Cranial aseptic osteomyelitis associated with pyoderma gangrenosum.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

TP53 Abnormalities and MMR Preservation in 5 Cases of Proliferating Trichilemmal Tumours.

Proliferating trichilemmal tumours (PTT) are defined by a benign squamous cell proliferation inside a trichilemmal cystic (TC) cavity. A possible explanation of this proliferative phenomenon within the cyst may be molecular alterations in genes associated to cell proliferation, which can be induced by ultraviolet radiation. Among other genes, alterations on TP53 and DNA mismatch repair proteins (MMR) may be involved in the cellular proliferation observed in PTT. Based on this assumption, but also taking into account the close relationship between the sebaceous ducts and the external root sheath where TC develop, a MMR, a p53 expression assessment and a TP53 study were performed in a series of 5 PTT cases, including a giant one. We failed to demonstrate a MMR disorder on studied PTT, but we agree with previous results suggesting increased p53 expression in these tumours, particularly in proliferative areas. TP53 alteration was confirmed with FISH technique, demonstrating TP53 deletion in most cells.

Cranial aseptic osteomyelitis associated with pyoderma gangrenosum. / Osteomielitis aséptica craneal asociada a pioderma gangrenoso.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

Clear Cell Hidradenoma-Basal Cell Carcinoma Collision.

ABSTRACT: A 91-year-old man presented with a tumor on the left temporal area, clinically suspicious of basal cell carcinoma. The histopathologic study showed a central solid-cystic tumor composed by 3 different types of cells (clear or finely granular cells, polygonal cells, and squamoid cells). It had a sclerotic stroma. At the periphery, another tumor composed by smaller interconnected nests was evident. Some nests were separated from the stroma by clefts. The stroma of this second tumor was highly cellular. There was a sharp delimitation between both tumors, with no transitional area. Immunochemistry demonstrated they are different tumor. A diagnosis of clear cell hidradenoma-basal cell carcinoma collision was performed. To the best of our knowledge, this is the first description of this challenging association.

Hemangioendotelioma kaposiforme con fenómeno de Kasabach-Merritt / Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon

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Complete regression of melanoma associated with vitiligo.

A 53-year-old woman presented with vitiligo. A pigmented lesion was disclosed in the physical examination. Its histopathologic study showed the presence of a band of melanophages in an uneven distribution. Fibroplasia and telangiectasias were also observed, but neither nevus nor melanoma cells were found. A short time afterwards, the patient developed a metastasis in an inguinal lymph node. In spite of high-dose interferon treatment, the patient died two years after the diagnosis. This case associates two uncommon events: a) the whole regression of a melanoma and b) vitiligo associated with melanoma. Although both processes have a similar pathogenic mechanism, this association is exceptional and probably influences the prognosis.

Dermatofibrosarcoma protuberans atrófico angiomatoso / Angiomatous and atrophic variant of dermatofibrosarcoma protuberans

Un varón de 13 acudió por una placa atrófica de aspecto vascular localizada en la zona lumbar, que correspondió a un dermatofibrosarcoma protuberans,que presentaba múltiples vasos sanguíneos de distintos tamaños.El dermatofibrosarcoma protuberans atrófico es una variante clínica de dermatofibrosarcoma protuberans más frecuente en niños, su diagnóstico amenudo se retrasa debido a una presentación clínica inusual, y a la menor tendencia a realizar biopsias en niños. Nuestro caso es excepcional porqueademás presentaba una marcada proliferación vascular

A 13-year-old boy presented an atrophic erythematous patch of vascular appearence on his lumbar area, which corresponded to a dermatofibrosarcomaprotuberans, however, it showed multiple blood vessels of different sizes.The atrophic dermatofibrosarcoma protuberans is considered a clinical variant that is more frequent in children. Its diagnosis is usually delayed becausethe uncommon clinical picture and the less tendency to perform biopsies in children. In addition, our case is exceptional because of the conspicuousvascular proliferation

Comparative study of pagetoid dyskeratosis between acrochordons and soft fibromas.

