Mycosis fungoides presenting with extensive pyoderma gangrenosum-like ulcers.

Mycosis fungoides (MF) may present with atypical clinical manifestations. Usually it mimics various chronic dermatoses, with the appearance of ulcers during the tumour stage. Infrequently, cutaneous ulcers are the main or initial sign of lymphoma. We report the case of a man who presented multiple skin lesions that clinically appeared to be pyoderma gangrenosum (PG). However, histological and immunohistochemical examination revealed MF. This case illustrates that PG-like ulcers maybe atypical cutaneous manifestations of MF and exceptionally the presenting sign of this disease.

[Diagnosis by electron microscopy of recessive dystrophic epidermolysis bullosa]. / Diagnóstico por microscopía electrónica de la epidermolisis ampollar distrófica recesiva.

Inherited epidermolysis bullosa (EB) includes a number of distinctive diseases that are characterized by the presence of fragile skin and the tendency to develop blisters and erosions. The current classification separates the types of EB on the basis of the ultrastructural level of the blisters. The electron microscopy is very important for the diagnosis and in the recessive dystrophic EB shows that the lamina densa forms the roof of the blister and that the number of anchoring fibrils are absent or reduced. We present the case of a 30 year old woman with a diagnosis of recessive dystrophic EB diagnosed by electron microscopy.

Prevalence of cutaneous manifestations in 200 patients with eating disorders.

BACKGROUND: Eating disorders are increasing and show a variety of symptoms. They mainly include anorexia nervosa (AN), bulimia nervosa (BN), and eating disorders not specified (EDNOS). They predominate in females and represent an important danger, especially in teenagers. In serious cases, they may be life-threatening. Objective To determine the prevalence of cutaneous findings in patients with eating disorders and to compare the results with those found in the literature. METHODS: An observational, transverse, and prospective study was performed. Two hundred patients of recent admission to ALUBA (association that fights against BN and AN), a psychiatric unit for eating disorders, were included: 122 BN; 62 AN; 16 EDNOS. RESULTS: Patients with eating disorders show dermatologic manifestations (alopecia, xerosis, hypertrichosis, caries, nail fragility) that are secondary to starvation. Russell's sign, seen as calluses on the dorsal aspect of the hands, is a consequence of self-induced vomiting and the local trauma of the superior incisors. This sign represents a compensatory behavior to overeating and predominates in the BN group. CONCLUSION: The recognition of dermatologic signs could be of immense value and could lead to the early diagnosis and treatment of these eating disorders.

[Ulcerated cutaneous lymphoma. 10 years of experience]. / Linfomas cutáneos ulcerados. Diez años de experiencia.

Patients with lymphomas and cutaneous ulcers have a poor prognosis. Commonly the ulcers occur later in the course of lymphomas and may be the source of sepsis. From 59 patients with lymphoma and skin involvement, 12 patients who presented with ulcers were retrospectively analyzed between January 1990 to December 1999. More frequently ulcers were multiple, necrotic, infected and placed on tumors. Sepsis was the main cause of mortality in 10 patients and most cases were secondary to Staphylococcus aureus and Pseudomonas aeruginosa. Factors associated with poor prognosis were febrile neutropenia after chemotherapy and generalized involvement by the lymphoma. Mean survival of deceased patients after onset of ulcers was 6.27 months. We emphasize the importance of an appropriate microbiological study and a prompt therapy.

Diagnóstico por microscopía electrónica de la epidermolisis ampollar distrófica recesiva. / [Diagnosis by electron microscopy of recessive dystrophic epidermolysis bullosa]

Inherited epidermolysis bullosa (EB) includes a number of distinctive diseases that are characterized by the presence of fragile skin and the tendency to develop blisters and erosions. The current classification separates the types of EB on the basis of the ultrastructural level of the blisters. The electron microscopy is very important for the diagnosis and in the recessive dystrophic EB shows that the lamina densa forms the roof of the blister and that the number of anchoring fibrils are absent or reduced. We present the case of a 30 year old woman with a diagnosis of recessive dystrophic EB diagnosed by electron microscopy.

Linfomas cutáneos ulcerados. Diez años de experiencia. / [Ulcerated cutaneous lymphoma. 10 years of experience]

Patients with lymphomas and cutaneous ulcers have a poor prognosis. Commonly the ulcers occur later in the course of lymphomas and may be the source of sepsis. From 59 patients with lymphoma and skin involvement, 12 patients who presented with ulcers were retrospectively analyzed between January 1990 to December 1999. More frequently ulcers were multiple, necrotic, infected and placed on tumors. Sepsis was the main cause of mortality in 10 patients and most cases were secondary to Staphylococcus aureus and Pseudomonas aeruginosa. Factors associated with poor prognosis were febrile neutropenia after chemotherapy and generalized involvement by the lymphoma. Mean survival of deceased patients after onset of ulcers was 6.27 months. We emphasize the importance of an appropriate microbiological study and a prompt therapy.

Nevoid keratosis of the nipple.

A patient with nevoid keratosis of the nipple is described. The clinical features, histology, therapy, and prognosis are discussed.

