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Biomedical subjects

Luis Requena

Publications and source records attributed to Luis Requena.

32 records · Page 2Linked to original sources

Acral arteriovenous tumor developed within a nevus flammeus in a patient with Sturge-Weber syndrome.

The Sturge-Weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with Sturge-Weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with Sturge-Weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with Sturge-Weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with Sturge-Weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.

Facial Neoplasms↗

Primary cutaneous signet-ring cell melanoma: a clinico-pathologic and immunohistochemical study of two cases.

Signet-ring cell malignant melanoma is a rare histopathologic variant of malignant melanoma with only a few described cases. We report the clinical, histopathological, and immunohistochemical features of two cases of primary cutaneous signet-ring cell malignant melanoma. Neoplastic melanocytes showed abundant clear cytoplasm compressing the nuclei to the periphery, sometimes resulting in a signet-ring appearance. Immunohistochemically, neoplastic melanocytes in both cases expressed immunoreactivity for the usual melanocytic markers S-100 protein and Melan-A, although only the second case resulted HMB-45 positive. We review the literature about this rare cytologic variant of malignant melanoma and discuss the differential diagnosis with other cutaneous neoplasms that may show a signet-ring cell appearance of their neoplastic cells.

Aged↗

Ductal carcinoma arising from a syringocystadenoma papilliferum in a nevus sebaceus of Jadassohn.

We present an example of ductal carcinoma connected to a syringocystadenoma papilliferum situated in a nevus sebaceus of Jadassohn on the scalp of a 22-year-old woman. The ductal carcinoma involved the entire thickness of the dermis and extended to the subcutaneous fat. Because syringocystadenoma papilliferum is considered a hamartoma with apocrine differentiation, the ductal carcinoma here described was interpreted as an apocrine ductal carcinoma. Syringocystadenocarcinoma papilliferum is an exceedingly rare neoplasm, most examples of which seem to have arisen in its benign counterpart, syringocystadenoma papilliferum. From a histopathologic point of view, syringocystadenocarcinoma papilliferum usually shows a papillary configuration similar to that of syringocystadenoma papilliferum. In contrast, the case here described a ductal carcinoma superficially connected to a syringocystadenoma papilliferum, but mostly composed of small ductal structures embedded in a desmoplastic stroma and involving the full thickness of the dermis. We review the literature about the malignant neoplasms arising in the nevus sebaceus of Jadassohn.

Adult↗

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.

Acneiform Eruptions↗

Circumscribed palmar or plantar hypokeratosis: a distinctive epidermal malformation of the palms or soles.

BACKGROUND: Epidermal malformations of the skin include a group of heterogeneous developmental defects that result from errors in morphogenesis of the epidermis during intrauterine life. OBJECTIVE: The purpose of this study was to report the clinical and histopathologic features of a distinctive epidermal malformation involving the skin of the palms or soles. METHODS: Ten patients were included in this study. All of them showed the same clinical features that consisted of a solitary circumscribed and circular area of erythematous depressed skin on the palm or on the sole. Diagnosis was confirmed by histopathologic study. RESULTS: All patients were middle aged or elderly. Nine patients were women and one was a man. The lesions showed predilection for the skin of the thenar and hypothenar regions of the palm or the medial side of the sole. Histopathologic study demonstrated a depression of the epidermis, with a sharp stair between normal and involved skin. The epidermis covering the depression showed markedly thinner horny layer and a slightly diminished granular cell layer when compared with adjacent noninvolved skin. Keratinocytes of the squamous cell layer, granular cells, and corneocytes showed, otherwise, a normal appearance. Serial sections failed to demonstrate cornoid lamellation. CONCLUSION: On the basis of the clinical and histopathologic findings in these 10 patients, we have named this malformation circumscribed palmar or plantar hypokeratosis. This lesion seems to be a distinctive entity that has not been previously described.

Adult↗

Acquired elastotic hemangioma: A clinicopathologic variant of hemangioma.

