Twelve patients with 16 leg ulcers, existing for at least 3 months and not responsive to conventional inpatient therapy of at least 3 weeks, were treated with repeated applications of cultured allogenic keratinocyte sheets. A marked decrease in size was seen in all ulcers but 2. Complete closure of the ulcer was seen in 62% of the ulcers within 8 weeks. Healing was due to enhanced granulation and increased epithelialization, starting from the periphery of the wound. This edge effect suggests that the epidermal allografts act by stimulation of migration and/or multiplication of the acceptor’s keratinocytes, rather than by take of the allograft.
We studied the effect of 2 months of treatment with budesonide (BUD) (Pulmicort), an inhaled corticosteroid, on the bronchial hyperresponsiveness to house-dust mite antigen (BHR-HDM) and to histamine (BHR-H). We also investigated whether BUD started 20 to 24 h after the development of a late asthmatic reaction (LAR) would influence the antigen-induced increase in nonspecific bronchial hyperresponsiveness to BHR-H. Thirty-one children with mild asthma who were atopic to HDM were randomized double blind into two parallel groups. Fifteen patients inhaled 0.2 mg BUD three times a day. Sixteen inhaled placebo in a similar way. Treatment began 20 to 24 h after antigen exposure and continued for 2 months. BHR-H and BHR-HDM were measured prior to and at the end of treatment. BHR-H was also determined 3 days after each antigen provocation. In the children receiving BUD, mean BHR-H and mean BHR-HDM were decreased approximately twofold after 2 months. No increase in BHR-H was observed after 3 days in the BUD group, irrespective of whether a LAR occurred. In patients in whom BUD treatment was withheld after the second antigen provocation, the protective effect of BHR-H was abolished. We conclude that 2 months of treatment with an inhaled corticosteroid causes a decrease in BHR-H and BHR-HDM. When an inhaled corticosteroid is administered 20 to 24 h after antigen provocation, It may protect against the antigen-induced increase in BHR-H. After treatment is discontinued, the protective effect wears off rapidly.
We report a patient who developed a generalized dermatitis after a period of progressive local intolerance to continuous subcutaneous infusion of hydromorphone for cancer pain. Sensitization to hydromorphone was proved by a positive patch test. Infusions with an equianalgesic dose of diacetylmorphine were well tolerated, without local or systemic side-effects, and prolonged the duration of infusion sites.
The Shwachman syndrome comprises exocrine pancreatic insufficiency, growth retardation, and bone marrow hypoplasia resulting in neutropenia. Clinical, morphological, and ultrastructural studies, as well as hair analysis, were performed in a patient with Shwachman's syndrome and severe ichthyosis. Clinical findings were lamellar ichthyosiform desquamation on the extremities. The hair was scanty and short on the scalp, in the eyelashes, and in the eyebrows. The nails were hyperkeratotic. Morphologic findings were slight, regular acanthosis and severe diffuse hyperkeratosis with variable parakeratosis. The granular layer was thickened. The papillary dermis showed very slight perivascular lymphocyte infiltration. The most prominent ultrastructural finding was the presence of solitary or multiple droplets of varying size in the cytoplasm of the keratinocytes. Hair analysis revealed no abnormalities; the cystine concentration in hair specimens was normal.
Mosaic RASopathies are a molecularly heterogeneous group of (neuro)cutaneous syndromes with high phenotypical variability. Postzygotic variants in KRAS have been described in oculoectodermal syndrome (OES), encephalocraniocutaneous lipomatosis (ECCL) and epidermal nevus syndrome (ENS). This study confirms the continuum of mosaic neurocutaneous RASopathies showing codon 146 KRAS variants in an individual with OES and, for the first time, in an individual with (isolated) epidermal nevus. The presence of a nevus psiloliparus in individuals with OES indicates that this finding is not specific for ECCL and highlights the phenotypical overlap between ECCL and OES. The presence of the somatic KRAS variant in the nevus psiloliparus resolves the underlying molecular etiology of this fatty-tissue nevus. In addition, this finding refutes the theory of non-allelic twin-spotting as an underlying hypothesis to explain the concurrent presence of two different mosaicisms in one individual. The identification of codon 146 KRAS variants in isolated epidermal nevus introduces a new hot spot for this condition, which is useful for increasing molecular genetic testing using targeted gene sequencing panels.
A term female firstborn infant had unexplained nonimmune fetal hydrops and recurrent left chylothorax at 4 weeks of age. A few months before conception, her mother had had acute dystrophic nail changes and is being treated for recurrent sinusitis, bronchiectasis, and a deficiency of serum IgG2. We suggest that they both suffer from a dominantly inherited congenital lymphedema syndrome known as `yellow nail dystrophy.' Prenatal manifestation of this disorder has not been reported previously. The child's anthropometric and neurological development was normal at 1 year of age, whereas mild ankle edema and marbling of the skin of the limbs were salient clinical findings. Inherited lymphedema leading to nonimmune fetal hydrops also has been recognized in chromosomal disorders, Noonan's syndrome, multiple pterygium syndrome, pulmonary lymphangiectasis, and mixed-vessel lymphatic dysfunction. Indicators of parental lymphedema are not on record in those instances.
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