R. Ruiz‐Maldonado

Acute disseminated epidermal necrosis types 1, 2, and 3: Study of sixty cases

Sixty patients with acute disseminated epidermal necrosis (ADEN) were hospitalized and carefully studied. They included thirty-nine patients with drug-associated Stevens-Johnson syndrome, five patients with drug-associated Lyells syndrome, and sixteen patients with transitional ADEN. On the basis of growing evidence of an association between erythema multiforme major and drugs and between Lyells syndrome and drugs, and because of the existence of transitional cases, a unitary hypothesis for this group of cases is proposed. Considering the lack of precise definitions and the confusing current terminology, we define and propose the following terms: ADEN type 1 for drug-associated Stevens-Johnson syndrome, ADEN type 2 for drug-associated transitional cases, and ADEN type 3 for drug-associated toxic epidermal necrolysis, or Lyells syndrome. The most frequent underlying diseases in our patients were seizures, and the most frequently suspected cause of ADEN was the use of anticonvulsants. All our patients were treated with supportive therapy; none received corticosteroids. The general mortality rate was 15%. The recognition of ADEN type 2 (transitional) has important prognostic and therapeutic implications.

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma

Hydroa vacciniforme-like lymphoma (HVLL) is an Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorder of childhood that occurs mainly in Central and South America and Asia. We present the clinicopathological features of 20 Mexican children with HVLL with a median age of 8 years at diagnosis (range, 1-15). All patients presented with skin lesions involving sun-exposed areas, but not exclusively. Fever, lymphadenopathy, and hepatosplenomegaly were often observed. Most patients were treated with immunomodulators and/or immunosuppressive agents, resulting in temporary remission. For 13 patients follow-up was available for a median of 3 years (range, 1 month-13 years). Three patients with long follow-up (9-13 years) are alive with disease. Four patients died, 2 after developing systemic lymphoma. Histologically, the skin showed a predominantly angiocentric and periadnexal Epstein-Barr early RNA+ lymphoid infiltrate with variable atypia and subcutaneous involvement. Fifteen patients showed a T-cell phenotype (12, αβ; 2, γδ; 1, silent phenotype) and monoclonal T-cell receptor-γ rearrangements, whereas 6 exhibited a natural killer (NK)-cell phenotype. Four patients had hypersensitivity to mosquito bites. One patient showed both phenotypes. HVLL is an EBV-associated lymphoproliferative disorder of αβ-, γδ-, or NK-cell phenotype with a broad clinical spectrum, usually prolonged clinical course, and risk for progression to systemic disease.

Treatment of 18 children with scabies or cutaneous larva migrans using ivermectin

Summary In addition to onchocerciasis and other filarial diseases, ivermectin has been used for the treatment of scabies, head lice, larva migrans and gnathostomiasis. However, there is concern regarding the safety of its use in children under 5u2003years of age or weighing less than 15u2003kg. We present our experience in 18 children (aged 14u2003months to 17u2003years), with scabies or cutaneous larva migrans successfully treated with ivermectin. They included four cases of crusted scabies associated with immunosuppression and seven cases of common scabies four of whom had associated clinical mental retardation, immunosuppression or hypomobility. A further seven patients had cutaneous larva migrans. Fifteen patients were cured with a single dose of ivermectin, and three patients with crusted scabies required a second dose. None of our patients suffered significant adverse effects. We believe that ivermectin is a safe and effective alternative treatment of cutaneous parasitosis in children.

GORHAM'S SYNDROME

A 6-year-old boy was referred to the National Institute of Pediatrics, Mexico City, in 1987 with an extensive, brownred discoloration of the skin that involved his right upper limb. The lesion had appeared at age 3 years. The mother first noted a spot over the wrist that rapidly progressed in the following 6 months to reach the present size. Since then, she also had noticed a progressive thinness of the same limb with inability to move it. At age 5 years the boy fell down and fractured his right humerus. Aside from immobilizing the fracture, he had not received any treatment. The past and families histories were noncontributory. General examination was entirely negative except for the presence of a brown-red pigmentation over the right upper limb extending to the axilla, with only the shoulder and dorsum of the hand unaffected (Fig. 1). There was also hypertrichosis and increased local sweating. The extremity was strikingly hypotrophic with the patient having difficulties in moving it. Its strength was diminished. Routine laboratory tests were normal. A cutaneous biopsy showed an intradermal and subcutaneous proliferation of vessels lined by flattened endothelium. Some vessels were small, but others were dilated and large, almost sinusoidal. Most of them contained some red blood cells. A fibrous stroma of various thicknesses surrounded these vascular spaces. Moderate stromal mononuclear inflammation was also observed (Fig. 2). Radiographs of the right upper extremity revealed radiolucent foci resembling osteoporosis in the three long bones, which had suffered concentric reduction resulting in a striking tapering of them. An old fracture of the humerus was also evident. There was marked atrophy of musculature and soft tissues (Fig. 3). Radiographs of the chest were normal except for the presence of osteoporosis in the right scapula, tapering of the right clavicle, and partial erosion of the first and second right ribs. An x-ray series did not show anomalies in any other bone. Bone biopsy was not done, but an arteriogram showed abnormally dilated vascular spaces arising from the branches of the right humeral, radial, and ulnar arteries. An abdominal scan gave normal results. The patient received treatment with prednisone for 3 months at a daily dose of 2 mg/kg of body weight and radi-

Human gnathostomiasis (nodular migratory eosonophilic panniculitis).

