[Lymphangioma circumscriptum neviforme].
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Child↗
Biomedical subjects
O Tellechea
Publications and source records attributed to O Tellechea.
Nasal septum perforation as the presenting sign of lupus erythematosus.
Nasal septum perforation is an uncommon and not well known feature of lupus erythematosus (LE). In general, it occurs during exacerbations and in a context of systemic vasculitis. Very rarely it can be a presenting sign, accompanying more usual manifestations of LE. We report the case of a 30-year-old woman who presented with a 2-year history of painful, slowly progressive nasal septum perforation. Laboratory study disclosed positive antinuclear antibodies, circulating immune complexes, hypocomplementemia, nuclear epidermal deposition of IgG in normal skin and transitory positive antiphospholipid antibodies. Symmetric peripheral joint arthritis, photosensitivity and diffuse alopecia subsequently developed. This case seems unique in that the nasal septum perforation occurred as an isolated presenting sign; it emphasizes the value of this feature in the diagnosis of LE.
Adult↗
Pemphigus vulgaris with nail involvement presenting with vegetating and verrucous lesions.
We report the case of a 68-year-old female with longstanding insulin-treated diabetes mellitus, observed for the first time in our department in August 1999 with multiple painful erosive lesions of the oral cavity and many bullous or erosive lesions on the abdominal wall, back, and thigh. She also had vegetating and verrucous lesions, similar to common warts, involving the hands and feet, mainly on the palms, palmar surface of the fingers, and nail folds. Her lesions were present for 1 year. Skin and mucous biopsies showed the characteristic histopathologic findings of pemphigus vulgaris, with an epidermal intercellular IgG deposition on direct immunofluorescence. Histology of a warty lesion of the finger also showed suprabasal acantholysis. After partial improvement with low doses of oral steroids and azathioprine, her disease progressed to involve the oral cavity, trunk, hands, feet, and scalp. Control of her disease required successive treatments of mycophenolate mofetil and cyclophosphamide, as well as corticosteroids. A partial response was obtained with all these treatments. After being controlled by cyclophosphamide that was slowly tapered, she is now well controlled with azathioprine and oral steroids, showing only discrete lesions of the oral mucosa after 1 year of followup. We report this case of pemphigus vulgaris with unusual clinical aspects, namely vegetating and verrucous lesions as well as nail involvement, rarely described in this disease.
Aged↗
Temporal arteritis presenting with scalp ulceration.
We report the case of a 75-year-old-woman who presented with bilateral scalp ulcerations and blindness, accompanied by severe headache and scalp tenderness, due to bilateral temporal arteritis without systemic involvement. A biopsy taken from the border of an ulceration showed evidence of giant cell arteritis. She was treated with oral prednisone, 60 mg per day. The ulcerations healed in a few weeks but the vision loss was irreversible. This case highlights for temporal arteritis the importance of accurate and timely diagnosis as well as the need for prompt therapy with systemic steroids in order to avoid major complications, namely loss of vision. It also demonstrates that scalp necrosis and ulcerations are skin signs associated with a poor prognosis.
Aged↗
[The blue rubber bleb nevus syndrome or Bean's syndrome. A rare cause of digestive hemorrhage].
A 71-year-old male who had previously suffered from chronic alcoholism was admitted to the Internal Medicine Service of Coimbra Hospital Center in January 1996 due to asthenia, loss of weight, icterus and abdominal pain, clinical features that had begun six months before admittance to hospital. A physical examination revealed that, in addition to icterus, the patient presented multiple hemangiomas of 1 to 5 cm in diameter, located in the oral cavity, neck, breast and left axilla. These lesions were bluish, elevated and with a rubber-nipple consistency, and had been developing for about 15 years. Subsequent examination revealed normocytic normochromic anemia, cholestatic icterus and the existence of a gastrointestinal hemangioma located in the esophagus. Excisional biopsy of an element proved that it was cavernous hemangioma. A subsequent angio-scintigraphy indicated other aspects suggestive of deep hemangiomas located in the legs, face and cervical region. The authors had the opportunity of examining other members of the patient's family, who apparently did not exhibit similar lesions. They concluded that it was a case of blue rubber bleb nevus syndrome (BRBNS), probably in its sporadic form. Treatment was essentially conservative and the patient is well.
Aged↗
[A skin lymphoepithelial tumor (skin lymphadenoma)].
