Shiitake Flagellate Dermatitis: the First Case Reported in Ireland.

Shiitake (Lentinula edodes) is the second most commonly consumed mushroom worldwide. The first case of shiitake mushroom flagellate dermatitis was described in Japan in 1977 and it is now being reported in the western world. We describe the first reported case in Ireland.

Prevalence of methylchloroisothiazolinone and methylisothiazolinone contact allergy in facial dermatitis: a single centre Irish study.

BACKGROUND: There has been an increase in methylchloroisothiazolinone (MCI) and especially methylisothiazolinone (MI) contact allergy in recent years. OBJECTIVES: We examined the prevalence and demography of MCI/MI and MI contact allergy in patients presenting with facial dermatitis. METHODS: Eighty patients with facial dermatitis were patch tested to the British Society for Cutaneous Allergy standard series, preservatives and own products between January 2012 and March 2014. RESULTS: A total of 14 (17.5%) patients had positive reactions to this preservative. Five (6.3%) patients tested positive to MCI/MI (0.02%), four (5%) patients tested positive to MI (0.2%) and five (6.3%) patients tested positive to both preparations. Six (7.5%) of these patients were atopic. All patients were female and the mean age was 53.8 years. Sources of allergen were in cosmetics and skin care products. CONCLUSIONS: Contact allergies to MCI/MI and MI are commoner than what was reported before and is an emerging cause of facial dermatitis. The detection rate of this allergen was increased by 5% in our cohort since the inclusion of MI (0.2%) in the standard series.

Development of mycosis fungoides after bone marrow transplantation for chronic myeloid leukaemia: transmission from an allogeneic donor.

We report on a patient who developed donor-derived cutaneous T-cell lymphoma (CTCL) 4 years after successful treatment of chronic myeloid leukaemia with an allogeneic bone marrow transplant. The patient developed an eczematous rash unresponsive to topical therapy and immunosuppression. When CTCL was diagnosed in the recipient, his sibling donor had been attending his local dermatology unit with a maculosquamous rash, which proved subsequently to be mycosis fungoides. An identical pattern of donor and recipient clonality assessment and T-cell receptor gene sequencing indicated that the CTCL was probably transmitted in the bone marrow harvest. This suggests that CTCL cells circulate in the marrow at an early subclinical stage in this disease. This is the second case of donor-derived CTCL reported to date.

Drug-associated linear IgA disease mimicking toxic epidermal necrolysis.

Linear IgA disease (LAD) is an uncommon, acquired, autoimmune blistering disorder with a characteristic linear IgA deposition along the basement membrane zone. LAD can be idiopathic or drug-induced. Drug-related LAD most commonly occurs after exposure to vancomycin, but phenytoin and other medications have been implicated. A small number of cases of drug-induced LAD clinically resembling TEN have been reported. We report a case of phenytoin-associated LAD clinically mimicking TEN in a 57-year-old woman. This case shows the necessity of investigating TEN patients for autoimmune bullous diseases, using immunofluorescence studies.

Genital lichen sclerosus associated with morphoea or systemic sclerosis: clinical and HLA characteristics.

Although patients with both morphoea and lichen sclerosus have been reported previously, in the majority of these reports the lichen sclerosus has been extragenital. We report nine patients in whom genital lichen sclerosus coexisted with scleroderma spectrum disorders including seven with morphoea, one with morphoea and lichen planus, and one with systemic sclerosis. The clinical features, associated autoimmune disease, autoantibodies and HLA type are reported. Antibodies to Borrelia burgdorferi were not detected in any of the patients. The coexistence of these diseases raises a number of intriguing questions about the relationship between them.

Lichen sclerosus premenarche: autoimmunity and immunogenetics.

Lichen sclerosus is among the most frequently seen paediatric vulval disorders. In adults a strong association between lichen sclerosus and autoimmune diseases, and also with HLA class II locus DQ7, has been well demonstrated in women and a weaker association in men. These associations have not previously been studied in children, although in other autoimmune diseases, the HLA associations have been strongest in children. We performed HLA tissue typing and looked for autoimmune associations in a group of 30 children with vulval lichen sclerosus. HLA DQ7 was present in 66% of female children with lichen sclerosus compared with 31% in controls. Previous studies reported DQ7 in 51% of adult female patients and 45% of male patients. Sixteen per cent of the children were homozygous for DQ7 as opposed to 5% of controls. In the childhood group, only 4% had another autoimmune disease, but 56% of their parents or grandparents did. Age differences make comparison difficult, but the family history of autoimmunity appears to be strong in the early-onset group, in addition to the stronger association with DQ7.

Xanthoma disseminatum with respiratory tract involvement and fatal outcome.

Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from acute respiratory failure. The post-mortem findings and the importance of respiratory tract disease in this unusual condition are discussed.

Lichen sclerosus: evidence that immunological changes occur at all levels of the skin.

