Hematological and molecular characteristics of Hb Sarrebourg [beta131(H9)Gln-->Arg (CAG-->CGG)] found in a Spanish family.

We report the hematological and molecular characteristics of Hb Sarrebourg [beta131(H9)Gln-->Arg (CAG-->CGG)] found in nine members of a Spanish family. All the carriers were heterozygous for the variant and hematologically normal. Up to now this hemoglobin (Hb) variant has only been reported once before, in a boy of Turkish origin.

Localization of Sweet's syndrome in radiation-induced locus minoris resistentae.

Sweet's syndrome is a reactive dermatosis clinically characterized by fever, leukocytosis, and multiple, erythematous, painful plaques. Histopathologically, the lesions show edema of the papillary dermis and a dermal infiltrate mainly composed of neutrophils, without vasculitis. We describe a patient with breast carcinoma and Sweet's syndrome mostly involving previously irradiated skin.

Dermatitis de contacto alérgica profesional a la resinaepoxi y urticaria alérgica de contacto al látex en trabajadorde una fábrica de raquetas de paddle / Occupational allergic contact dermatitis from epoxy resin and allergic contact urticaria from latex in a worker at paddleball paddle factory

La resina epoxi es un material industrial ampliamente usado y con un gran poder sensibilizante. Desde hace varios años el látex es un alergeno bien conocido entre los trabajadores de la salud y, recientemente, se esta relacionando con otras profesiones. En este trabajo se expone el caso clínico de un paciente de 20 años de edad que trabajaba en una fábrica de raquetas de paddle y al cabo de un año y medio presentó sensibilización simultánea a RE y al látex con un cuadro clínico de eccema de contacto en las manos y de dermatitis aerotransportada. Se estudió al paciente y las pruebas epicutáneas mostraron una positividad (++) a la RE y una prueba de "prick" al látex positiva (AU)

Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases.

BACKGROUND: Specific cutaneous involvement in patients with multiple myeloma (MM) is very uncommon. It usually occurs in late stages of MM as a reflection of increased tumor cell burden. We studied 8 patients with cutaneous involvement of MM without underlying bony lesions and reviewed the literature on this rare dermatologic manifestation. DESIGN: We were particularly interested in the clinical course of patients with MM and cutaneous metastases, including survival once metastases were detected and the possible influence of various forms of therapy. Our goal was also to identify the immunoglobulin and the light-chain type in these cases, with emphasis on any possible association between a particular immunoglobulin class and cutaneous involvement, as well as the histopathologic, immunohistochemical, and cytogenetic features of the neoplastic plasma cells involving the skin. SETTING: University department of dermatology, university hospital, and private practice. PATIENTS: Medical records and biopsy specimens from 8 patients with MM and specific cutaneous lesions were reviewed. RESULTS: Cutaneous lesions consisted of multiple erythematous or violaceous nodules or plaques with a wide anatomical distribution. Histopathologically, 2 different patterns were identified: nodular and diffuse interstitial. Neoplastic plasma cells showed atypical features, and in 1 case they displayed a spindle shape, giving a sarcomatoid appearance to the lesion. Immunohistochemical studies demonstrated that neoplastic plasma cells were strongly positive for CD79a, CD138, and epithelial membrane antigen, and variably positive for VS38c and CD43. In each case the immunoglobulin profile and the light-chain type expression of the neoplastic cells were the same as those identified in the serum of the patients: 5 cases were IgA lambda; 2 cases were IgG kappa; and 1 case was IgA kappa. In cases 2, 3, and 4, polymerase chain reaction investigations revealed monoclonal rearrangement for IgH genes, whereas the investigations for human herpesvirus 8 and Epstein-Barr virus yielded negative results. Fluorescent in situ hybridization investigations in these 3 cases demonstrated that the cutaneous neoplastic plasma cells showed the deletion of the rb-1 (retinoblastoma) gene. Despite aggressive chemotherapy, all 8 patients died a few months after the development of cutaneous involvement. CONCLUSIONS: In our series, there was a perfect correlation of immunoglobulin and light-chain type between the serum electrophoresis and the cutaneous plasma cells. Patients with MM showed a short survival once cutaneous metastases appeared independently of the therapy. The deletion the rb-1 gene may provide prognostically relevant information to identify a high-risk subset of patients with MM.

Sensibilización a los sistemas de resina epoxi en una nueva serie de pacientes instaladores de suelos especiales / Sensitization to epoxi system in a new series of special flooring workers

Los pavimentos especiales compuestos de resina epoxi se caracterizan por una alta resistencia a factores mecánicos, químicos y medioambientales, por lo que se utilizan en el recubrimiento de superficies sometidas a un intenso desgaste. La dermatitis alérgica de contacto a la resina epoxi es frecuente entre los trabajadores especializados en la instalación de estos suelos. Son características la rapidez de sensibilización, la localización preferente en cara y manos y la afectación de pacientes jóvenes. En 1994, publicamos una serie de 15 pacientes instaladores de suelos especiales evaluados en el Servicio de Dermatología Laboral de la Escuela Nacional de Medicina y Seguridad del Trabajo (ENMST) entre 1989 y 1992. Se presenta una nueva serie de 13 pacientes evaluados entre enero de 1995 y abril de 2002 con el objeto de realizar un análisis comparativo entre las dos series (AU)

Enfermedad de Grover en un paciente con insuficiencia renal crónica en hemodiálisis / Grover' s disease in a patient with chronic renal failure on hemodialysis

