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Biomedical subjects

Alain Claudy

Publications and source records attributed to Alain Claudy.

25 records · Page 2Linked to original sources

No recurrence of posttransplantation Kaposi's sarcoma three years after renal retransplantation.

Kaposi's sarcoma (KS) develops in 0.5-5% of organ transplant patients; it usually regresses upon treatment reduction, but this may result in graft loss necessitating return to dialysis and/or retransplantation. Until now posttransplantation KS is considered to recur upon reintroduction of immunosuppressive treatment, a fact that has limited retransplantation of patients with previous KS. We report a patient with posttransplantation KS who received a second renal transplantation after having been off immunosuppressive treatment for 10 years, in whom KS has not recurred more than 3 years after retransplantation. This unique observation suggests that retransplantation of patients with previous posttransplantation KS is possible.

Adult↗

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy): a neurovascular disease diagnosed by ultrastructural examination of the skin.

BACKGROUND: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a recently recognized neurovascular disease due to mutations of the Notch3 gene, manifesting with strokes or stroke-like episodes, major psychiatric symptoms and dementia. The diagnosis can be confirmed either by molecular analysis or by ultrastructural examination of the brain or more simply the skin. METHODS: The skin of a patient with a suspected diagnosis of CADASIL was studied by electron microscopy. RESULTS: Characteristic granular osmiophilic material within the basement membrane surrounding pericytes and smooth muscle cells of small and medium-sized vessels of the skin were found, confirming the diagnosis of CADASIL. CONCLUSIONS: CADASIL is an additional example of a neurologic disease that can be diagnosed thanks to electron microscopic examination of the skin. Dermatopathologists should be aware of these ultrastructural findings, all the more so since the disease could be more common than originally thought.

Basement Membrane↗

Melanotrichoblastoma: immunohistochemical study of a variant of pigmented trichoblastoma.

Trichoblastomas (TBs) are benign skin tumors recapitulating the differentiation of hair follicles. Several pathologic variants have been described, including the rare pigmented TB. We report a deeply pigmented nodular tumor excised on the scalp of a 32-year-old African woman, which was clinically suggestive of blue nevus or melanoma. Histologically, the tumor presented features of TB, remarkable by virtue of heavy melanin deposits found within and around tumor nests. By immunohistochemistry, abundant dendritic melanocytes with features of hair follicle melanocytes (expression of S100 protein, tyrosinase, and, most importantly, gp100/HMB-45) were found within the tumor masses. By analogy with melanoacanthoma, a tumor consisting of a combined proliferation of epidermal keratinocytes and melanocytes, we called this tumor "melanotrichoblastoma" and view it as a peculiar variant of (pigmented) TB.

Adult↗

Melanocyte stimulation in focal dermal hypoplasia with unusual pigmented skin lesions: a histologic and immunohistochemical study.

Focal dermal hypoplasia (FDH) or Goltz syndrome is a rare genodermatosis transmitted in a dominant, X-linked mode. It is characterized clinically by atrophic skin lesions, multiple mucocutaneous papillomas, hyperpigmented linear skin lesions, and several skeletal and visceral anomalies. We followed over several years a female patient with FDH, who had the characteristic atrophic cutaneous lesions and periorificial papillomas, who developed at the periphery of atrophic lesions peculiar lentigo-like pigmented macules. Immunohistologically, increased melanin deposits within the epidermis and the dermis were seen, produced by stimulated epidermal melanocytes expressing the HMB-45 antigen. These findings further support the contention that cutaneous lesions of FDH may be progressive, and provide a physiopathologic basis for understanding the hyperpigmented lesions of FDH.

Adolescent↗

Clinical quiz. Rubinstein-Taybi syndrome (synonyms: broad thumbs and great toes, characteristic facies, and mental retardation -- broad thumb-hallux syndrome).

A 28-year-old mentally retarded, institutionalised woman was referred to us for evaluation of multiple plantar warts and ingrown nails of both great toes. The patient was born to unrelated parents of North African origin and had one brother and three half-brothers, all of whom were healthy. Physical examination revealed short stature, slight obesity, facial abnormalities (Fig. 1), short and broad thumbs and big toes (Figs. 2 and 3). A keloid was found on the right forearm, that had developed after surgical correction of a fracture (Fig. 4). Ill-defined hyperpigmented macules were observed on the trunk. The patient also presented pruritic eczematous lesions of the limbs and the back that had been present for some years and were recurrent despite treatment with emollients and local steroids. Androgenetic-type alopecia of moderate severity was seen on the vertex of the scalp. Past medical history included polydactylism of the feet and clinodactyly of the thumbs (both corrected surgically), respiratory tract infections, Wolf-Parkinson-White syndrome and refractive errors necessitating glasses.

Adult↗

Pathological score for the evaluation of allograft rejection in human hand (composite tissue) allotransplantation.

The experience obtained from the human hand allografts (HHA) performed to date suggests that the skin is a priviledged target of allograft rejection in this setting. The aim of this study was to establish a pathological score for assessing the severity of HHA rejection. The pathological slides of 89 skin biopsies obtained from the allografted limbs of six HHA recipients from day 0 to 5 years post-graft were retrospectively examined. According to the severity of the pathological changes observed, the following grades of rejection are proposed: 0: no rejection, I: mild rejection, II: moderate rejection, III: severe rejection, IV: very severe rejection. This grading system can be used as a basis for monitoring allograft rejection and for assessing the effects of the immunosuppressive treatment aiming at reversing HHA rejection; it can also be used for monitoring rejection of other skin-containing CTA.

Adult↗