The British journal of dermatology
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Case Reports
Childhood sclerodermatomyositis: report of a case with the anti-PM/Scl antibody and mechanic's hands.
We report a boy with overlap manifestations of systemic sclerosis and dermatomyositis (sclerodermatomyositis) whose disease showed a changing clinical pattern, and who had mechanic's hands, which are a cutaneous marker of myositis. Serological studies revealed antinuclear antibodies with a homogeneous nucleolar pattern. The anti-PM-Scl antibody was demonstrated by immunoblotting. ⋯ After a follow-up period of 11 years, no progression to severe systemic involvement was detected, and aggressive treatment was not administered. The recognition of subsets of patients with homogeneous clinical features and serological markers should permit the recognition of separate conditions among overlap syndromes. This would have prognostic and therapeutic implications.
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Two hundred and twenty-one amateur tattoos and 27 professional tattoos were treated with a Nd/YAG laser (lambda 1064 nm and 532 nm). The response was expressed as the percentage area cleared of tattoo. Seventy-nine per cent of amateur black tattoos were > or = 75% clear after one to five treatments (mean 2) at 1064 nm. ⋯ Red tattoos responded well to 532 nm and were > or = 75% clear after one to five treatments. Yellow, orange, blue and green tattoos were resistant to treatment. Side-effects included minor scarring in 1.2% of tattoos and transient pigmentary changes in 77% of patients.
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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. ⋯ 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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Case Reports
Autoantibodies to bullous pemphigoid and epidermolysis bullosa acquisita antigens in an infant.
We describe a 1-year-old boy with multiple tense blisters on the skin, who showed circulating autoantibodies directed to both bullous pemphigoid and epidermolysis bullosa acquisita antigens. The patient's serum IgG antibodies bound to the 290-kDa epidermolysis bullosa acquisita antigen with immunoblot analysis of human dermal extracts. Immunoblot analysis also demonstrated that the patient's serum autoantibodies were reactive with recombinant NC16a domain of the 180-kDa bullous pemphigoid antigen. This study confirmed the presence of circulating autoantibodies directed to both bullous pemphigoid antigen and epidermolysis bullosa acquisita antigen.
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The clinical and histopathological classification of erythema exudativum multiforme major (EEMM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are difficult, due to the lack of clear-cut criteria. Based on a new clinical classification, 149 of 219 (68%) histopathological specimens, from a total of 534 patients with EEMM, SJS and TEN, have been reviewed. A comparison was made with the clinical picture, and any past history of infection or drug intake. ⋯ Eosinophils were less common in the patients with the most severe forms of TEN, in whom there was detachment of more than 30% of the skin surface area. No differences in the history for drug intake, or for infection with Mycoplasma pneumoniae, herpes simplex and other organisms, could be detected between patients with or without eosinophils in their skin sections. This dermatopathological study of patients with EEMM, SJS and TEN indicates that the epidermal type of erythema multiforme is the pathological correlate for these diseases.