BACKGROUND: In recent years, many new cutaneous vascular proliferations have been described. This is partly due to the current epidemic of AIDS, which has forced dermatopathologists to establish the diagnosis of the earliest patch-stage lesions of AIDS-related Kaposi's sarcoma and some of the recently described cutaneous vascular proliferations that may histopathologically mimic lesions of early Kaposi's sarcoma. OBJECTIVE: This article reports the clinical, histopathologic, and immunohistochemical findings in 6 cases of a clinicopathologic variant of cutaneous hemangioma, which we have named acquired elastotic hemangioma. METHODS: Six cases of acquired elastotic hemangioma were studied from clinical, histopathologic, and immunohistochemical standpoints. The histopathologic differential diagnosis with other similar cutaneous vascular proliferations is discussed. RESULTS: Acquired elastotic hemangioma appears on the sun-damaged skin of the dorsal aspect of the forearms or on the lateral aspects of the neck of middle-aged or elderly women. Clinically, lesions present as solitary erythematous plaques with variable morphology and in only some cases have a clearly angiomatous appearance. On histopathologic examination, acquired elastotic hemangioma is characterized by a band-like proliferation of capillary blood vessels involving the superficial dermis and arranged horizontally parallel to the epidermis. CONCLUSION: Acquired elastotic hemangioma is a distinctive clinicopathologic variant of hemangioma that should be differentiated from other cutaneous vascular proliferations. On histopathologic examination, it is characterized by capillary proliferation involving the dermis.

Aged↗

Congenital giant melanocytic nevus with pigmented epithelioid cells: a variant of epithelioid blue nevus.

Epithelioid-cell blue nevus is an unusual cytologic variant of blue nevus that has been recently described mostly in patients with Carney complex, although the lesion may also appear in patients with no evidence of Carney complex. This variant of blue nevus is composed of melanin laden large polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive descent and, in contrast with the usual stromal changes in blue nevi, epithelioid-cell blue nevus exhibits no dermal fibrosis. This report describes a congenital giant melanocytic nevus with pigmented epithelioid cells located on the back of a 2-year-old male. The lesion was present at birth and the patient had no evidence of Carney complex. Histopathologically, the lesion consisted of a large and entirely intradermal melanocytic nevus composed of heavily pigmented epithelioid melanocytes involving the full-thickness of dermis, but extending also to the subcutaneous fat and underlying soft tissues. Immunohistochemically, epithelioid neoplastic melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A, NK1C3, and microphthalmia transcription factor (MiTF) antibodies. MIB-1 cellular proliferation marker was expressed in the nuclei of only a few scattered epithelioid melanocytes. This report demonstrates that epithelioid-cell blue nevus is a distinctive histopathologic variant of blue nevus that may also appear as a giant congenital melanocytic nevus.

Biomarkers, Tumor↗

Clear-cell porocarcinoma in situ: a cytologic variant of porocarcinoma in situ.

Poromas are benign neoplasms composed of poroid and cuticular cells. Four histopathologic variants of poromas are accepted, according to the architectural features of the neoplasm: hidroacanthoma simplex or intraepidermal poroma; eccrine poroma, which is a poroma connected to the epidermis that extends to superficial dermis; dermal duct tumor, which develops when the neoplasm is composed of small, solid aggregations of poroid and cuticular cells confined to the dermis with little or no connection with the epidermis; and poroid hidradenoma, which is a solid-cystic, dermal poroma. The malignant counterpart of hidroacanthoma simplex is named malignant hidroacanthoma simplex or porocarcinoma in situ. This report describes an example of clear-cell malignant hidroacanthoma simplex, a cytologic variant of porocarcinoma in situ, which, to our knowledge, has not been previously reported. In contrast with other clear-cell neoplasms, a relation with diabetes mellitus could not be clearly established in this case.

Acrospiroma↗

Pigmented mammary Paget disease and pigmented epidermotropic metastases from breast carcinoma.

