Anogenital giant condyloma in an infant with liver transplantation.

Human papillomavirus (HPV) types 6 and 11 were detected in a 3-year-old girl with extensive anogenital condylomata. Although sexual abuse must be considered, non-sexual transmission is evident in at least 57% of children with anogenital warts. Perinatal transmission may occur in approximately 24.5% of infants born to HPV-positive mothers. We present an immunosuppressed child with giant condylomata and discuss transmission, work up, and treatment.

Persistent pruritic papules and plaques associated with adult-onset Still's disease: report of six cases.

Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well-known, and included as one of the major criteria of adult-onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. These lesions show unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with focal hyperkeratosis. We describe herein six cases of AOSD, which presented with skin lesions of persistent pruritic papules and plaques. All six cases were female, and three of them were elderly women. The patients presented with linear erythematous streaks, scaly erythema, keratotic papules, infiltrative plaques and irregular coalesced erythemas. By contrast, histological features were characteristic, and dyskeratotic cells were found in the horny layers as well as in the upper layers of the epidermis. Persistent pruritic eruption is an important cutaneous sign for the diagnosis of AOSD.

Genital pyoderma gangrenosum: report of two cases and published work review of Japanese cases.

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non-infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74-year-old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89-year-old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30-89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia-localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia-localized pyoderma gangrenosum tends to be without systemic complications.

Photoletter to the editor: Proliferating pilomatricoma with no recurrence during a 3-year follow-up.

Proliferating pilomatricoma is a proliferative variant of pilomatricoma. Although it is considered as a benign tumor, local recurrence may occur. We report a case of a 49-year-old Japanese man with 3-year history of an asymptomatic subcutaneous tumor in the nuchal area. Histological evaluation demonstrated a cystic lesion lined by a basaloid epithelium at the periphery and filled with eosinophilic cornified material containing shadow cells in upper part of the tumor, and multilobular proliferation of basaloid cells in association with small foci of shadow cells in the remaining part. Based on these findings, the diagnosis of proliferating pilomatricoma was made. No recurrence has been observed during a 3-year follow-up.

Pathogenic link between hydroa vacciniforme and Epstein-Barr virus-associated hematologic disorders.

OBJECTIVES: To determine the pathogenic association of latent Epstein-Barr virus (EBV) infections with both typical hydroa vacciniforme (HV) and severe HV-like eruptions, and to survey the complications and outcomes of patients. DESIGN: Case series. PATIENTS: Twenty-nine patients with HV or severe HV-like eruptions. INTERVENTIONS: In situ hybridization and immunostaining of biopsy specimens; extraction of DNA samples from cutaneous lesions and/or peripheral blood mononuclear cells for EBV DNA assay. MAIN OUTCOME MEASURES: Clinicopathologic manifestations, hematologic findings, complications, and outcomes; presence of latent EBV infection. RESULTS: T cells positive for EBV-encoded small nuclear RNA (EBER) were detected, to various degrees, in cutaneous infiltrates in 28 (97%) of 29 patients, including all 6 patients with definite HV with a positive phototest reaction, 11 of 12 patients with probable HV without evidence of photosensitivity, and all 11 patients with severe HV associated with systemic symptoms. In addition to EBER-positive T cells, many cytotoxic T lymphocytes expressing T-cell intracellular antigen 1 and granzyme B were present in the cutaneous lesions. Natural killer (NK) cells were absent or at a background level. The UV-induced cutaneous lesions showed histopathologic findings consistent with those of HV, containing many EBER-positive cells. Although no hematologic abnormalities were found in the definite and probable HV groups, the amounts of EBV DNA were increased in the peripheral blood mononuclear cells. By contrast, the severe HV group had markedly increased levels of EBV DNA associated with NK-cell lymphocytosis, and complications including chronic active EBV infection, hypersensitivity to mosquito bites, and hemophagocytic syndrome. Five patients with severe disease died of EBV-associated NK/T-cell lymphomas or hemophagocytic syndrome 2 to 14 years after onset. CONCLUSION: Both typical and severe HV are included within the spectrum of cutaneous disorders mediated by EBV-infected T cells, and the severe HV group may have overt EBV-associated NK/T-cell lymphoproliferative disorders with a frequently fatal outcome.