The study of a case of cutaneous lymphadenoma and a literature review allowed us to verify that, in contrast to immunohistochemical findings, clinical and histological dates are basically reproducible. Accordingly the eccrine or hair follicle natures of the pleonasm cannot be either excluded or confirmed. The initial terms "cutaneous lympho-epithelial tumor" or "epithelial lymphohistiocytic tumor" seem more suitable than that of "cutaneous lymphadenoma" to describe this peculiar skin neoplasm.
Adult↗
Sebaceous adenitis.
A 32-year-old man had an asymptomatic erythematous, annular and circinate eruption on the face for 2 months. Histologic examination revealed sebaceous lobules surrounded by a lymphomonocytic infiltrate and some areas of necrotic sebocytes. The lesions healed without treatment in 1 month. These clinical features can be observed in "neutrophilic sebaceous adenitis," recently described by Renfro et al. The most striking abnormality is the nearly exclusive perisebaceous distribution of the dermal cellular infiltrate with inflammatory cell permeation of the sebaceous epithelium.
Adult↗
Oesophageal lichen planus.
Lichen planus is a common skin and mucosal disease, with very rare symptomatic oesophageal involvement. We report a case of painful dysphagia due to oesophageal lichen planus in a 60-year-old woman who also had oral, cutaneous and genital lichen planus lesions. Steroid treatment produced considerable improvement of all lesions and a rapid symptomatic remission.
Anti-Inflammatory Agents↗
Tubular apocrine adenoma with eccrine and apocrine immunophenotypes or papillary tubular adenoma?
A case of papillary tubular adenoma is reported. On microscopic examination the lesion, located on the scalp, showed a tubular-branching pattern, opening on the skin surface, and features of decapitation secretion. Immunohistochemical evidence of both eccrine and apocrine differentiation was found. This case, which on a clinicopathological basis alone could be classified as tubular apocrine adenoma, illustrates the difficulties in contrasting the latter to its eccrine counterpart (papillary eccrine adenoma) and suggests that the terms papillary tubular adenoma or tubulopapillary hidradenoma more accurately describe these lesions.
Adenoma↗
Dermal cylindroma. An immunohistochemical study of thirteen cases.
Thirteen dermal cylindromas (DC) have been studied immunohistochemically using a panel of antibodies that stain different portions of normal eccrine and apocrine glands. Distinct staining patterns were found in the different cell populations of the tumor. Although the expression of cytokeratins (CK) 19 and 1/10/11 in occasional duct structures could indicate excretory (ductal) differentiation, a link between DC and apocrine secretory coil is suggested by the expression of alpha-1-antichymotrypsin, lysozyme, human milk factor globulin 1, alpha smooth muscle actin (1A4), and CK 8 and 18. The presence of intermingled S-100 protein-, HLA DR-, and CD1a-positive cells argues for the existence of Langerhans cells within the neoplasm. DC shares epithelial membrane antigen, carcinoembryonic antigen, mucin-like carcinoma-associated antigen (B12), laminin, collagen IV, fibronectin, and CD34(QBEND/10) expression with both eccrine and apocrine glands.
Actins↗
Congenital sensory neuropathy with anhidrosis.
A 6-year-old girl had congenital sensory neuropathy with anhidrosis (CSNA), one of the five variants of a group of very rare genetic disorders of the peripheral nervous system--hereditary sensory neuropathies (HSN). Clinical, laboratory, and physiopathologic aspects are discussed. Dermatologic findings of anhidrosis and self-mutilation suggest the diagnosis.
Child↗
Monoclonal antibody Ber EP4 distinguishes basal-cell carcinoma from squamous-cell carcinoma of the skin.
Twenty-two cases of basal-cell carcinoma (BCC) and 21 cases of squamous-cell carcinoma of the skin (SCC) were stained for Ber EP4 using indirect immunofluorescence and avidin-biotin-peroxidase techniques. The results showed that all BCCs were diffusely and intensely labeled, whereas none of the SCCs expressed Ber EP4, irrespective of their histological type or grade of differentiation. This difference may prove useful in (a) histologically differentiating between these two neoplasms; and (b) in clarifying the histogenesis of neoplasms considered to be intermediate between BCC and SCC, or derived from BCC. In addition, because significant differences between the staining of hair follicles and BCC were noted, evaluation for Ber EP4 expression could assist in distinguishing between BCC and hair follicles in Mohs micrographic sections.
Antibodies, Monoclonal↗
Trichilemmal carcinoma: review of 8 cases.