Se presenta un caso de enfermedad de Grover, con patrón histopatológico tipo enfermedad de Darier, en un paciente varón de 69 años de edad con antecedentes personales de insuficiencia renal crónica secundaria a un carcinoma urotelial multicéntrico de larga evolución que había sido tratado con sucesivas intervenciones quirúrgicas. El paciente refería que el inicio de las lesiones cutáneas se había producido poco tiempo después del comienzo del tratamiento de hemodiálisis. Se discute la posible implicación de la insuficiencia renal crónica y/o la hemodiálisis en la patogenia de la enfermedad de Grover (AU)

Dermatitis alérgica de contacto no profesional a propóleo / Nonoccupational allergic contact dermatitisfrom propolis

El propóleo es un producto elaborado por las abejas a partir de la resina de determinadas especies vegetales. La dermatitis alérgica de contacto al propóleo fue originalmente una enfermedad profesional descrita en apicultores. En la actualidad, sin embargo, afecta fundamentalmente a pacientes que usan productos «naturales» que contienen propóleo con fines cosméticos o terapéuticos. Se presenta un caso de dermatitis alérgica no profesional a propóleo en una mujer de 44 años de edad. La paciente se trató un herpes simple labial aplicándose una solución de propóleo. Pocas horas después desarrolló un eccema agudo en el labio y piel perioral próxima. La prueba epicutánea con la solución comercial de propóleo fue muy positiva (+ + +) a las 48 y las 96 h. La prueba de parche con True-Test® fue positiva para bálsamo de Perú entre otros. Este resultado puede deberse a una reacción cruzada ya que el bálsamo de Perú y el propóleo tienen varios componentes en común. En España, el uso creciente de productos destinados para uso tópico como cosméticos y medicinas «naturales» podría condicionar con el tiempo un incremento en la frecuencia de sensibilización al propóleo (AU)

Dermatitis artefacta?

A 35-year-old man presented with a 2-month history of intensely pruritic excoriated and crusted linear lesions on the dorsa of the left hand and left forearm (Fig 1). The patient had worked in construction for 2 years, and his job consisted mainly in covering the facades of buildings with cement. The patient was right-handed and used a black rubber glove as a protective measure only on his left hand (Fig 2). He reported that the lesions resolved partially during holidays and weekends and clearly flared in association with his work. There was no history of atopic dermatitis, drug use, or intolerance to metals, rubber, or fruits. On physical examination, linear excoriations with crusts were observed on the dorsa of the left hand, extending to the ventral and dorsal aspects of the forearm, involving the whole area that was in contact with the glove. Lichenified erythematous plaques and excoriations on the dorsal surface of the metacarpophalangeal joints and scaly lesions on the dorsal surfaces of the fingers were also present. On the palm, only discrete hyperkeratosis was seen. The right hand and forearm were free of lesions. He complained of intense pruritus when wearing the rubber glove and admitted to continuous scratching to relieve his discomfort, inducing the linear and excoriated lesions. Treatment with topical corticosteroids was initiated, with progressive resolution of the lesions.

Síndrome de Schnitzler / Schnitzler´s syndrome

El síndrome de Schnitzler se define como una constelación única de signos clínicos y biológicos que incluye la presencia de urticaria crónica, fiebre intermitente, dolores óseos, artralgia o artritis y una gammapatía monoclonal de tipo IgM. Presentamos el caso de una paciente con brotes diarios de lesiones urticarianas evanescentes muy refractarias al tratamiento con antihistamínicos y esteroides orales, asociadas a picos febriles y dolores óseos. La paciente presentaba leucotrombocitosis, anemia asociada a trastorno inflamatorio crónico y aumento de la velocidad de sedimentación globular. El proteinograma reveló la presencia de una paraproteinemia monoclonal de tipo IgM con cadenas ligeras kappa en la inmunofijación. Mediante técnicas de radiodiagnóstico se identificaron focos de osteocondensación en la columna vertebral. El estudio histopatológico de las lesiones cutáneas demostró hallazgos de urticaria sin vasculitis. De acuerdo con estos hallazgos clínicos, analíticos y radiológicos se estableció el diagnóstico de síndrome de Schnitzler. (AU)

Melanocitosis dérmica de la parte alta de la espalda / Upper back dermal melanocytosis

Se han descrito numerosas variantes clínicas de melanocitosis dérmica que incluyen el nevo azul, la mancha mongólica, el nevo de Ota y el nevo de Ito, así como otros tipos menos frecuentes que se denominan genéricamente melanocitosis dérmicas atípicas. Presentamos el caso de una melanocitosis dérmica persistente adquirida que afecta la región interescapular de un paciente de raza blanca y 44 años de edad, y que correspondería a la denominada melanocitosis dérmica de la parte alta de la espalda. (AU)

Chloracne: histopathologic findings in one case.

BACKGROUND: Chloracne is an acneiform eruption due to poisoning by halogenated aromatic compounds having a specific molecular shape. This condition is always a symptom of systemic poisoning by chemical chloracnegens and not just a cutaneous disorder. METHODS: We have studied a patient with severe chloracne who showed cutaneous lesions involving mostly the face and the axillae. RESULTS: Histopathologic study of the facial lesions demonstrated that almost every vellus hair follicle was involved, showing a dilated infundibulum filled by a keratotic plug. This keratotic material was mostly composed of orthokeratotic basket-weave basophilic corneocytes, namely infundibular keratin, although there were also some dilated infundibula containing eosinophilic laminated or granular sebum at their center. Small infundibular cysts were more numerous than comedones. Mature and well-developed sebaceous glands were seen at the base of many of the dilated infundibula and no squamous metaplasia of the sebaceous glands or ducts could be demonstrated. Hyperpigmentation of the lesions resulted from hyperproduction of melanin by a normal number of melanocytes along the basal layer of the epidermis and infundibular epithelium. Abundant melanin granules also impregnated the corneocytes of the infundibular plugs. CONCLUSIONS: Our findings support the notion that tiny infundibular cysts rather than comedones represent the basic lesions of chloracne.