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J M de Moragas

Publications and source records attributed to J M de Moragas.

At least 19 recordsLinked to original sources
PubMed2001

Clinical, histopathologic, and therapeutic aspects of subepidermal autoimmune bullous diseases with IgG on the floor of salt-split skin.

BACKGROUND: About 12% of patients with subepidermal autoimmune bullous disease and immunoglobulin G (IgG) at the dermal-epidermal junction present diseases other than bullous pemphigoid. MATERIALS AND METHODS: We report the clinical, histopathologic, and therapeutic aspects of eight cases of subepidermal bullous disorder with IgG on the floor of salt-split skin. RESULTS: A predominant neutrophilic infiltrate was detected in six of the eight patients. In one patient, the inflammatory infiltrate was neutrophilic and eosinophilic in the same proportion. A good response to dapsone alone or combined with prednisone was observed in six patients. CONCLUSIONS: The salt-split skin direct immunofluorescence test is useful for its diagnostic and therapeutic implications.

Adult↗
PubMed1999

Repeat direct immunofluorescence (DIF) test, using, 1 M NaCl treated skin, in the subepidermal autoimmune bullous diseases that contain IgG at the dermal epidermal junction.

Knowledge of autoimmune bullous diseases has greatly increased with the recognition of new entities, and the use of the direct immunofluorescence (DIF) using 1 molar per liter of sodium chloride (1 M NaCl) treated skin has been proposed. To estimate the frequency with which the different DIF patterns are present, we performed a systematic study of the skin or oral mucosa samples in which linear deposits of IgG at the basement membrane zone were detected by routine DIF in the last 6 years. The DIF tests were done on 56 samples before and after splitting the epidermis from the dermis with 1M NaCl. In 40 biopsies (72%) IgG was found on either the epidermal side or on both sides after 1M NaCl split. These cases corresponded to bullous pemphigoid (n=33), herpes gestationis (n=5) and cicatricial pemphigoid (n=2). In 6 cases (10.7%), IgG deposits were observed only on the floor, five corresponding to bullous pemphigoid and one to bullous pemphigoid-like eruption induced by amoxicillin. Repeat direct immunofluorescence using 1M NaCl split skin indicates that at least 12% of patients who were initially diagnosed as bullous pemphigoid, may in fact suffer a different entity, requiring other techniques to achieve the right diagnosis. This test can be a useful routine screening for autoimmune bullous diseases.

Dermis↗
PubMed1999

Amelanotic lentigo maligna melanoma: report of a case and review of the literature.

Amelanotic lentigo maligna melanoma (ALMM) is an infrequent presentation of lentigo maligna melanoma, less than thirty cases having been reported to date. Hypopigmented or erythematous macules on the face of older women, resembling Bowen's disease or eczema, are the most common clinical presentation. We report a case of ALMM in a 73-year-old woman. Therapeutic trials with cryotherapy, 5-fluorouracil, and azelaic acid were unsuccessful, and the lesions were eventually cured by surgical excision. ALMM requires early clinical suspicion and histopathologic confirmation of diagnosis in every patient presenting with a slowly enlarging erythematous or hypopigmented macule, especially when located on the face of an older woman with a light complexion.

Aged↗
PubMed1998

Multiple follicular hamartomas with sweat gland and sebaceous differentiation, vermiculate atrophoderma, milia, hypotrichosis, and late development of multiple basal cell carcinomas.

We describe a 46-year-old woman with multiple symmetrical papulocystic lesions on the face, neck, chest, back, and upper arms since childhood. Vermiculate atrophoderma, entropion, and progressive loss of eyelashes was also noted. Since the age of 30, 12 basal cell carcinomas have developed on the face. Histopathologic examination revealed multiple anastomosing nests and strands that arose from the lower part of a hair follicle in early lesions. In more advanced lesions, multiple keratinizing microcysts within a moderately sclerotic stroma were noted in the upper and mid dermis. In some biopsy specimens, focal areas showing sweat gland (ductal) and sebaceous differentiation were also observed. Basal cell carcinomas developed from the basaloid component of the hamartomatous proliferation.