Clinical and histological data from 8 trichilemmal carcinomas are reviewed. The tumors occurred mainly on sun-exposed skin of the face of elderly people as small solitary, frequently ulcerated nodules. Histologically, they consisted of multilobulate, infiltrative growths, connected to the epidermis and pilosebaceous structures and showing features reminiscent of the outer root sheath of the hair follicle. The presence of lobules of clear, glycogen-rich cells with peripheral palisading, hyaline basement membranes, trichilemmal keratinization, and abortive follicular sheaths and the absence of ductal or acinar differentiation allow distinction from other clear cell tumors of the skin. Although the histological picture suggests a high-grade malignant neoplasm, trichilemmal carcinoma has an indolent course. No recurrence or metastases have been observed.
Aged↗
[Eccrine porocarcinoma. A review of 24 cases].
From 1982 to 1992, 24 cases of eccrine porocarcinomas were recorded in our files. They always present as solitary well circumscribed nodules or plaques of 0.5 to 2 cm and they are mainly located on the face and the lower limbs in patients, predominantly females, aged of more than 5 decades. The average evolution time before diagnosis was 2 years; a case was observed in a 12-y-old patient with xeroderma pigmentosum. After surgical removal, only one local recurrence and one case with lymph node metastases were observed. The lesions showed two different histological types: the former is initially characterized by the intra-epidermal spreading of well circumscribed cell nests and by a secondary invasion of the dermis, the latter exhibits a trabecular pattern penetrating the dermis, sharing some microscopic aspects with the benign eccrine poroma. Whatever the histological type, the tumor is mainly built up by small cuboidal cells together with some large clear cells; the latter exhibit often atypias, which are focally distributed in the trabecular poroma-like variant. Dyskeratosis, malpighian metaplasia and necrotic comedo-like foci are frequently encountered. Ductal differentiation may be intracellular and occur together with focal keratinization. In one case with intra-epidermal cell islets, lymph node metastases showed the same cellular pattern; this superficial spreading type of porocarcinoma has the same malignant behaviour as the trabecular penetrating type. The eccrine porocarcinomas derive from the acrosyringeal cells: the ductal structures and the foci of keratinization express the epithelial membrane antigen (EMA), the carcino-embryonic antigen (CEA) and the alpha-lactalbumine related antigen; the tumor cells are S100 proteine negative.(ABSTRACT TRUNCATED AT 250 WORDS)
Adenocarcinoma↗
Desmoplastic trichilemmoma.
Seven cases of desmoplastic trichilemmoma (DT), a recently described pseudomalignant variant of trichilemmoma, are reviewed. The tumor generally occurs in men after the fifth decade of life and presents as a small solitary nodule on the face. It is frequently misdiagnosed clinically as a basal cell carcinoma or a papilloma. Histologically DT displays a superficial lobular growth arranged about a central prominent desmoplastic stroma. At the periphery, the tumor lobules show the typical features of trichilemmoma. In contrast, at the center the cells assume a more random pattern of cords and strands traversed by the hyaline stroma, mimicking invasive carcinoma. The tumor's architectural pattern, in particular the perilobular hyaline mantle, enables DT to be differentiated from basal cell carcinoma and malignant trichilemmoma. Immunohistochemical analysis failed to demonstrate human papilloma virus (HPV), epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), and alpha-lactalbumin in tumor epithelium. Keratin was expressed by the central pseudoinvasive epithelial cords. Neither factor XIIIa nor keratin expression was found in the stromal cells, which stained only for vimentin. These findings suggest that DT is not an HPV-induced epithelial proliferation and that the stroma is not the result of degenerative changes in tumor epithelium. Instead, there appears to be a fibroblast-mediated, dendrocyte-independent, stromal reaction producing this appearance.
Aged↗
[Cutaneous fungal embolism disclosing infection of aorto-iliac prosthesis].
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Aorta, Abdominal↗
[Dermal myxoma of nerve sheats (neurothecoma)].
The histological study of a painful cutaneous nodule located in the interscapular region, which had been present for 20 years in a 59-year old male patient has show typical features of dermal myxoma of the nerve sheaths. This was a lobulated myxomatous tumour with fusiform and dendritic cells plus a few epithelioid and multinucleate cells. Immunostaining was positive for the S100 protein and negative for factor XIIIa and EMA. These data suggest that this tumour was of schwannian origin.
Diagnosis, Differential↗