been studied by various authors, there is a paucity of studies on erythroderma in the pediatric age group. It poses a greater challenge to the dermatologist and pediatrician because of its potential life threatening nature. In a study conducted by us in a large Indian hospital to delineate the causes of neonatal and infantile erythroderma, the causes identified were infections (40%), ichthyosiform erythroderma (25%), atopic dermatitis (15%), infantile seborrheic dermatitis (10%) and unidentified (10%). In another study of childhood erythroderma, etiologically, drugs (29%) showed the highest incidence, followed equally (18%) by genodermatoses, psoriasis and staphylococcal scalded skin syndrome (SSSS). The management of childhood erythroderma is mainly supportive with correction of the hematologic, biochemical and metabolic imbalance if required. In this review, the causes of childhood erythroderma, the clinical features useful to the diagnosis and management are discussed.

Neonatal dermatological emergencies.

The neonates are unique in several ways in comparison with older children and adults which render them highly susceptible to severe, sometimes life threatening dermatological disorders. The neonatal dermatological emergencies are a diagnostic and therapeutic challenge. A wide range of dermatoses such as infections, genodermatoses, metabolic disorders and vascular tumors may require emergency care. The clinical presentation also varies from generalized involvement of skin to localized disease with or without systemic symptoms. Irrespective of the etiology and clinical presentation, these disorders are associated with significant morbidity and mortality. With the availability of effective drugs and monitoring facilities, and awareness of need for immediate care, there has been a significant decline in the fatality rate associated with neonatal dermatological emergencies. Knowledge of clinical presentations, rapid diagnostic methods, emergency care and monitoring of progress of the disease helps in comprehensive multidisciplinary care of neonates with these disorders.