Yu-Hung Wu

Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child

Hydroa vacciniforme (HV) is a chronic photosensitivity disorder induced by ultraviolet radiation. Hydroa vacciniforme‐like lymphoma is a rare cutaneous T‐cell lymphoma occurring mainly in childhood. Recent studies have demonstrated an association between chronic latent Epstein‐Barr virus (EBV) infection and both the benign skin disorder and the lymphoma. The authors report a 6‐year‐old boy with chronic EBV infection, HV‐like skin eruptions, and chronic hepatitis. Histopathologic examination of a skin biopsy specimen demonstrated epidermal ballooning degeneration and dense superficial and deep perivascular and periappendageal lymphoid cell infiltrates extending to the fat lobules. Some blood vessels in the deep plexus were infiltrated by predominantly CD4+ and TIA‐1+ cytotoxic T cells. The EBV genomes were found within tissue from three skin biopsies and peripheral blood cells. Monoclonal T‐cell receptor gene rearrangement was present in skin biopsy specimens. Although no lymphoma has been found during 2 years of follow‐up treatment, the possibility of lymphoma developing out of the current smoldering stage is of concern. The clinical manifestations of lymphoproliferative disorder and chronic active EBV infection are discussed.

Netherton syndrome: mutation analysis of two Taiwanese families

Netherton syndrome (NS) is a severe autosomal recessive skin disorder characterized by congenital ichthyosiform erythroderma, hair shaft abnormalities, and atopic diathesis. Recently, pathogenic mutations were identified in serine protease inhibitor Kazal-type 5 (SPINK5), the gene that encodes lympho-epithelial Kazal-type related inhibitor (LEKTI), a type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. In the present report, we describe the mutation analysis of two Taiwanese patients with NS. Patient 1 has heterozygous mutations; the maternal allele has novel T808I (C–T transition in codon 808) and the paternal allele has recurrent R790X (C–T transition in codon 790). Patient 2 is homozygous for a novel polymorphism R267Q (G–A transition in codon 267). The change was not detected in the patient’s father. Haplotype analysis revealed that the patient was homozygous for the 5 single nucleotide polymorphisms in the genomic sequence of SPINK5 as well as the flanking (GT)17 and D5S413, in addition to the discrepancy of R267Q. Nevertheless real-time quantitative PCR analysis revealed no microdeletion in the genomic sequence of SPINK5. Thus uniparental disomy of maternal SPINK5 allele was indicated.

Isotopic response of fungal granuloma following facial herpes zoster infections – report of three cases

Background  An isotopic response is the occurrence of a new skin disease at the site of another unrelated, healed skin disorder.

NEMO Gene Mutations in Chinese Patients with Incontinentia Pigmenti

BACKGROUND/PURPOSE Incontinentia pigmenti is a rare, X-linked, dominant genodermatosis affecting skin, teeth, eyes, and central nervous system. Symptoms are associated with mutations in the nuclear factor-kappa B essential modulator (NEMO) gene on chromosome Xq28. Here, a subpopulation of Chinese patients with incontinentia pigmenti were examined to investigate the frequency and pattern of NEMO mutations, and to analyze their clinical features. METHODS From January 1996 to August 2006, 52 participants (21 probands and 31 family members) were screened for symptoms of incontinentia pigmenti and NEMO gene mutations. We designed a NEMO-specific PCR primer, referred to as In2S, to detect a deletion of exon 4-10 of the NEMO gene, which represents the mutation most frequently associated with incontinentia pigmenti. For participants without this deletion, all exons were sequenced to screen for other NEMO mutations. In addition, the clinical manifestations and family histories of the participants were analyzed. RESULTS Exon 4-10 was deleted in 13 probands, and one proband had a novel point mutation (G549C) in exon 5 that converted a glutamine to a histidine. Seven probands (33%) had no mutation in any of the exons of the NEMO gene. One of four participants who presented with hyperpigmentation also had the exon 4-10 deletion. One patient had a positive family history before the study took place, but no NEMO mutation was identified in any of the family members. Remarkably, the mothers of three of the probands exhibited the exon 4-10 deletion; however, their clinical manifestations were subtle and unrecognizable. CONCLUSION Mutational analysis of the NEMO gene was helpful in diagnosing incontinentia pigmenti among participants with a nearly normal phenotype or an incomplete form of the disease that only caused hyperpigmentation symptoms.

