Search

We report an 11-year follow-up of a case of granulomatous slack skin. The patient was first treated surgically followed by a rapid relapse. Then he was treated by alpha-interferon during 15 months. When this treatment was stopped, the disease relapsed again. Extensive surgery was undertaken. Recently, a new relapse has occurred which was again treated by extensive surgery. No other manifestation of a lymphoproliferative disorder appeared. The clinical, histological, immunohistochemical, ultrastructural, molecular biological and genetic studies with a review of 39 other cases described in the literature are presented., (Copyright 2003 S. Karger AG, Basel)

Thirty-nine tumor-bearing patients with metastatic melanoma were treated with 3 subcutaneous injections of the MAGE-3.A1 peptide at monthly intervals. No significant toxicity was observed. Of the 25 patients who received the complete treatment, 7 displayed significant tumor regressions. All but one of these regressions involved cutaneous metastases. Three regressions were complete and 2 of these led to a disease-free state, which persisted for more than 2 years after the beginning of treatment. No evidence for a cytolytic T lymphocyte (CTL) response was found in the blood of the 4 patients who were analyzed, including 2 who displayed complete tumor regression. Our results suggest that injection of the MAGE-3.A1 peptide induced tumor regression in a significant number of the patients, even though no massive CTL response was produced.

Mycobacterium simiae is an ubiquitous species rarely involved as a cause of human infection. Its pathogenicity remains therefore unclear and controversial. Disseminated infections with M. simiae occur rarely and only 7 cases have been reported in patients with acquired immunodeficiency syndrome (AIDS). At least, two were mixed infections caused by M. simiae and M. avium-intracellulare. We report the case of two AIDS patients presenting with pure M. simiae infections: one was a disseminated infection and the other a pulmonary infection. Epidemiology and pathogenicity of M. simiae in pulmonary, extra-pulmonary and disseminated infections are reviewed.

About two cases of Buschke-Loewenstein tumor--one of the penis in man infected with HIV and another of perianal area-, the authors insist on the relative frequency of Buschke-Loewenstein tumor in non-circumcised and homosexual groups. They emphasize the continuous precancerous spectrum of Buschke-Loewenstein tumor.

Mycobacterium simiae is commonly found in nature and its role as a pathogen has been controversial. A case of disseminated M. simiae infection with blood, pulmonary and cutaneous localization is reported here. The pathogenic role of M. simiae was clearly demonstrated as it was the only organism isolated from sputum, broncho-alveolar lavage fluid, as well as blood and skin tissue. Identification of M. simiae by conventional testing may be difficult. Analysis of fatty and mycolic acid patterns, as performed in this case, is necessary to confirm its identification.

Human genes MAGE-1 and MAGE-3 code for antigens that are recognized on melanoma cells by autologous cytolytic T lymphocytes. These antigens may constitute useful targets for specific anti-tumor immunization of cancer patients, since genes MAGE-1 and MAGE-3 are expressed in a number of tumors of different histological types, but are not expressed in normal adult tissues other than testis. This also applies to genes MAGE-2 and MAGE-4, which are closely related to MAGE-1 and MAGE-3. We have analyzed the expression of these 4 MAGE genes in cutaneous melanoma. Sixteen of 100 primary tumors vs. 69 (48%) of 145 metastases from individual patients expressed MAGE-1. Similar differences in the frequency of gene expression between primary and metastatic tumor samples were observed for MAGE-2, MAGE-3, and MAGE-4. MAGE expression in primary tumors was correlated with tumor thickness: there was a significantly increased frequency in the expression of MAGE-1, -2 and -3 in tumors of greater thickness. Benign and dysplastic nevi, as well as in situ melanomas, did not express any of the 4 MAGE genes.

A 63-year-old woman presented with a large, subumbilical ulcerated nodule that was a Ki-1+ anaplastic large-cell lymphoma with exclusively cutaneous localization. This high-grade lymphoma, in this case, has a less severe prognosis. Surgical resection or local radiotherapy seems sufficient for the localized cutaneous forms.