A 42‐year‐old woman was first seen in January 1996 with a 7‐cm diameter erythematous–edematous, pruritic plaque on her right shoulder. The lesion had first appeared 3 weeks previously on the right side of the thorax and migrated to the shoulder. The patient had eaten raw fish (sashimi) in Japanese restaurants two or three times a year during the past few years.

Nodular scabies mimicking urticaria pigmentosa in an infant

autosomal recessive disorder that manifests as an acral and circumorificial dermatitis and is associated with low zinc levels. Mutations in the SLC39A4 gene located on chromosome 8q24.3 have recently been found to be responsible for this disease. Following informed consent, genomic DNA was extracted from peripheral blood samples taken from our patient and his parents using a standard cold water lysis method. PCR amplification of the SLC39A4 gene was performed using 12 pairs of primers as described previously. However, higher annealing temperatures (compared to published data) were required for exon 2 and exon 10, at 60 C and 65 C, respectively. Automated sequencing revealed a single nucleotide substitution (751C fi A) in exon 4, resulting in an amino acid change of arginine to tryptophan at residue 251. The mutation R251W had been reported previously as a possible mutation with uncertain implications. However, this missense change has also been considered to be a nonpathogenic polymorphism because the recorded cDNA sequence (NM130849) and genomic DNA sequence (AF205589) show contradictory C and A at nucleotide position 751, respectively. In our family, the patient and both parents were all homozygous for R251W. This finding suggests that R251W is indeed a polymorphism and as such has implications for the interpretation of other published mutation reports in acrodermatitis enteropathica. Although the patient had an acral and periorificial dermatitis that responded to zinc supplements, his parents remain unaffected. The main factors responsible for low zinc levels in this infant are almost certainly prematurity and inadequate dietary zinc. Premature infants are susceptible to hypozincaemia as the amount of zinc in the fetus increases threeto fourfold in the last trimester. In addition, low maternal breast milk zinc levels are likely to have been a major contributory factor (lactogenic acrodermatitis). We have previously reported two infants born at term, with low zinc levels due to low maternal breast milk zinc ) a deficiency or functional abnormality of a carrier molecule responsible for transport of zinc in the milk was postulated. In this case, maternal breast milk zinc was not measured, as breast milk zinc falls exponentially during the course of lactation, such that by week 15, a low level may not be reliably detectable. We conclude that this infant had clinical features consistent with acrodermatitis enteropathica with no pathogenic SLC39A4 gene mutation. This child almost certainly had infantile acquired zinc deficiency secondary to prematurity and low maternal breast milk zinc and is therefore unlikely to require long-term zinc supplementation.

Interferon alpha‐2B in juvenile hyaline fibromatosis

firmed the role of fexofenadine in the pustular aggravation. The pathomechanism of this phenomenon was apparently unrelated to H1-receptor blockage, as other agents of the same class were well tolerated in this case. Fexofenadine is a derivative of the H1-receptor blocker terfenadine, which was withdrawn from the market due to its arrhythmogenic potential. Interestingly, two cases of terfenadine-induced exacerbation of psoriasis were reported before its discontinuation. However, to our knowledge, there are to date no published reports of aggravation of psoriasis due to fexofenadine. Considering the widespread use of nonsedating H1-receptor antagonists such as fexofenadine and the high prevalence of psoriasis (1% to 2% of the general population), it would appear that this adverse effect is quite rare. Physicians should be aware that fexofenadine, like its predecessor terfenadine, has the ability to cause potentially serious exacerbations of psoriasis.

Dermatitis artefacta in a teenager after awareness of his HIV-positive diagnosis.

known as bee’s glue. It is present in topical medications and in popular remedies. It accounts for 5% of sensitized cases in CVI, and 2.5% in anogenital disease. In this patient contact sensitization probably occurred due to previous long-term topical treatment for varicose eczema. Contact allergy to propolis accounted for the intractability of vulval eczema when being treated with steroid ointments containing this compound. Vulval eczema can be a difficult condition to treat. This case illustrates the importance of patch testing and in considering prescribed medicaments as a possible source of allergen. The use of pimecrolimus cream has not, as far as we are aware, been previously described in the treatment vulval eczema and may prove useful for these cases in the future.