Atrophy↗
PubMed1998

Occurrence of hepoxilins and trioxilins in psoriatic lesions.

We recently found that normal human epidermis produces relatively high amounts of hepoxilins and trioxilins in vitro. Therefore, the aim of this study was to demonstrate the presence of these compounds in psoriatic lesions. Extracts from scales of patients with chronic stable plaque psoriasis were analyzed by a combination of high performance liquid chromatography and gas chromatography-mass spectrometry techniques. We found that the levels of hepoxilin B3 were more than 16-fold higher in psoriatic scales than in normal epidermis (3.2+/-2.3 and < 0.2 ng per mg, respectively), whereas hepoxilin A3 was not detected in any sample. Trioxilins were semiquantitated and referred to 12-hydroxyeicosatetraenoic acid, ratios of trioxilins A3 and B3 12-hydroxyeicosatetraenoic acid in psoriatic lesions were 0.65+/-0.23 and 0.32+/-0.28, respectively, and they were not detected in normal epidermis. The presence of a great amount of trioxilin A3 strongly suggests that hepoxilin A3 was present in psoriatic lesions and it was totally degraded to trioxilin A3 during the analysis procedure. Our results demonstrate that hepoxilins and trioxilins are produced by human skin in vivo and that the levels of these compounds are increased in psoriasis. The reported biologic activities of hepoxilins indicate that they could amplify and maintain the inflammatory response. Our results reinforce the idea that these compounds could play a role as mediators in the inflammatory response in skin, particularly in psoriasis.

8,11,14-Eicosatrienoic Acid↗
PubMed1998

Brooke-Spiegler syndrome variant: segregation of tumor types with mixed differentiation in two generations.

Brooke-Spiegler syndrome (BSS) is an autosomal dominantly inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. Other lesions have been reported to occur in patients with BSS, including parotid basal cell adenomas, milia, organoid nevi, basal cell carcinomas, and spiradenomas. Spiradenomas and cylindromas have so many features in common that they have been regarded as polar extremes belonging to a spectrum of cutaneous adnexal neoplasms. We report on a 61-year-old woman with multiple spiradenomas on the scalp and periauricular areas and her 28-year-old daughter, with multiple facial trichoepitheliomas. Occasional features of pilar and ductal differentiation were found in tissue specimens of tumors from the mother and daughter, respectively. Co-existence of multiple spiradenomas and trichoepitheliomas and segregation of the predominant type of tumor in different members of a family with BSS have been reported occasionally, but never, to our knowledge, in combination. The presence of mixed differentiation in tumor specimens from both patients provides additional evidence in support of the folliculosebaceous apocrine unit (FSAU) hypothesis. Mutations in genes regulating proliferation and differentiation of putative stem cells of the FSAU would give rise to different combinations of adnexal skin tumors as well as to other neoplasms.

Adult↗
PubMed1998

Kaposi's sarcoma in a patient with erythroblastopenia and thymoma: reactivation after topical corticosteroids.

We report a 69-year-old female with erythroblastopenia and thymoma who developed lesions of Kaposi's sarcoma (KS) after thymectomy, 2 months after the initiation of therapy with methylprednisolone. Control of mucocutaneous KS lesions was obtained with radiotherapy, interferon alfa-2b and withdrawal of systemic immunosuppressive therapy. Erosive oral lichen planus appeared later, and after therapy with topical corticosteroids a new lesion of KS developed that regressed after withdrawal of topical corticosteroids. The detection of HHV-8 only in lesional skin supports the hypothesis that this virus can trigger the development of KS lesions.

Administration, Topical↗
PubMed1998

[Multiple and unilateral angiofibromas of the face: forme fruste of Bourneville tuberous sclerosis].

BACKGROUND: Multiple facial angiofibromas are considered a characteristic and pathognomonic feature of tuberous sclerosis. In contrast, the observation of localized multiple angiofibromas limited to one side of the face is a uncommon. OBSERVATIONS: We cared for two patients with multiple clustered unilateral angiofibromas (although one patient also presented a few angiofibromas on the other side) without other features associated with the sclerosis tuberous complex. DISCUSSION: The possibility that these patients presented an independent disorder or a minor form of tuberous sclerosis is discussed.