Pigmented mammary Paget disease is a rare clinicopathologic variant of mammary Paget disease. It has been described in female and male patients with intraductal mammary carcinoma extending to the epidermis of the nipple and areola through a lactiferous duct. Pigmented cutaneous metastases from breast carcinoma are uncommon variants of epidermotropic metastatic breast carcinoma. All these lesions may mimic malignant melanoma clinically and histopathologically. From a histopathologic point of view, involvement of the dermoepidermal junction by neoplastic cells of the mammary carcinoma seems to be a prerequisite for development of the clinical pigmentation. We report three examples of pigmented mammary Paget disease and six cases of pigmented epidermotropic metastases from breast carcinoma, which were studied from both the histopathologic and immunohistochemical points of view. Two cases of pigmented mammary Paget disease and all cases of pigmented epidermotropic metastatic breast carcinoma showed the proliferation of dendritic melanocytes arranged as solitary units along the dermoepidermal junction and intermingled with the neoplastic cells of the mammary carcinoma in the superficial dermis. In one case of pigmented mammary Paget disease, there was abundant melanin within the cytoplasm of the Paget cells, but an increased number of melanocytes could not be demonstrated. Local production of melanocytic chemotactic factor by neoplastic cells of the mammary carcinoma when they reach the dermoepidermal junction has been postulated as the cause of the melanocytic proliferation and clinical hyperpigmentation of these epidermotropic breast carcinomas. Another possibility is the phagocytosis or transfer of melanin from melanocytes to the intraepidermal neoplastic cells of the breast carcinoma. Pigmented mammary Paget disease and pigmented epidermotropic metastatic breast carcinoma should be differentiated from melanoma clinical and histopathologically.

Adult↗

Benign vascular proliferations in irradiated skin.

Several types of cutaneous vascular proliferations have been described in areas of irradiated skin, including both benign lesions, such as benign lymphangiomatous papules, atypical vascular lesions, or benign lymphangioendothelioma, and malignant neoplasms such as high-grade angiosarcomas. This report describes the clinicopathologic features of 15 cases of different types of benign cutaneous vascular proliferations arisen within irradiated skin. All patients were female ranging in age from 33 to 72 years, and they had received postoperative external radiotherapy for treatment of breast carcinoma (14 cases) or ovarian carcinoma (one case). In those cases in which the latency interval period between radiotherapy and the development of the vascular lesion was known from the clinical records, the latency interval period elapsed between radiotherapy and diagnosis of the vascular lesion ranged from 3 to 20 years. The most common clinical presentation of the cutaneous lesions consisted of papules, small vesicles, or erythematous plaques on the irradiated field. Histopathologically, most lesions consisted of irregular dilated vascular spaces, with a branching and anastomosing pattern, thin walls, and lymphatic appearance involving the superficial dermis. A discontinuous single layer of endothelial cells with flattened nuclei lined these vascular channels, and numerous small stromal papillary formations also lined by endothelial cells projected into the lumina of the dilated lymphatic vessels. These cases were classified as benign lymphangiomatous papules or plaques. Two cases showed different histopathologic findings because they consisted of poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis and lined by inconspicuous endothelial cells. In some areas these irregular slit-like vascular spaces dissected collagen bundles of the dermis. These cases were classified as atypical vascular proliferations mimicking benign lymphangioendothelioma or patch-stage Kaposi's sarcoma. All cases showed similar immunohistochemical findings and the endothelial cells lining the vascular spaces expressed immunoreactivity for CD31, but they stained only focally positive for CD34 or were negative for this marker. Immunohistochemical investigations for alpha-smooth muscle actin failed to demonstrate a complete peripheral ring of actin-positive pericytes in most of the neoformed vascular structures. This immunohistochemical profile also supported the lymphatic nature of these vascular proliferations developed in irradiated skin. Although some of these lesions may mimic histopathologically patch-stage Kaposi's sarcoma or well-differentiated angiosarcoma, the follow-up of the patients of this series demonstrated that the vascular proliferations arisen in irradiated skin invariably showed a benign biologic behavior.

Actins↗

Gigantic cutaneous horns of the scalp: lesions with a gross similarity to the horns of animals: a report of four cases.

