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Biomedical subjects

J J Grob

Publications and source records attributed to J J Grob.

119 records · Page 7Linked to original sources
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↗
PubMed1986

[Antiphospholipid antibodies: new data in the pathogenesis of collagenosis].

Antiphospholipid antibodies (APAB) were first detected as a result of the disturbances they cause in routine biological tests. These effects include lupus anticoagulant (LA) in APTT test and false serological reactions for syphilis in VDRL test. With more sensitive techniques using purified phospholipid antibodies (ELISA, RIA) antibodies directed specifically against cardiolipidin or other phospholipids can be assayed. Beside being responsible for positivity of the VDRL test in the context of syphilis, APAB (false serological reactions for syphilis, LA, anticardiolipin antibodies) have also been detected in systemic lupus erythematosus (SLE) and lupus-like syndromes, after intake of certain drugs, and, more rarely, in a number of diseases (table I). The specificity of APAB detected in syphilis (antiphosphatidylcholine) is different from that of APAB detected in SLE (antiphosphatidylserine and anticardiolipin). A given APAB can react with several phospholipids, but such crossreactions do not occur haphazardly with all phospholipids. Crossreactions are observed between APAB and polynucleotides on the one hand, and between antinuclear antibodies and phospholipids on the other. This pattern has led to speculate that APAB are directly involved in the pathogenesis of SLE; however since these crossreactions are not extensive, such speculation is controversial. The presence of APAB in the form of LA, false serological reactions for syphilis or anticardiolipin antibodies is associated with a number of problems including recurrent thrombosis, repeated abortions, thrombocytopenia and positive Coombs' tests or, more rarely, pulmonary hypertension, migraine, epilepsy, chorea, and transverse myelitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies↗
PubMedDate not supplied

[Cutaneous adverse effects during selective serotonin reuptake inhibitors therapy: 2 cases].

BACKGROUND: Selective serotonin reuptake inhibitors are widely used in the treatment of depressive and obsessive-compulsive disorders because of their low-frequency adverse effects. We report two cases of cutaneous adverse effects during selective serotonin reuptake inhibitors therapy. CASE REPORTS: A man who complained of chronic anal idiopathic pruritus was treated with citalopram (Seropram(R)) 10 mg b.i.d. Six days after the beginning of the antidepressive treatment, he developed an extensive papular and purpuric erythema with keratinocytes necrosis and dermal leucocytoclastic vasculitis. Cutaneous lesions remained for several weeks, as the half-life of citalopram is very long (33 to 36 hours) but did not relapse. A women developed painful papular and purpuric erythema mainly located in sun-exposed sites, during therapy with paroxetine (Deroxat(R)) 20 mg b.i.d., which had been introduced one month before to treat depression. Cutaneous lesions healed spontaneously in 2 weeks after the discontinuation of paroxetine and with sun avoidance and didn't relapse. DISCUSSION: Adverse cutaneous effects of selective serotonin reuptake inhibitors are rare but the knowledge of these reactions is important because toxic epidermal necrolysis and Stevens-Johnson syndrome had been reported during fluoxetine (Prozac(R)) and fluvoxamine (Floxyfral(R)) treatment. Different serotonin uptake blockers could be involved in the same allergic reaction, suggesting cross reactivity, although these drugs have different chemistry structures. It is advisable to substitute after an adverse effect a medication from one of the other classes of antidepressants.

Aged↗
PubMedDate not supplied

[Extracorporeal photochemotherapy in therapy-refractory subacute lupus].

BACKGROUND: Extracorporeal photopheresis is a leukapheresis therapy that uses psoralen and ultraviolet A irradiation. We report the case of a woman with a refractory sub acute lupus which dramatically but transitionally responded to extracorporeal photopheresis. CASE REPORT: This women, born in 1960, developed erythematous and squamous patches located on face and neckline, associated with hyperpigmented and atrophic lesions on the arms and shoulders. Investigations confirmed the diagnosis of subacute lupus without systemic disease. All lesions progressed, despite all conventional therapies leading to major aesthetic prejudice. Extracorporeal photopheresis was initiated, and after two months, all lesions, including atrophic and healing lesions had regressed, but laboratory abnormalities did not change. Extracorporeal photopheresis was well tolerated. However, treatment was discontinued nine months later, since the cutaneous lesions relapsed. COMMENTS: Extracorporeal photopheresis could be efficient in the treatment of cutaneous autoimmune diseases through several immunomodulatory mechanisms. Extracorporeal photopheresis is a potent alternative agent in the therapy of refractory dermatological diseases

Adult↗