Allergic contact dermatitis from incense.

Incense burning has been widely used in Asia, both for its reputed anxiolytic effects and more recently to enhance the ambience of the home (1). Stick incense is made from a combination of fragrant gums, herbs, wood powders, and adhesive materials (2). When it is burned, it releases fragrant smoke. Incense smoke has been reported to induce respiratory disease, lung cancer, and dermatitis (2, 3). We report a further case of contact dermatitis arising from its prolonged and frequent use.

Necrolytic acral erythema without hepatitis C infection

Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. More than 30 patients have been reported since 1996, all of whom had anti‐hepatitis C virus antibody. A 32‐year‐old Taiwanese woman had been diagnosed with and treated for systemic lupus erythematosus with lupus nephritis about 10 years earlier. Soon thereafter, she noted several well‐demarcated keratotic plaques with erythematous borders on her feet, with sparing of the soles. Histopathology showed diffuse parakeratosis with a neutrophil infiltrate, hypogranulosis, pale upper keratinocytes, scattered and grouped dyskeratotic cells, psoriasiform hyperplasia and a mild lymphocytic infiltrate in the upper dermis. The diagnosis was made after three biopsies. The lesions regularly worsened just before and during menstruation, but patch and intradermal tests for progesterone and estrogen were negative. There was no evidence of either hepatitis B or hepatitis C infection. The lesions did not respond to treatment with zinc. The rash regressed spontaneously when corticosteroids were stopped and recurred when they were restarted, finally resolving completely after she was treated with high‐dose pulse steroids for her lupus.

Relapsing polychondritis complicated with Sweet's syndrome and normolipemic neutrophilic xanthomatosis successfully treated by dapsone.

Relapsing polychondritis is a rare disorder characterized by recurrent episodes of inflammation and degeneration of cartilaginous tissues. Sweet’s syndrome is characterized by recurrent painful inflammatory papules, fever, leukocytosis, diffuse neutrophilic infiltrate, and good response to systemic glucocorticoids. Normolipemic xanthoma is defined by xanthoma identified in a normolipemic patient. We report a patient with normolipemic neutrophilic xanthomatosis, Sweet’s syndrome, and relapsing polychondritis that improved rapidly with dapsone. A 70-year-old man had yellowish papules on his chest and abdomen two years after the onset of relapsing polychondritis (Fig. 1a). Histopathologic examination of a skin biopsy specimen demonstrated foamy cells in the

Allergic contact dermatitis induced by zinc pyrithione in shampoo: a case report

Shampoo-induced allergic contact dermatitis is difficult to diagnose clinically because it can involve multiple and variable areas where the shampoo flows. Zinc pyrithione is a common active agent in medicated shampoo that is known to have good anti-dandruff and antifungal effects. Despite its low risk of sensitization, cases of allergic contact dermatitis still occur because of the popularity of such products. We report a 33-year-old man who developed pruritic rash on his scalp, face, neck, and hands after using a new shampoo containing zinc pyrithione. A patch test revealed a positive reaction to zinc pyrithione and personal shampoo containing zinc pyrithione.

Panfolliculoma: report of two cases

Panfolliculoma is a distinctive and unusual benign follicular neoplasm. It shows differentiation towards all components of the hair follicle, including the upper and the lower segments. We report two cases of this rare disease. The first patient presented with a painful nodule on the scalp and the second patient had an asymptomatic skin-colored nodule on his left eyebrow. Histopathologic examination of both cases revealed lobular proliferation of solid-cystic follicular structures. The cystic structures contain laminated keratin, indicating infundibular differentiation. The solid tumor part was composed of basaloid germinative cells, matricial cells, and cells with trichohyalin granules, representing inner root sheath differentiation. The range of differentiations was highlighted using immunohistochemical stains, including labeling of cytokeratin 1, 5, 10, 14 (using 34βE12), cytokeratin 5/6, CD34 (outer root sheath), and Ber-EP4 (lower segment of hair follicle). Panfolliculoma should not be confused with trichofolliculoma or trichoblastoma.

Hydroxyzine-induced acute generalized exanthematous pustulosis

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