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P Combemale

Publications and source records attributed to P Combemale.

At least 37 records · Page 2Linked to original sources
PubMed1998

The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases.

Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.

Adolescent↗
PubMed1998

[Infiltrating lipoma].

BACKGROUND: Deep or infiltrating lipoma is an often misdiagnosed clinical entity. We report two typical cases. CASE REPORTS: Two women, aged 92 and 62 years, were seen for a tumefaction on an upper limb with progressively increasing volume. The clinical presentation suggested deep lipoma, confirmed by magnetic resonance imaging and histology which eliminated liposarcoma, the main differential diagnosis. Surgical excision was successful. There has been no recurrence. DISCUSSION: These two observations recall the clinical, diagnostic and therapeutic features of infiltrating lipoma.

Aged↗
PubMed1998

[African tick-bite rickettsiosis: the 1st case of autochthonous infection?].

BACKGROUND: African tick-bite fever is caused by R. africae. All cases reported to date in France have occurred among patients who came from southern Africa, the endemic zone. We report the first case, to our knowledge, of a patient infected in France. CASE REPORT: A 69-year-old white man who had never left France developed fever and 3 "black spots" on the legs with lymphangitis and enlarged nodes. The clinically suspected diagnosis was confirmed by positive serology reactions to R. africae. As cross immunity with R. conorii can occur, the diagnosis was further confirmed by western blot for R. africae and by the persistence of the reaction after adsorption of the R. conorii serum. DISCUSSION: African tick-bite fever was identified as a clinical entity different from Mediterranean spotted fever by Kelly in 1992 who demonstrated the causal role of R. africae. The typical clinical presentation associates fever, several black spots, lymphangitis and multiple node enlargement. The serological diagnosis is difficult owing to cross immunity with R. conorii. Western blot and polymerase chain reaction are required for definitive diagnosis. In our case, the infection was probably due to a R. africae imported into France in the luggage of the patient's daughter who had spent 3 months in Zimbabwe.

Aged↗
PubMed1996

Alteration of matrix macromolecule synthesis by fibroblasts from a patient with pachydermoperiostosis.

Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a very rare genetic disease characterized by pachydermia, periostosis, arthralgia, and finger clubbing. Its pathophysiology is still unclear, but previous studies have reported connective tissue hypertrophy in the skin of these patients. We investigated the synthesis of collagen, fibronectin, and proteoglycans by fibroblasts from affected and unaffected skin from one patient with pachydermoperiostosis and four normal donors. We found that collagen synthesis was largely decreased in fibroblasts from the diseased skin, whereas the synthesis of the small dermatan-sulfate-containing proteoglycan decorin strongly increased. Fibroblasts from the unaffected skin of the patient exhibited syntheses of these macromolecules similar to control fibroblasts from healthy donors. Northern blot and dot blot analyses showed decreased pro alpha 1 (I) collagen in patient's affected and unaffected skin fibroblasts whereas increased decorin mRNA levels were found in fibroblasts from the patient's affected skin. No change in cell proliferation was observed. These data demonstrate an alteration of fibroblast biosynthetic activity in the skin lesions of pachydermoperiostosis, which may be responsible, at least in part, for the patient's phenotype.

Adult↗
PubMed1996

Translocation, t(17;22)(q22;q13), in dermatofibrosarcoma protuberans: a new tumor-associated chromosome rearrangement.

A translocation, t(17;22)(q22;q13), was identified in two cases of dermatofibrosarcoma protuberans (DP). They bring to four the number of DP cases characterized by an identical t(17;22)(q22;q13), which can be considered as a new tumor-associated chromosome rearrangement. To date, this translocation has been found only in DP and its juvenile form, giant-cell fibroblastoma. This finding has two major consequences. First, it casts light on the development and significance in DP of ring chromosomes which consistently harbor sequences derived from chromosomes 17 and 22. Second, the identification of this new chromosome marker, and eventually of the underlying molecular rearrangement, should help to classify DP, a soft-tissue tumor of still uncertain cell origin. In addition, it could be used to differentiate DP from truly benign or malignant entities, in order that this tumor of intermediate malignancy could be adequately managed.

Adolescent↗
PubMed1995

'Milia en plaque' in the supraclavicular area.

'Milia en plaque' is an unusual skin disease. Up to date only 6 cases have been reported, and all of them were located in the retro-auricular area. We report herein the first case of milia en plaque developed in the supraclavicular area. The absence of a known aetiologic factor in contrast to the previously published cases suggests that the present case belongs to the group of primary milia.

Adult↗
PubMed1995

[Cutaneous larva migrans, autochthonous in France. Apropos of a case].