Hiperpigmentación benigna de las mucosas / Bening hyperpigmentation of the mucous membranes

La hiperpigmentación de las mucosas labial y genital ocurre entre el 3 por ciento y el 15 por ciento de la población normal, caracterizándose por la aparición de máculas de coloración oscura (parda o negra). En el examen histopatológico se observa hiperpigmentación de la capa basal y ausencia de hiperplasia de melanocitos. Son lesiones benignas que muchas veces simulan clínicamente un melanoma y plantean, además, otros diagnóstico diferenciales. Se agrupa como hiperpigmentación benigna de las mucosas (HBM) a la mácula melanótica labial (MML), melanosis genital (MG) y el síndrome de Laugier-Hunziker (SLH) en base a las características clínicas e histopatológicas. Se establece la actitud ante lesiones pigmentadas de las mucosas labial y genital

Hiperpigmentación benigna de las mucosas / Bening hyperpigmentation of the mucous membranes

La hiperpigmentación de las mucosas labial y genital ocurre entre el 3 por ciento y el 15 por ciento de la población normal, caracterizándose por la aparición de máculas de coloración oscura (parda o negra). En el examen histopatológico se observa hiperpigmentación de la capa basal y ausencia de hiperplasia de melanocitos. Son lesiones benignas que muchas veces simulan clínicamente un melanoma y plantean, además, otros diagnóstico diferenciales. Se agrupa como hiperpigmentación benigna de las mucosas (HBM) a la mácula melanótica labial (MML), melanosis genital (MG) y el síndrome de Laugier-Hunziker (SLH) en base a las características clínicas e histopatológicas. Se establece la actitud ante lesiones pigmentadas de las mucosas labial y genital (AU)

[PCR determination of an association between class II HLA and pemphigus vulgaris]. / Determinación por PCR de la asociación entre antígenos HLA clase II y pénfigo vulgar.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. It is characterized by the presence of an autoantibody directed against desmoglein 3, which causes acantholysis and blister formation. In this study, we examined the HLA antigens of 30 caucasian argentinian patients compared with 199 controls. We used the PCR-SSO method (Polymerase Chain Reaction-Sequence Specific Oligonucleotide). We found that PV patients had significantly increased frequencies of HLA DR4 (RR = 3.80, P = 0.001) and HLA DR 14 (RR = 5.97, P = 0.0001). As in other populations, two associated alleles were found: the first was DR beta 1*0402 (RR = 44.70, P = 10.7) and DQ beta 1*0302 (RR = 71.82, P = 10(-7)) and the second was DR beta 1*1401 (RR = 117.94, P = 10(-7)) y DQ beta 1*0503 (RR = 86.95, P = 10(-7)).

Determinación por PCR de la asociación entre antígenos HLA clase II y pénfigo vulgar. / [PCR determination of an association between class II HLA and pemphigus vulgaris]

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. It is characterized by the presence of an autoantibody directed against desmoglein 3, which causes acantholysis and blister formation. In this study, we examined the HLA antigens of 30 caucasian argentinian patients compared with 199 controls. We used the PCR-SSO method (Polymerase Chain Reaction-Sequence Specific Oligonucleotide). We found that PV patients had significantly increased frequencies of HLA DR4 (RR = 3.80, P = 0.001) and HLA DR 14 (RR = 5.97, P = 0.0001). As in other populations, two associated alleles were found: the first was DR beta 1*0402 (RR = 44.70, P = 10.7) and DQ beta 1*0302 (RR = 71.82, P = 10(-7)) and the second was DR beta 1*1401 (RR = 117.94, P = 10(-7)) y DQ beta 1*0503 (RR = 86.95, P = 10(-7)).

Manifestaciones cutáneas de la anorexia y bulimia / Dermatological aspects in bulimia and anorexia nervosa

Se analizan los aspectos dermatológicos de 100 pacientes con patología de la conducta alimentaria, de los cuales 60 correspondieron a bulimia nerviosa (BN) y 40 a anorexia nerviosa (AN). La mayoría eran de sexo femenino, con edad promedio de 17 años. Fueron características comunes de ambas patologías xerosis, acné, alopecia y caries, destacándose en BN dermatitis artefacta, carotenodermia y signo de Russell y en AN hipertricosis y amenorrea. Se enfatiza la importancia del reconocimiento de estos cuadros que están en crecimiento y donde el diagnóstico precoz es fundamental para iniciar un tratamiento adecuado (AU)

Manifestaciones cutáneas de la anorexia y bulimia / Dermatological aspects in bulimia and anorexia nervosa

Se analizan los aspectos dermatológicos de 100 pacientes con patología de la conducta alimentaria, de los cuales 60 correspondieron a bulimia nerviosa (BN) y 40 a anorexia nerviosa (AN). La mayoría eran de sexo femenino, con edad promedio de 17 años. Fueron características comunes de ambas patologías xerosis, acné, alopecia y caries, destacándose en BN dermatitis artefacta, carotenodermia y signo de Russell y en AN hipertricosis y amenorrea. Se enfatiza la importancia del reconocimiento de estos cuadros que están en crecimiento y donde el diagnóstico precoz es fundamental para iniciar un tratamiento adecuado