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Biomedical subjects

J J Bonerandi

Publications and source records attributed to J J Bonerandi.

At least 91 records · Page 5Linked to original sources
PubMed1988

Disseminated pustular dermatosis in polycythemia vera. Relationship with neutrophilic dermatosis of myeloproliferative disorders: study of neutrophil function.

An unusual case of disseminated pustular eruption associated with polycythemia vera is described. This eruption can be included in the group of neutrophilic dermatoses of myeloproliferative disorders (pyoderma gangrenosum, Sweet's syndrome). A defect in superoxide anion (O2-) generation is detected. The fact that both the clinical condition and the neutrophil function improved with the use of dapsone suggests that the O2- generation defect may play a role in the pathogenesis of the eruption. From an evolutionary standpoint, the occurrence of a neutrophilic dermatosis in polycythemia vera could be an unfavorable sign of preleukemia.

Aged↗
PubMed1988

Dysplastic naevus in non-familial melanoma. A clinicopathological study of 101 cases.

In 101 patients with non-familial cutaneous melanoma (CM), melanocytic naevi were counted and classified according to clinical criteria. Only 8% of the patients had very atypical naevi. These atypical naevi were few in number and only one patient exhibited dysplastic naevus syndrome. An histological study was undertaken on the hypothesis that, in a given individual, if the most clinically atypical naevus is not histologically dysplastic it is unlikely that any of the others are. The most clinically atypical naevus in each patient was biopsied. Estimated in this way the prevalence of dysplastic naevi in patients with non-familial CM was only 18%. Comparison of patients with and without dysplastic naevi did not suggest that they constituted two different subsets. An attempt to correlate clinical diagnosis and histological features in this group of patients showed that the diagnosis of dysplastic naevi on the basis of clinical criteria alone is difficult and not reliable.

Adult↗
PubMed1988

Non lymphoblastic T cell lymphomas with prevalent skin involvement different from mycosis fungoides or Sézary's syndrome. A retrospective study of 6 cases in Europe.

A retrospective study of 23 cases of nonlymphoblastic T cell lymphomas with no features of either mycosis fungoides (MF) or Sézary syndrome (SS) was carried out. 6 patients exhibited cutaneous (or mucous) lesions and are presented herein. There was no detectable visceral or node involvement except in 1 patient. 3 cases were immunologically proven to be of peripheral T cell origin. Except T cell lymphomas in Japan and in the Caribbean area, the frequency of prevalent skin involvement is reportedly very low in non-MF/SS non lymphoblastic T cell lymphomas. However, it is likely that this frequency has been underestimated in Western countries. The histologic features of our cases run the heterogeneous spectrum of recently described node-based forms of peripheral T cell lymphomas: 'angioimmunoblastic lymphadenopathy with dysproteinemia'-like lymphoma, large T cell lymphoma, multilobulated cell lymphoma, T zone lymphoma. The histologic pattern of skin infiltration was different from that of MF/SS. One case was initially suggestive of a pseudolymphoma and had a prolonged evolution but the other patients suffered from rapid extracutaneous involvement and fatal evolution with resistance to treatment. Cutaneous plasmacytomas were seen in 1 case. Hence, certain cutaneous T cell lymphomas exhibit histologic, immunological and developmental features similar to those of node-based peripheral T cell lymphomas but different from those of MF/SS.

Aged↗
PubMed1988

[Cutaneous signs of Noonan's syndrome. Apropos of a case with ulerythema ophyogenes, disseminated pilar and sudoral keratosis and progressive alopecia].

A case of typical Noonan syndrome (NS) with eye abnormalities, pterygium colli, cryptorchid testes, lymphoedema and asymmetrical cardiac septal hypertrophy is reported in a 8-month old infant. This case was particularly interesting since it included skin manifestations which enabled an early diagnosis to be made. Ulerythema ophryogenes has already been proposed as a cutaneous marker of NS, but the keratinization disorders in our patient also included disseminated keratosis of both hair follicles and sweat glands orifices. Abnormally brittle short curly hair has already been reported, but our patient exhibited progressive alopecia which is very rare in NS. Biochemical hair analysis did not show any abnormalities of aminoacids. All these features were suggestive of keratosis follicularis spinulosa decalvans. It therefore seems very likely that NS is associated with keratinization disorders but ulerythema ophryogenes might only be the limited form of these disorders. The other skin manifestations of NS are reviewed. Since the patient had 4 "café au lait" spots, the relation of NS with Von Recklinghausen syndrome, and neurofibromatosis-Noonan syndrome is discussed. Watson's Leopard and cardio-facial syndromes overlap with, and may represent subsets of NS.

Abnormalities, Multiple↗
PubMed1987

Keratitis, ichthyosis, and deafness (KID) syndrome. Vertical transmission and death from multiple squamous cell carcinomas.

A father and daughter had typical clinical features of the keratitis, ichthyosis, and deafness (KID) syndrome, as described by Skinner et al in 1981. To our knowledge, ours is the first observation of a vertical transmission of this syndrome. The mechanism of inheritance is uncertain. These two patients as well as the 26 previously described exhibited a typical hyperkeratotic eruption, which should not be confused with ichthyosis. The characteristic features are diffuse hyperkeratosis, keratotic plaques, reticulated hyperkeratosis on the face, peribuccal grooves, and heavy-grain leatherlike keratoderma. The occurrence of multiple squamous cell carcinomas underlines the seriousness of this congenital ectodermal defect. Etretinate can relieve the lesions without actually improving the chances of survival.

Adult↗
PubMed1986

Cutaneous manifestations associated with the presence of the lupus anticoagulant. A report of two cases and a review of the literature.

Two patients with the lupus anticoagulant exhibited unusual cutaneous manifestations. They both fulfilled four criteria for systemic lupus erythematosus and had experienced deep venous thrombosis. The first patient suffered from a leg ulcer that resembled a pyoderma gangrenosum. The second patient presented erythematous and purplish macules on the fingertips. The histologic studies showed only microthrombosis in the dermal vessels without vasculitis, although such lesions in systemic lupus erythematosus are usually attributed to vasculitis. The association of these cutaneous lesions with lupus anticoagulant has never been reported. It is likely that this association is not fortuitous. After a review of the literature, it seems possible to individualize a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies. Thrombosis, spontaneous abortions, neurologic manifestations, pulmonary hypertension, positive results of a Coombs' test, and thrombocytopenia can be included in this syndrome, which overlaps with systemic lupus erythematosus. Certain cutaneous symptoms are associated with the presence of lupus anticoagulant or other antiphospholipid antibodies: leg ulcers, distal cutaneous ischemia, widespread cutaneous necrosis, and livedo. They can be considered as the dermatologic manifestations of this syndrome.

Adult↗