[Allergic contact dermatitis: how to re-induce tolerance?]. / Eczéma allergique de contact: comment ré-induire une tolérance?

Allergic contact dermatitis (ACD) is a skin inflammatory disease mediated by activation of CD8+ cytotoxic T cells specific for haptens in contact with the skin. CD4+ T cells behave as both regulatory and tolerogenic cells since they down-regulate the skin inflammation in patients with ACD (regulation) and prevent the development of eczema (tolerance) in normal individuals. Thus, ACD corresponds to a breakdown of immune tolerance to haptens in contact with the skin. Several regulatory CD4+ T cell subsets (Treg), especially CD4+CD25+ natural Treg cells, are involved in immunological tolerance and regulation to haptens through the production of the immunosuppressive cytokines IL-10 and TGF-beta. Ongoing strategies to re-induce immune tolerance to haptens in patients with eczema include improvement of existing methods of tolerance induction (oral tolerance, low dose tolerance, allergen-specific immunotherapy, UV-induced tolerance) as well as development of new drugs able to activate IL-10 producing Treg cells in vivo. Ongoing and future progress in this area will open up new avenues for treatment of eczema and more generally autoimmune and allergic diseases resulting from a breakdown of tolerance to autoantigens and allergens, respectively.

CpG immunostimulatory sequences enhance contact hypersensitivity responses in mice.

Bacterial DNA and synthetic cytidine-phosphate-guanosine-oligodeoxynucleotides (CpG ODN) potently activate dendritic cells (DC) and therefore have been proposed as adjuvants for vaccination strategies. Although CpG ODN are considered as safe adjuvants this study shows that CpG ODN are responsible for enhanced antigen-specific skin inflammatory reactions. We used the murine model of contact hypersensitivity (CHS) to 2,4-dinitrofluorobenzene (DNFB) in which hapten-specific CD8+T cytotoxic 1 cells are effector cells. Subcutaneous injection of CpG ODN, 1 d before sensitization enhanced the CHS response to DNFB and resulted in increased recruitment of CD8+ T cells at the challenge sites, whereas control ODN injection did not have any effect. This effect was local and not systemic as it was only observed when DNFB was applied at the same site as the CpG motifs. CpG ODN-induced enhancement of CHS was due to increased antigen-presenting cell functions of DC since: (i) CpG ODN-injected skin revealed upregulated expression of major histocompatibility complex class II, CD80, and CD86 molecules and (ii) CpG ODN treatment of DNFB-derivatized DC enhanced the intensity of CHS responses after in vivo transfer. Taken together, the results show that CpG ODN may be responsible for immune side-effects such as worsening of T cell-mediated skin diseases.

Severely calcified intravenous leiomyomatosis with cardiac extension.

Intravenous leiomyomatosis is a rare benign tumor. In this report we described a case of 72-year-old female who presented with a tumor of this type. Thirty years earlier she had received hysterectomy for uterine myoma. Echocardiography, computed tomography, and inferior vena cavography revealed an intracardiac tumor with calcification arising from the left internal iliac vein. A simultaneous operation was performed successfully with a median sternotomy and left retroperitoneal approach under cardiopulmonary bypass support. Histopathological examination revealed that the resected tumor was a calcified intravenous leiomyoma with cardiac extension. This case showed the three interesting features compared with other reports. Among all reported cases of intravenous leiomyomatosis, this patient and another were the oldest at 72 years old, severe calcification of intravenous leiomyomatosis was infrequent, and the delay (30 years) from hysterectomy to removal of the intracardiac tumor was the longest.