Gigantic cutaneous horns, grossly similar to the horns seen in animals, are exceedingly rare in humans. After finding one case in practice, we searched our departmental files for similar cases and examined them grossly and microscopically. Four cases were identified. All occurred as solitary lesions in older women on the parietal-occipital region of the scalp. They had a growth history of up to 30 years; the women hid these horns in their hair. Grossly, the horns were yellow-grey, and there were shallow furrows running along the length of the horns. The length ranged from 17 to 25 cm, and the width was up to 2.5 cm. All four lesions showed similar histologic changes. Microscopically, the gigantic horns consisted of a mixture of squamous epithelial cells and tricholemmal keratinized debris. In one case the base of the horn was directly connected with a mass composed of benign tricholemmal cysts of the scalp. Mitoses were common, but atypical mitoses were not observed. The nuclei of the squamous cells were bland without pleomorphism, hyperchromasia, or atypia. Follow-up of all patients was uneventful: all patients were well and without signs of recurrence or metastasis 2-15 years after the surgical excision. Gigantic cutaneous horns are rare and benign. We think that they represent an extremely well-differentiated variant of proliferating tricholemmal tumor with an unusual and remarkable gross pattern.

Aged↗

Erythema nodosum.

Erythema nodosum is the most frequent clinico-pathological variant of the panniculitides. The disorder is a cutaneous reaction consisting of inflammatory, tender, nodular lesions, usually located on the anterior aspects of the lower extremities. The process may be associated with a wide variety of diseases, being infections, sarcoidosis, rheumatologic diseases, inflammatory bowel diseases, medications, autoimmune disorders, pregnancy, and malignancies the most common associated conditions. The typical eruption consists of a sudden onset of symmetrical, tender, erythematous, warm nodules and raised plaques usually located on the shins, ankles and knees. Often the lesions are bilaterally distributed. At first, the nodules show a bright red color, but within a few days they become livid red or purplish, and finally they exhibit a yellow or greenish appearance taking on the look of a deep bruise. Ulceration is never seen and the nodules heal without atrophy or scarring. Some clinical variants of erythema nodosum have been described under different names, including erythema nodosum migrans, subacute nodular migratory panniculitis, and chronic erythema nodosum, but probably they are just clinical variants which may all be included within the spectrum of erythema nodosum. Histopathologically, erythema nodosum is the stereotypical example of a mostly septal panniculitis with no vasculitis. The septa of subcutaneous fat are always thickened and variously infiltrated by inflammatory cells that extend to the periseptal areas of the fat lobules. The composition of the inflammatory infiltrate in the septa varies with age of the lesion. In early lesions edema, hemorrhage, and neutrophils are responsible for the septal thickening, whereas fibrosis, periseptal granulation tissue, lymphocytes, and multinucleated giant cells are the main findings in late stage lesions of erythema nodosum. A histopathologic hallmark of erythema nodosum is the presence of the so-called Miescher's radial granulomas, which consist of small, well-defined nodular aggregations of small histiocytes arranged radially around a central cleft of variable shape. Treatment of erythema nodosum should be directed to the underlying associated condition, if identified. Usually, nodules of erythema nodosum regress spontaneously within a few weeks, and bed rest is often sufficient treatment. Aspirin, nonsteroidal anti-inflammatory drugs, such as oxyphenbutazone, indomethacin or naproxen, and potassium iodide may be helpful drugs to enhance analgesia and resolution. Systemic corticosteroids are rarely indicated in erythema nodosum and before these drugs are administered an underlying infection should be ruled out.

Diagnosis, Differential↗

[Multiple linear spiradenoma with a linear distribution].

We report the case of a 32-year old woman who presented with multiple nodules in the left arm and breast that had been present for 15 years. These nodules had a bluish colour and were occasionally painful. She had gone under excision of three other nodules on the left arm with the histopathological diagnosis of spiradenoma. All of the nodules were disposed in a linear arrangement and underneath a maculo-papular erythematous lesion which clinically seemed to be an epidermal nevus. The case described in the literature of multiple spiradenomas are very rare and multiple linear spiradenomas are even rarer.

Adenoma, Sweat Gland↗