INTRODUCTION: Cutaneous larva migrans is rarely contracted in temperate countries. CASE REPORT: When his house became flooded, he had to stand for a long period of time with mud up to the thigh. Some days later, he developed multiple erythematous, serpiginous pruritic tracts moving 1-2 cm per day over preexisting lesions of the right leg. Local and systemic treatment with thiabendazole led to rapid and definitive cure. DISCUSSION: Cutaneous larva migrans results from the migration of hookworm larvae in the dead-end human host. It is mainly an imported disease and native cases in Europe as reported here are rare. This case demonstrates that the conditions leading to the development of cutaneous larva migrans are rarely found simultaneously in temperate zones.

Adult↗
PubMed1994

Epidermolysis bullosa simplex with mottled pigmentation. Case report and review of the literature.

Epidermolysis bullosa simplex with mottled pigmentation (EBS-MP) is a rare genodermatosis of which only 4 pedigrees have so far been reported. We present herein a new family with EBS-MP comprising a peculiar punctate digital keratoderma. The propositus was an 18-year-old patient who had suffered since birth from posttraumatic bullae, followed by mottled pigmentation, plantar keratosis, nail dystrophy and peculiar punctate keratoses of the fingers. Histology, immunofluorescence and electron microscopy of a bullous lesion showed an intraepidermal cleavage and an increased number of melanosomes within basal keratinocytes, dermal macrophages and Schwann cells. The precise genetic defect responsible for EBS-MP is not known but could be due to two distinct, closely linked mutations. The nosologic relationship between EBS-MP and other forms of EBS as well as cases of hereditary bullous poikilodermic acrokeratosis is discussed.

Adolescent↗
PubMed1994

[Pachydermoperiostosis. An ultrastructural study].

BACKGROUND: Pachydermoperiostosis (PDP) is a rare genetically determined disease belonging to the group of hypertrophic osteoarthropathies. Its aetiopathogenesis remains unclear. Most hypotheses favour an exogenous stimulation of fibroblasts. METHODS: A clinically typical patient with PDP was studied by electron microscopy with particular reference to the dermis and its cellular constituents. Fibroblasts from involved skin were cultured and studied in comparison with control cells. RESULTS: Remarkable modifications of the structure of the dermis were observed, encompassing irregular caliber of collagen fibres, extracellular deposits of microfibrils and of amorphous granular substance corresponding to the Alcian blue positive deposits seen by conventional histochemistry. The in vitro growth of fibroblasts was normal. CONCLUSION: Authors reviewed aetiopathogenic hypotheses. Our data suggest a genetically determined alteration of extracellular matrix production by fibroblasts as a possible explanation for the development of PDP.

Adult↗
PubMed1994

[Xanthogranuloma and cutaneous mastocytosis in adults].

Xanthogranuloma (XG) is a rare disease in adults. The authors report a case of XG in an 20 years-old woman. This observation is particularly interesting: First by the pathology of the cutaneous lesion which showed a dermal infiltrate composed of histiocytes and a few foamy cells but without Touton giant cells. Second by is association with a mastocytosis which disappeared when XG appeared. These data led the authors to discuss the misleading histology pattern of XG and also the pathogenesis of XG. In our case, it would appeared as a benign reactive process against mastocytosis.

Adult↗
PubMed1994

[Darier-Ferrand dermatofibrosarcoma. Apropos of a case with frontal involvement].

One case of dermato-fibro-sarcoma protuberans is reported on a 36 years old man. It was an extensive forehead tumor which needed a radically surgical treatment. These excision called a reconstruction with cutaneous graft and scalp flaps. Three years after treatment, there was no evidence of recurrence or lymphadenopathy. The aesthetic results is good. The authors recalled with this observation the important points of this disease particularly characterized by slow invasive growth and local recurrence.

Adult↗
PubMed1994

Transient adult T-cell leukemia/lymphoma picture during varicella infection in an HTLV-1 carrier.

HTLV-1 (human T-lymphotropic virus type 1) is associated with tropical spastic paraparesis, adult T-cell lymphoma (ATL), and also with opportunistic infections. The risk for developing ATL in HTLV-1 healthy carriers is low, between 1 and 4%. Nothing is known about the events promoting the evolution from the healthy carrier state to symptomatic ATL. We describe the case of a 44-year-old French Caribbean man with a chronic and recurrent strongyloidiasis in which the occurrence of a hemorrhagic and necrotic varicella led to the discovery of an infection by HTLV-1 and an acute form of ATL. All hematological data were normal before the onset of varicella. ATL completely disappeared at the same time as the varicella healed. This leads us to hypothesize that acute infections such as the reactivation of varicella-zoster may act as a promoting factor for the development of ATL in healthy HTLV-1 carriers.

Adult↗