Hemangioendotelioma retiforme: descripción de dos casos y revisión de la literatura

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with distinctive morphological features that, provided a high rate of local recurrences an occasional metastases, was first described as an angiosarcoma of flow malignancy grade. Twenty three well-documented cases have been previously reported. We report two new cases of RH and review the literature. One patient was a 82 year old female with a slow growing, indurated, and ill-defined plaque in the dorsum of the right hand; the other patient was a 31-year-old female with a tumor in the sole. Histologically both tumors showed the characteristic features of RH with a dermohypodermal infiltrative proliferation of elongated, arborescent and thin-walled vascular channels lined with hobnail endothelial cells without atypia nor mitotic figures. The differential diagnosis has to be made with Dabska’ tumour, hobnail hemangioma and conventional angiosarcoma. The report of new cases of this infrequent vascular tumour can be of help in determining more precisely the clinical and morphologic spectrum of RH.     

Xantomatosis cerebrotendinosa: descripción de 4 casos

Cerebrotendinous xanthomatosis (CTX) is an uncommon autosomal recessive disease caused by mutation of the CYP27A1 gene. It is characterized by the presence of xanthomas in different tissues, principally brain and tendon, due to the accumulation of β-cholestanol. Diagnosis is confirmed by measurement of serum β-cholestanol and urinary bile alcohol levels. Therapy with chenodeoxycholic acid has been shown to be the most effective treatment and can halt progression of the disease. We present 4 patients with a history of neurological disorders since childhood and who were diagnosed with CTX after developing tendon xanthomas. Although diagnostic suspicion depends to a large extent on recognition of tendon xanthomas, these are not an early sign of the disease, which can present with neurological disorders, cataracts, and chronic diarrhea. Early diagnosis of CTX therefore rests on measurement of serum β-cholestanol levels, even in absence of tendon xanthomas.     

Efluvio en línea de aparición inmediata asociado a trasplante capilar de alta densidad: descripción de 28 casos

We describe 28 patients who experienced effluvium with previously unreported features shortly after hair transplant surgery. Notable features were as follows: a) a linear morphology; b) immediate onset (1-3 days); c) association with dense-pack grafting in areas of receding hairline at the temples (Mickey Mouse pattern); d) a progressive increase in the diameter of the hair loss line (wave-like pattern); e) in some cases, subsequent concentric linear effluvium on the crown (donut pattern); and f) other forms of previously unreported immediate-onset effluvium. The linear morphology could be the result of dense packing, which can cause perilesional hypoxia and loss of miniaturized hairs around the recipient area. Since linear hair loss can cause patient concern about graft failure, we recommend taking images of transplanted and nontransplanted areas immediately after surgery and warning patients in advance about these transient effects, which are fully reversed in 3 months.     

Miasis forunculoide: descripción de dos casos con estudio histológico de las diferentes larvas

—Myasis means infestation of organs and tissues of vertebrates by larvae of dipterous. We report two cases of furuncular myiasis, uncommon disorders in Spain because the species of the flies responsible for the process do not live in our country. The first case was a myiasis due to Cordylobia anthropophaga, or tumbu fly, that usually resides in the countries of Africa at the Sounth of the Sahara. The second case was due to Dermatobia hominis, which usually resides in the countries of Central or South America.Cutaneous lesions consisted of nodular lesions with superficial ulcers and larvae emerging from the ulcers. These larvae were studied histopathologically, emphasizing the characteristics that allow a differential diagnosis between them.     

Carcinoma basocelular de la vulva. Descripción de dos casos y revisión de la literatura

—Although basal cell carcinoma is the most common malignant tumor of the skin, vulvar basal cell carcinoma accounts for less than 5% of all vulvar neoplasms.We are reporting the cases of basal cell carcinoma of two women aged 43 and 66, that showed vulvar ulcer, itching and bleeding. Both patients had excisional biopsy; one developed local recurrence and was treated by wide local reexcision.Basal cell carcinoma is a rare but important consideration in the differential diagnosis of cutaneous vulvar lesions. Accurate diagnosis depends upon a high index of suspicion, biopsy and histopathologic examination. Complete local excision is curative, although the recurrence rate is high, and metastases have been reported.     

Cirugía reconstructiva de la región cigomática-medial de la mejilla: Presentación de 5 casos

The cheek is the largest anatomical subunit of the face. It is a bilateral structure and symmetry must therefore be preserved. Peripherally it is related to important natural orifices whose location must also be maintained during surgical reconstructions. This is particularly important in the medial zygomatic subunit, whose delicate junction with the lower eyelid means that care must be taken to avoid ectropion. We present 5 options for the reconstruction of surgical defects secondary to the excision of tumors in this region.     

Lupus eritematoso discoide crónico en la infancia. Presentación de dos casos

—Discoid lupus erythematosus is uncommon in childhood. This disease presents several important differences with its adults counterpart: a male predominance, a low indicence of photosensivity and frequent progression to systemic lupus erythematosus.Two cases of childhood «discoid lupus erythematosus» in girls with onset age 4 and 9-years are described. They presented generalized and localized disease respectively and they have been a favorable response to antimalarials.