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A 24-year-old female patient with congenital ichthyosiform erythroderma, deafness and vascularizing keratitis yet also examining normal sweating and tear production when tested is reported. This unique finding indicates that a certain subgroup of patients with this generalized ectodermal disturbance may benefit from treatment with the aromatic retinoids.
A presentation of a 3-year-old girl with a congenital hemidysplasia of the left side together with ichtyosiform erythroderma, the so-called CHILD-syndrome. A short review of the syndrome is given.
Erythroderma and exfoliative dermatitis developed in a 17-year-old boy following therapy with phenytoin sodium. Immunologic studies performed early in the course of the dermatitis disclosed panhypogammaglobulinemia and a marked increase in T lymphocytes that responded poorly in vitro to T-cell mitogens. After therapy with prednisone, the dermatitis improved somewhat, and the patient's lymphocytes proliferated in vitro when exposed to phenytoin. We speculate that our patient's acute hypersensitivity reaction may have been mediated by an excessive number of phenytoin-sensitized suppressor-cytotoxic T lymphocytes and may represent a disorder of immunoregulatory T cells.
This is a description of a case in a man who developed non-Hodgkin lymphoma of small cleaved cells preceded by erythroderma with leukemoid reaction. Non- caseous granulomas with Langhans' cells were found in the skin biopsy. The etiologic agent was not identified until the neoplasia that killed him was demonstrated. Autopsy study showed lymphomatous infiltration in all ganglionaries groups, the skin, lungs, kidney, liver, spleen, adrenal glands and bone marrow. One of the interesting aspects of the case in the tissue reaction to the neoplastic process, a fact already described but rarely observed.
The congenital ichthyosiform erythrodermas have become readily treatable with an aromatic retinoid. Satisfactory initial results over the first few months of treatment were achieved when the new preparation was given to Transvaal patients known to have these disorders and hitherto treated in vain. The pharmacology of the drug and therapeutic experiences in 6 cases are described.
A 23-year-old woman, who had nonbullous congenital ichthyosiform erythroderma since her childhood, was diagnosed as nephrotic syndrome caused by systemic lupus erythematosus (SLE). She was pregnant but experienced fetal loss at the age of 25. Although 10 mg/day of oral prednisolone was administered, low levels of serum complement, proteinuria, thrombocytopenia (6.0 x 10(4)/mm3) and biological false positive for STS continued. When she was 27 years old, right hemichorea developed. She was admitted to our hospital at the age of 28 because of low levels of serum complement, high titers of anti ds-DNA antibody, profuse proteinuria, gingival bleeding and thrombocytopenia (1.5 x 10(4)/mm3). The nephrotic syndrome gradually improved after 1 g/day of methylprednisolone for 2 days and the oral prednisolone dosage was then increased up to 40 mg/day, and was tapered to 10 mg/day. Epileptic attack (minor seizure) occurred at the age of 29. Continuous low levels of serum complement and high titers of anti ds-DNA antibody were improved by adding 50 mg/day of cyclophosphamide. However, high levels of beta 2 GPI dependent anticardiolipin antibody and lupus anticoagulant activity were observed throughout the study. Our patient was a very rare case of congenital ichthyosis with typical antiphospholipid antibody syndrome and SLE. A few cases of acquired ichthyosis associated with SLE has been reported, and ichthyosis developed only in active stage of SLE. However, our patient's ichthyosiform lesions were not changed throughout the course.
We present two cases of cutaneous erythroderma induced by carbamazepine therapy, both cases with just cutaneous affection and only one suspicious drug. A cutaneous patch study, with standard battery from the Spanish Contact Dermatitis Research Group, vaseline with carbamazepine at 10% and lectures at 48 and 96 hours, was conducted. The result was positive for carbamazepine and negative for all other patches in both cases. All controls were negative. This is a very simple test, easy to perform, specific and with low side effects, allowing the diagnosis of this pathology.
A case of peripheral neuropathy following treatment with piroxicam is reported. The patient developed muscle pain and asymmetrical sensory distal neuropathy of the lower limbs a few months after the onset of the treatment. Piroxicam was withdrawn and full recovery was achieved within one year. The simultaneous appearance in the same patient of erythroderma with microvasculitis on nerve and skin biopsies suggests a common type III hypersensitivity mechanism in the pathogenesis of these two side-effects of piroxicam.
Chronic generalized erythroderma (CGE) is a persistent, cutaneous, inflammatory response triggered by some underlying benign or malignant cause. Understanding the definition, pathogenesis, incidence, cutaneous and systemic manifestations, etiology, diagnosis, prognosis, and treatment of CGE with particular emphasis on nursing implications will help dermatology nurses care for these patients.
Over the past 8 years, we have followed a child born as a harlequin baby, who survived due to treatment with retinoids. His condition evolved clinically towards the erythrodermic form of lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma, NBCIE). According to ultrastructural and biochemical criteria, our patient originally presented with type II harlequin ichthyosis. Investigations showed an abnormal keratinosome structure and extrusion, a keratin pattern characteristic for epidermal hyperproliferation, and an absence of conversion of profilaggrin to filaggrin. Persisting keratinocyte hyperproliferation, associated with the presence of a dermal infiltrate, is in agreement with the present clinical picture of severe NBCIE. However, abnormal lamellar body production and defective filaggrin processing, which is not one of the diagnostic criteria of NBCIE, persist in the patient's skin. Further studies of the epidermal lipid composition, and of possible mutations of the keratinocyte transglutaminase gene performed on epidermal cell cultures of harlequin ichthyosis, will be necessary before type II harlequin ichthyosis can be accepted as an extremely severe form of NBCIE.
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