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.

Vulvar involvement in autoimmune bullous diseases.

The autoimmune blistering diseases are characterized by involvement of the skin and mucous membranes. On mucosal surfaces, although the initial lesion is often a blister, friction results in erosions. We report on 140 female patients with a variety of blistering diseases examined in the bullous disease clinic. Vulval involvement was found in 5 of 55 adult patients and in 2 of 3 girls with bullous pemphigoid. Sexual abuse was initially suspected in one child. Twenty-six women had cicatricial pemphigoid; of these, 14 had vulval involvement, often with severe scarring. Chronic bullous disease of childhood was identified in 20 girls; perineal involvement was present in 16 causing initial misdiagnosis of herpes simplex in one case and sexual abuse in another. Vulval involvement was less frequent in linear IgA disease (the adult counterpart of chronic bullous disease of childhood), occurring in 9 of 22 patients. One of two adults and two of three girls with epidermolysis bullosa acquisita had vulval involvement. Finally, of nine female patients with pemphigus, four had vulval involvement, all of whom had pemphigus vulgaris. Blistering diseases are rare, but vulval involvement is common across the spectrum of these diseases. When mucosal involvement predominates, diagnostic difficulties can arise.

Seborrhoeic dermatitis of the scalp--a manifestation of Hailey-Hailey disease in a predisposed individual?

A 59-year-old man was found to have typical Hailey-Hailey disease of the back, neck and axillae. In addition, he had fine white scaling in the scalp and postauricular areas. Despite the clinical appearance of seborrhoeic dermatitis, a biopsy of his scalp showed prominent suprabasal epidermal separation with acantholysis. We propose that in a genetically predisposed individual, Hailey-Hailey disease can assume atypical and non-specific appearances.

Allergic contact dermatitis and vulvar dermatoses.

Vulval itch and discomfort are often intractable symptoms and the response to standard therapy can be unsatisfactory. There is some evidence that vulvar sensitivity to irritants is higher than that of forearm skin, but the incidence of relevant allergic contact sensitivity amongst this patient population is unknown. The patch-test data over a 5-year period of 135 patients with persistent vulval symptoms were reviewed and 63 patients had positive results. Of these, 39 had positive results considered to be relevant to their clinical condition. Medicaments and their constituents were responsible for most of these reactions and more than half the patients had multiple allergies.

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.

Mucosal lichen sclerosus/lichen planus overlap syndromes.

Lichen sclerosus and lichen planus affecting cutaneous sites are easily distinguishable clinical. Clinical signs on mucosal sites, however, may not allow differentiation between these diseases, and reliance is frequently placed on histopathological findings. We report a series of seven patients with clinical evidence of coexisting vulval lichen sclerosus and lichenoid oral lesions. All patients had vulval biopsies, and four had oral biopsies. Histology of all the vulval biopsy specimens showed features consistent with lichen sclerosus. Two of these patients developed vulval intraepithelial neoplasia during the course of their disease, and one progressed to a well-differentiated squamous carcinoma. Histology of the oral lesions showed features that were predominantly those of lichen planus. There are, however, few reports of histologically proven oral lichen sclerosus. Variations in histopathological descriptions of lichen sclerosus, depending on duration of disease, have been reported, particularly with regard to the position of the inflammatory infiltrate in relation to the dermo-epidermal junction. We believe that these patients may have oral lichen sclerosus, or at the very least make up a distinctive group who need to be identified and followed up, as their risk of oral dysplasia is unknown.

Bullous and haemorrhagic lichen sclerosus with scalp involvement.

We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this disease which merit consideration. Lichen sclerosus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women. The aetiology is unknown. Approximately 20% of affected patients have extragenital lesions that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhagic forms may occur but these are generally localized and reports of extensive or generalized involvement are rare. We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.

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.

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.

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.

Vulval cicatricial pemphigoid may mimic lichen sclerosus.

We report two patients with vulval signs suggestive of lichen sclerosus. This diagnosis was never substantiated histologically and microscopic appearances were non-specific. In both cases the development of lesions at other cutaneous and mucosal sites alerted us to the possibility that the autoimmune bullous disease, cicatricial pemphigoid, accounted for the entire clinical picture. Lichen sclerosus and cicatricial pemphigoid are distinctive conditions which clinically are usually readily differentiated on cutaneous surfaces. When the site of presentation is on the vulva the clinical picture may become obscure and this can lead to diagnostic confusion. It is important to establish the correct diagnosis as this predicts the clinical course and prognosis in each case.

The association between lichen sclerosus and antigens of the HLA system.

Although frequently linked clinically with autoimmune disease, no immunogenetic basis for lichen sclerosus has ever been established. In this study, we examined in detail the HLA antigens of 84 patients with histologically proven disease, compared with 357 controls. Patients with lichen sclerosus did not have the expected HLA A1, B8, DR3, DQ2 autoimmune profile. Instead, DQ7 was present in 39 of 78 (50%) of patients compared with 89 (25%) controls (P < 0.001). In addition, 61 of 78 patients (78%) had either DQ7, DQ8 or DQ9 antigens, or a combination of these, compared with 142 (40%) controls (P < 0.01). Raised levels of DQ7 correspond to a glutamic acid residue at position 45 of the DQB1 locus. Proline amino acids at position 55 of this DQB1 locus could explain the raised levels of DQ7, 8 and 9, and exert a secondary effect. There is preliminary evidence that the immunogenetic profile of patients with this disease may affect disease expression with regard to site and extent of involvement.

Hereditary hypotrichosis (Marie-Unna type) and juvenile macular degeneration (Stargardt's maculopathy).

Hypotrichosis of the Marie-Unna variety is a distinctive syndrome eponymously named following a publication in 1925 describing a family in which 27 individuals in seven generations were affected by a previously unreported type of hypotrichosis. Its inheritance is determined by an autosomal dominant gene and it usually occurs as an isolated abnormality. Hereditary macular degeneration (Stargardt's maculopathy) is also well recognized and has been reported in one family in association with alopecia areata but never in association with Marie-Unna hypotrichosis. Inheritance of Stargardt's maculopathy is autosomal recessive. Our patient demonstrates the co-existence of these two uncommon genetic disorders and it would appear that both defects have been independently inherited.

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.