An immunohistochemical approach was used to characterize the inflammatory infiltrate in vulval lichen sclerosus, using monoclonal antibodies to CD3, CD4, CD8, CD68 and HLA-DR. Significant numbers of CD4 + and CD8 + lymphocytes were observed in the dermal band of inflammatory cells in approximately equal proportions. Less numerous CD4 + and CD8 + lymphocytes also occurred adjacent to the dermoepidermal junction and occasionally in the lower epidermis. Increased numbers of cells staining with the monocyte/macrophage marker CD68 were also present in the band of inflammatory cells as well as being scattered diffusely throughout the sclerotic region. Expression of HLA-DR in the lichen sclerosus specimens was increased within the inflammatory infiltrate and around blood vessels in the dermis. All the vulval lichen sclerosus specimens also demonstrated some HLA-DR expression around the keratinocytes, suggesting that these keratinocytes might be involved in antigen presentation. We also studied the expression of CD44 and its isoforms 3G5 (marker of V3), 8G5 (marker of V6), 3D2 (marker of V4/5) and IE8 (marker of V8/9). CD44 has been proposed to play a part in lymphocyte homing, cell-matrix interaction (particularly with hyaluronic acid), lymphocyte activation and malignant progression of certain tumours. The epidermis of the lichen sclerosus specimens appeared to demonstrate a greater intensity of staining with the pan-CD44 marker F10-44, and reduced staining with 3G5, 3D2 and IE8 compared with normal skin. Like normal skin, the dermis of the lichen sclerosus specimens did not demonstrate staining with 3G5, 3D2, 8G5 or 1E8, but did show staining with F10-44. However, the pattern of the dermal staining with F10-44 reflected the position of the inflammatory infiltrate and was sparse in the five sections where there was a prominent sclerotic zone, but increased in the three sections where there was a prominent band of inflammation cells. Our results demonstrate evidence of immunological changes at all levels of skin involved by lichen sclerosus, including the epidermis.

Lichen sclerosus in adult men: a study of HLA associations and susceptibility to autoimmune disease.

Lichen sclerosus is a skin disorder of unknown prevalence affecting both men and women, and several studies have established HLA associations in women with this disease. Autoimmune disease associations in the form of a personal and/or family history of autoimmune disease have also been shown to be related to lichen sclerosus. In this study, we examined 58 men (mean age 38 years) with lichen sclerosus, 39 of whom had histologically proven disease. HLA tissue typing by phototyping was performed on these patients and contrasted with that of 602 control subjects. There was no difference in antigen frequencies of the HLA class I loci. The patient group was found to have an increased frequency of several HLA antigens of the class II loci: DR11, 13 of 58 (22%) patients vs. 75 of 602 (13%) control subjects (P = 0.05); DR12, five of 58 (9%) patients vs. 16 of 602 (3%) control subjects (P = 0.04); DQ7, 26 of 58 (45%) patients vs. 189 of 602 (31%) control subjects (P = 0.05). There were few autoimmune disease associations: two of 58 (3%) patients had a personal history of a different autoimmune disease, two patients were found to have abnormal thyroid function and six of 58 (10%) had a first-degree relative with an autoimmune disease. There was no difference in the frequency of the autoimmune haplotype HLA A1, B8, DR3/17, DQ2 compared with the control population. HLA DQ7 has now been shown to occur more frequently in both male and female patients with lichen sclerosus, which may reflect the immunopathogenesis of the disease. Autoimmune disease associations, however, are less common in men with lichen sclerosus.

Compositae sensitivity and chronic hand dermatitis in a seven-year-old boy.

Compositae dermatitis is rare in childhood, with few cases documented in the literature. We report a 7-year-old boy who presented with a dermatitis mainly affecting his dominant hand. Patch testing revealed contact sensitivity to sesquiterpene lactone (SL) mix, and subsequent testing with Compositae oleoresins showed sensitivity to daisy, dandelion and chrysanthemum. The dermatitis improved on avoidance of handling plants. We have not documented any other cases of Compositae sensitivity in 187 other children, aged under 16 years, who have undergone patch testing with SL mix between 1992 and 1996 in our department. This case illustrates that Compositae sensitivity may present with a localized dermatitis and that, although uncommon, sensitization may occur in early childhood.

A study of clinical and aetiological factors and possible associations of lichen sclerosus in males.

Lichen sclerosus is a chronic skin condition with a predilection for the genital area. In the present study, 35 male patients with lichen sclerosus were interviewed and examined. Blood screens were performed and histology was requested if not already performed. The findings indicate that lichen sclerosus in males exists as a spectrum of disease, ranging from a mild form with white plaques and few symptoms to a severe form with inflammation, atrophy and scarring with possible urological consequences. In many areas it differs from the condition in females; the association with autoimmune disease is weaker and there is less perianal and extragenital involvement. The association with malignancy in males is of lesser significance than initially believed.

The basement membrane zone in lichen sclerosus: an immunohistochemical study.

The alteration in expression of basement membrane zone (BMZ) components in lichen sclerosus was investigated by immunohistochemical staining of skin biopsies from seven patients with histologically confirmed disease compared with controls. Monoclonal antibodies and polyclonal sera directed against proteins of the hemidesmosomes, anchoring fibrils, lamina lucida, lamina densa and BMZ collagens were used. Characteristic histological appearances at the dermo-epidermal junction were reflected in widespread alterations in antigen expression in the epidermal basement membrane and the papillary dermis. Expression of the proteins which constitute the structural scaffold (collagen IV and VII) were increased in lichen sclerosus. Expression of hemidesmosomal proteins which mediate adhesion and cell to matrix interaction (alpha 6 beta 4 and bullous pemphigoid antigen) and expression of anchoring filament components were markedly reduced, suggesting that the epidermal cells are exposed to selective damage.

Disseminated superficial actinic porokeratosis occurring in two members of an Asian family.

Disseminated superficial actinic porokeratosis (DSAP) is a genodermatosis which usually affects fair-skinned individuals at sites of chronic sun exposure, although the face is usually spared. We now report the occurrence of this condition in a Pakistani mother and her 13-year-old daughter, both predominantly with facial lesions.