Angiofibroma↗
PubMed1998

[Mucocutaneous manifestations in acute HIV infection. 3 case reports].

We report three patients who developed a generalized rash with oral, genital or perianal ulcerations as a result of acute infection due to HIV. The primary infection was diagnosed by seroconversion (by means of EIA and Western blot techniques). Definitive diagnosis was established on days 52, 85 and 97 after the appearance of the rash. The p24 protein of the HIV was only detected in the early phase of the disorder in the two cases in which this study was carried out.

Acute Disease↗
PubMed1997

[Hematologic deficiencies in patients with recurrent oral aphthae].

BACKGROUND: The prevalence of iron, folic acid and vitamin B12 deficiencies and their role in the development of recurrent oral ulcerations is not well known. The aim of this study was to determine the prevalence of these deficiencies in our patients. PATIENTS AND METHODS: Iron, folic acid and vitamin B12 levels were studied in 80 patients with recurrent oral ulcerations (ROU) and the results were compared with a control group of 29 patients with different oral diseases. RESULTS: In the recurrent oral ulcers patients, deficiencies were detected in 21/80 patients (26.2%). In 18 cases they were pure: iron (4), folic acid (10) and vitamin B12 (4). In 3 patients, combined deficiencies were detected, being secondary to pernicious anaemia in two patients. In the control group, deficiencies were observed in 4/29 cases (13.7%). In three cases they were isolated (one case suffered from ferropenic anaemia and two patients of pernicious anaemia). CONCLUSIONS: Patients with recurrent oral ulcerations have more frequently iron, folic acid and vitamin B12 deficiencies than those with other diseases of oral mucosa. However, there were not significant differences when the frequency of deficiency of each one of such elements were taken into account separately.

Adolescent↗
PubMed1997

Systemic lupus erythematosus presenting with red lunulae.

Red lunulae have been observed in association with a large spectrum of cutaneous and systemic diseases. We describe a 13-year-old girl with painful red lunulae of the fingernails who subsequently developed systemic lupus erythematosus. The appearance of Beau's lines suggested inflammation of the nail matrix. Oral prednisone produced significant improvement in the nail changes. To our knowledge, red lunulae have not been previously recognized as the presenting sign of systemic lupus erythematosus.

Adolescent↗
PubMed1997

Histopathological changes of primary HIV infection. Description of three cases and review of the literature.

The histopathological changes observed in the cutaneous rash of three patients who suffered the acute phase of HIV infection are described. In all three patients a perivascular and interstitial inflammatory infiltrate was present in the upper and mid-reticular dermis. In one biopsy isolated areas of epidermal necrosis were observed and in the two other biopsies a perifollicular inflammatory infiltrate was detected with perforation in one case. Furthermore, a periductal infiltrate was observed in one of these biopsies.

Adult↗
PubMed1997

Carpal tunnel syndrome presenting as sclerodactylia, nail dystrophy and acro-osteolysis in a 60-year-old woman.

Skin changes associated with carpal tunnel syndrome have rarely been reported. A 60-year-old patient was referred to our Department of Dermatology for evaluation of an aseptic gangrene of the distal phalanx of the third finger of her left hand, progressive nail dystrophy, sclerodactylia and spontaneous ulcerations. These features were seen on the digits with sensory supply from the median nerve. The patient had hand paresthesias for at least 10 years. No abnormal results were found in biochemical, hematological or immunological determinations. An echo-Doppler study of the upper limbs was normal. An electromyographic study was consistent with a bilateral carpal tunnel syndrome (CTS). Ulcerative and mutilating CTS can occasionally produce sclerodactylia with ulcerative lesions on distal fingers, nail dystrophy and acro-osteolysis as a consequence of mechanical compression of the autonomic fibers of the median nerve. The clinical picture includes Raynaud's phenomenon in some patients. Correct diagnosis is important, since ulcerative and mutilating CTS is a marker of a severe neural lesion, which is amenable to treatment by surgical decompression.