Background: Subcutaneous ependymomas have been reported rarely in dermatologic reviews and, apparently, were never associated with other cutaneous malformations., Methods: A 60-year-old woman with a retrolumbar subcutaneous ependymoma and a giant bathing-trunk nevocellular nevus submitted to thorough dermatologic and neurologic investigation. The surgical material was extensively analyzed with light and electron microscope. The literature was reviewed., Results: The tumor fits exactly the classical prerequisites of the clinical and pathologic diagnosis. In contrast with the literature, it developed at 53 years, apparently after a trauma, within a congenital giant nevocellular nevus, at the retrolumbar level; it proved unrelated to any spinal cord alteration and so far appeared quite benign., Conclusions: The observation of a retrolumbar subcutaneous ependymoma is reported with detail; this tumor exceptionally recognized by dermatologists must be included in the differential diagnosis of lumps arising in the retrolumbar-retrosacral area. The most peculiar feature was its development within a giant bathing-trunk nevocellular nevus; such a fascinating association of two neurectodermal defects, as far as known, is presented for the first time.

A patient with chronic myeloid leukaemia was treated with hydroxyurea and developed longitudinal melanonychia on 10 of 20 nails. Such intense lesions are extremely rare.

We report the clinicopathological features of an old woman with a recent and progressive development of soft white-yellow papules of the neck and supraclavicular areas reminding of pseudoxanthoma elasticum without systemic involvement. The similarity of this entity with fibroelastopathic papular dermatoses of the neck is briefly discussed.

Mammary and extramammary Paget's disease is believed to remain non-pigmented, in contrast to superficial pagetoid melanoma; the same holds true for epidermotropic metastases of breast carcinoma. We recently had the opportunity to observe intensively pigmented epidermotropic metastasis of breast carcinoma, with dispersion of melanocytes in the dermal infiltrate. Similar observations, although exceedingly rare, have been reported: melanocytes 'may' pullulate within the dermo-epidermal foci of malignant epithelial cells. The final diagnosis relies on very careful analysis and, as far as possible, on immunohistological techniques.

About a case of pulmonary and dermatologic manifestations of sarcoidosis, in a Black African patient, the authors emphasize the clinical polymorphism of sarcoidosis. They insist on the diagnostic criteria of sarcoidosis which are not at all specific of the disease but necessitate a compatible interpretation; particularly in Black Africa where leprosy, tuberculosis and many others granulomatous diseases are endemic.

Myiasis, a rare infestation caused by mainly tropical fly larvae, is now more often encountered in Europe, because of the increasing intercontinental trips. A recent case allowed us to study some anatomopathological and entomological aspects of the African fly maggot.

The ultrastructural aspects of nucleus and cytoplasm of tumor cells and the interstitial material of dermatofibrosarcoma protuberans are described in detail, especially the very peculiar labyrinthic nucleus. Opinions about its development are reviewed; some, such as melanocytic, neural, histiocytic and smooth muscle proliferation, might be discarded. The myofibroblast appears as the most probable stem cell.

This is a review of historical facets of research in cutaneous neurohistology. The silver stains, used for some 150 years, led to the discovery of the neurone theory and to contemporary comparative studies. The cholinesterase methods, used since 1950, are the most convenient for general studies of nerves. The fluorescence technique of Falck (1962) is valuable for the study of adrenergic fibers. The electron microscope techniques used since 1950 have allowed the comprehensive description of myelinated and unmyelinated nerve fibers and of corpuscles. The most important unsettled questions pertain to the physiology of cutaneous nerves and precise definitions of cutaneous sensibility.

An ultrastructural investigation of erythropoietic protoporphyria reveals the high complexity of the basal complex that surrounds the superficial capillaries. There is a tremendous replication of the usual basement membrane, but in chips, with the constitution of an uneven network within an amorphous substance. Few collagen fibrils and microfibrils are present. Furthermore the architectural upheaval is characterized by the penetration of endothelial cell pseudopoda and of slender processes of perithelial cells. The basal complex is clearly encompassed by a layer of epithelial cells. The overall thickness reaches 5 micra.