BACKGROUND: Pagetoid dyskeratosis (PD) is considered a casual finding. We can find it in some conditions, including acrochordons and soft fibromas. OBJECTIVE: (1) to compare the presence of PD in soft fibromas and acrochordons and (2) to compare PD positive fibromas and PD negative fibromas. MATERIALS AND METHODS: We reviewed all acrochordons and soft fibromas diagnosed in the General Hospital of Lanzarote, Spain, between January 2001 and December 2002. We assessed the presence of PD, size, acanthosis, basal pigmentation, and the presence of pseudohorn cysts. RESULTS: Three hundred sixty one acrochordons and 164 soft fibromas were included in this study. There were striking differences in the presence of PD, size, acanthosis, and basal pigmentation between both entities. PD positive fibromas predominated in axillas. There were no other differences between PD positive fibromas and PD negative fibromas. CONCLUSION: Although soft fibromas and acrochordons are actually fibroepithelial polyps, including the presence of PD, there are striking differences between them. Thus, both conditions have to be considered as different entities. PD could be related to friction and moisture. PD has to be distinguished from other conditions such as Paget's disease, pagetoid melanoma, koilocytes, clear cell papulosis, among others.

Hidrocistoma apocrino sobre nevus sebáceo / Apocrine hidrocystoma arising from a sebaceus nevus

Una mujer de 41 años que tenía un nevus sebáceo congénito de cuero cabelludo desarrolló un hidrocistoma apocrino. Éste, también denominado cistadenoma apocrino o, más recientemente, quiste de la glándula apocrina, es una lesión quística infrecuente, casi siempre solitaria, que en ocasiones puede aparecer sobre un nevus sebáceo. Los tumores que se desarrollan sobre nevus sebáceos suelen aparecer en la edad adulta, con un porcentaje de tumores agresivos muy bajo. Por esta razón actualmente se desaconseja la exéresis profiláctica de los nevus sebáceos y sólo se recomienda un seguimiento para tratar aquellos casos que desarrollen lesiones asociadas (AU)

Crisiasis / Chrysiasis

Exponemos el caso de un varón de 73 años con antecedentes de una artritis reumatoide por la que había recibido tratamiento con sales de oro (aurotiomalato sódico) por vía intramuscular durante 8 años. Tras suspender dicho tratamiento desarrolló una pigmentación con tonalidad verdosa en dorso de manos y antebrazos. Las escleras presentaban una pigmentación similar, pero el resto de las mucosas, el pelo y las uñas estaban respetadas. El estudio histopatológico demostró la presencia de gránulos en el interior de macrófagos, así como libres en la dermis. El tratamiento continuado durante largos períodos con sales de oro puede producir una pigmentación en áreas fotoexpuestas denominada crisiasis. Aunque las mucosas están libres, las escleras pueden verse afectadas. Histopatológicamente se caracteriza por la acumulación de gránulos de oro en el citoplasma de los macrófagos dérmicos. Debido a la tendencia actual de utilizar terapias más agresivas para la artritis reumatoide, prácticamente la única patología en que se sigue usando sales de oro, probablemente la crisiasis sea cada vez más rara. (AU)

Miofibroma cutáneo del adulto / Cutaneous adult myofibroma

El miofibroma cutáneo es una entidad infrecuente, de la que recientemente se ha descrito la variante del adulto, que hoy día se considera independiente de la infantil. Clínicamente se manifiesta como un nódulo solitario, de consistencia firme y a menudo multilobulado. La lesión presenta una histopatología característica en la que se mezclan áreas 'miofibroblásticas' y hemangiopericitoiden. Presentamos el caso de un hombre de 73 años con un nódulo multilobulado localizado en muslo derecho de 10 años de evolución. La lesión era dolorosa a la manipulación. Se realizó una exéresis y el estudio histopatológico demostró un miofibroma cutáneo, con la particularidad de presentar algunos nódulos a distancia de la lesión principal. No ha presentado recidiva tras 2 años de seguimiento. Realizamos una revisión de esta entidad (AU)

Leucoedema frente a nevo blanco esponjoso. A propósito de un caso / Leukoedema versus white sponge nevus. A case report

El leucoedema es una patología oral supuestamente muy frecuente, mientras que el nevo blanco esponjoso es una rareza. Sin embargo, ambas entidades son muy parecidas en cuanto a clínica, histopatología, microscopia electrónica, evolución y tratamiento. Se presenta el caso de un varón de 46 años con lesiones blanquecinas en la mucosa oral. Se realiza un exhaustivo diagnóstico diferencial entre leucoedema y nevo blanco esponjoso, llegándose a la conclusión de que son entidades relacionadas (AU)

Foliculitis esclerosante de la nuca (acné queloideo) / Folliculitis nuchea scleroti cans (acne keloidalis nuchae)

Exponemos el caso de un varón negro de 35 años con una foliculitis esclerosante de la nuca(acne keloidalis nuclae). Presentaba unas pápulas de 1 año de evolución de consistencia dura, estables, pero con períodos de exacerbación. Dado que eran asintomáticas se dejó sin tratamiento. Se realiza una revisión de esta entidad (AU)