Carpal Tunnel Syndrome↗
PubMed1997

Primary neuroendocrine carcinoma of the skin expresses tyrosinase mRNA: detection by a specific nested PCR technique.

BACKGROUND: Tyrosinase transcripts have been identified in a wide range of normal organs and neoplasms such as cutaneous nevi, breast carcinomas, liposarcomas and schwannomas. The cells responsible for these positive results have not been accurately identified yet, but they might correspond to either fully differentiated melanocytes, melanocytic precursors or cells bearing potential for melanocytic differentiation. OBJECTIVE: To assess the expression of tyrosinase mRNA in surgical resection specimens and peripheral blood samples from 2 patients with primary neuroendocrine carcinomas of the skin (PNCS). METHODS: The expression of tyrosinase mRNA transcripts was tested by means of a reverse-transcriptase and nested PCR technique, using specific primers, in tissue samples from surgical specimens and heparinized blood samples from 2 patients with PNCS. RESULTS: The results of the test were positive in the 2 specimens of PCNS but negative in blood samples. CONCLUSION: Our findings provide further support for the hypothesis that PNCS originates from a pluripotent stem cell capable of neuroendocrine cell differentiation, keratin production, and, as reported herein, tyrosinase mRNA expression. It remains to be seen whether this technique can also be applied to patients with PNCS, as an adjunct to staging and treatment planning.

Aged↗
PubMed1997

Masson's intravascular papillary endothelial hyperplasia mimicking Stewart-Treves syndrome: report of a case.

Masson's intravascular papillary endothelial hyperplasia, also called Masson's pseudoangiosarcoma, represents a benign vascular proliferation, presently considered as a peculiar histopathologic reaction pattern of the endothelium to diverse stimuli. Differentiation from angiosarcoma represents the main diagnostic concern. We report a case of Masson's intravascular papillary endothelial hyperplasia presenting in a 55-year-old woman with ipsilateral lymphedema secondary to surgery and radiation therapy for breast carcinoma. The diagnosis was intravascular papillary endothelial hyperplasia. This is the first description of Masson's pseudoangiosarcoma in this clinical context, to our knowledge. We believe it represents a peculiar morphologic pattern of endothelium proliferation secondary to venous stasis and thrombosis due to lymphedema.

Arm↗
PubMed1997

Detection of Mycobacterium tuberculosis DNA in lobular granulomatous panniculitis (erythema induratum-nodular vasculitis).

OBJECTIVE: To determine, using polymerase chain reaction (PCR) amplification, if Mycobacterium tuberculosis complex DNA is present in the skin biopsy specimens of lobular granulomatous panniculitis. DESIGN: A retrospective descriptive study. SETTING: A university-based hospital. PATIENTS: From the 65 patients included in the study, we examined 72 paraffin-embedded skin biopsy specimens with a histologic diagnosis of erythema induratum or nodular vasculitis. The biopsy specimens were from the histopathological archives of the Departments of Dermatology and Pathology of the Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, from 1976 to 1994. Twenty-two biopsy specimens were excluded from the final analysis because we could not amplify the internal control. MAIN OUTCOME MEASURES: Detection of a 123-base pair fragment of the IS6110 insertion sequence specific for M tuberculosis complex. RESULTS: The results of PCR amplification were positive for M tuberculosis complex DNA in 77% of the skin biopsy specimens. No significant difference could be detected with respect to the age of the patients, ulceration of the nodules, reactivity to purified protein derivative, abnormal results of a chest x-ray examination, personal and family history of tuberculosis, and PCR results. The presence and degree of necrosis on histologic examination were significantly higher in the PCR-positive group (P = .04). None of the following variables were associated with PCR results: presence of vasculitis, degree of granulomatous infiltrates, number of giant cells, and presence of well-organized granulomas. CONCLUSIONS: The DNA of M tuberculosis can be detected in a considerable number of skin biopsy specimens of lobular granulomatous panniculitis. None of the clinical and histologic variables evaluated could accurately predict the results of PCR amplification.

Adult↗