The investigation and follow-up of three cases of EPP led us to reevaluate the signs and symptoms of the disease, the usefulness of early skin biopsies and protoporphyrin and ferrochelatase determinations. A review of the literature brought forth the salient features of this inborn error of porphyrin metabolism and stressed the value of treatment with beta-carotene and canthaxantine but also the risk of the so-called "gold dust" retinopathy.

Erythropoietic protoporphyria (EPP), a recently described form of porphyria, often remains unrecognized. We report the case of a 7-year-old girl admitted for investigation of photosensitivity since the age of 18 months without any significant objective cutaneous lesions. Clinical features, quantitative determinations of porphyrins in blood, urine and stools, ferrochelatase activity and cutaneous histopathology helped to confirm the diagnosis of EPP. A familial study was also performed. The clinical, laboratory and genetic characteristics of EPP are reviewed.

Calcium deposits develop in the vacuoles of fibroblasts and histiocytes within the granuloma at the periphery of the nodules. There is no clue for an epithelial origin.

Dermal alternariasis represents one of the new infectious diseases related to immunodepression, especially in kidney recipients. Periodic acid-Schiff and Grocott stains of paraffin sections and mycological cultures must be resorted to in order to establish the diagnosis. Ketoconazole should be the best treatment.

An ultrastructural investigation of dermal altenariasis has complemented previous studies. Alternaria tenuis was seen to be profusely present within the cytoplasm of multinucleate giant cells. Healthy elements were characterized by a thick, electron-lucent wall, ovoid osmiophilic inclusions and a poorly defined nucleus. Homogenous liquefaction and granular degeneration represented various types and steps of degradation; the wall subsisted for a while before collapsing and breaking into pieces. Penetration of the giant cell's cytoplasm within hollow tubes was observed.

A 46-year-old female, otherwise healthy, developed erythematous, pseudotumoral nodules, mainly on the hands; biopsies led to the diagnosis of Woringer-Kolopp disease. Anti-anergic chemotherapy with levamisole proved hazardous but, possibly, beneficial. Classification into benign localized, fatal generalized and intermediate disease is briefly commented upon. Differential diagnosis and tumor origin are discussed; while the Merkel cell hypothesis has been recently advanced strong analogies and slight discrepancies with mycosis fungoides advocate the T cell lymphoma theory.

Six patients of the same family present with micronodular and generalized familial angiomatosis. Four of them have been investigated. Their problem is purely esthetic; however an asymptomatic form of von Willebrand disease has been found in a female and one of her daughters. The light microscope reveals a network of dilated capillaries in the superficial dermis. Electron microscope investigation of the endothelium demonstrates on abundance of Weibel-Palade bodies, the presence of osmiophilic inclusions within clear vacuoles, the occurrence of long spacing collagen fibrils in the vicinity of endothelial and perithelial cells; furthermore, cytoplasmic projections within the lumen constitute the most dramatic and constant feature: there are many villosities, loops, coils, tufts and entanglements in all four cases.

A Stewart-Treves angiosarcoma (Stewart-Treves syndrome) developed 14 years after mastectomy (Halsted) and radiotherapy for breast cancer in a woman now 56 years old. Limited excision, graft and complementary radiotherapy have controlled the disease for 20 months. The microscopical and ultrastructural features of this angiosarcoma are presented and the last advances in immunopathology briefly commented.

While treated with isoxicam, a middle-aged lady developed first diffuse maculopapules and afterwards a syndrome of Lyell; similar observations of bimodal toxicodermatitis have been reported in the literature.

Clinical and histological features of idiopathic calcinosis of the scrotum are presented; calcium deposits are never associated with epithelial or connective tissue alterations. Identical lesions may arise in the skin at large; discrepancies and similitudes with cutaneous calculi are underlined.