Sarah Neill

British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010

This is an updated guideline prepared for the British Association of Dermatologists’ (BAD) Clinical Standards Unit, made up of the Therapy & Guidelines Subcommittee (T&G) and the Audit & Clinical Standards Subcommittee (A&CS). Members of the Clinical Standards Unit are: M.J. Tidman (Chairman T&G), L.C. Fuller (Chairman A&CS), N.J. Levell, P.D. Yesudian, J. Lear, J. Hughes, A.J. McDonagh, S. Punjabi, N. Morar, S. Wagle (British National Formulary), S.E. Hulley (British Dermatological Nursing Group), K.J. Lyons (BAD Scientific Administrator) and M.F. Mohd Mustapa (BAD Clinical Standards Manager).

Guidelines for the management of lichen sclerosus

Summary These guidelines for the management of lichen sclerosus have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence‐based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.

A clinical study of 23 cases of female anogenital carcinoma.

Background Vulval carcinoma is a relatively rare disorder that may have various aetiologies.

Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus.

Although the precise aetiology of lichen sclerosus is unknown, evidence for an autoimmune basis to the disorder is emerging. Indeed, circulating IgG autoantibodies to the glycoprotein extracellular matrix protein 1 (ECM1) have been demonstrated in the sera of about 75% of affected individuals. To assess this humoral immune response further, immunoblotting was performed using bacterial recombinant proteins spanning different domains of the ECM1 protein. The aim was to identify autoantibody‐reactive sites recognized by 90 lichen sclerosus sera. The subclass distribution of anti‐ECM1 IgG autoantibodies was also determined in 54 lichen sclerosus sera. Immunoblotting showed that the IgG autoantibodies from lichen sclerosus patients recognize multiple antigenic reactive sites on the ECM1 protein within both the amino terminus (50/90, 55.6%) and the protein loop cysteine‐rich repeat domains (54/90, 60%), although few sera (7/90, 7.8%) had antibodies to the carboxyl terminus of ECM1. IgG subclass analysis revealed that the anti‐ECM1 autoantibodies belong predominantly to the IgG2 subclass (48/54, 88.9%), either IgG2 alone (28/54, 51.9%) or in combination with one or more other IgG subclasses. No correlation was found between the site(s) of the ECM1 epitopes or the anti‐ECM1 IgG profile and any specific clinical parameters. Nevertheless, characterization of anti‐ECM1 antibodies does provide further insight into humoral immune responses and understanding disease mechanisms in lichen sclerosus.

Management of anogenital lichen sclerosus

Lichen sclerosus (LS) is a skin condition that affects genital and extra genital epithelia in both males and females of all ages and it may occur in association with other autoimmune disease. Currently, the first line effective treatment is an ultra‐potent topical corticosteroid. The long‐term sequelae of LS include scarring, malignancy, which is rare, and psychosexual disfunction, which is common.

Single-episode photodynamic therapy and vulval intraepithelial neoplasia type III resistant to conventional therapy

Background Photodynamic therapy (PDT) using topical 5‐aminolaevulinic acid (5‐ALA) has been suggested as an effective and tissue‐conserving method of treating carcinoma in situ of the vulva. Objectives To evaluate PDT in patients with vulval intraepithelial neoplasia type III (VIN III). Methods Topical PDT was performed in six patients with VIN III. Five of the six patients had persistent disease following treatment with other modalities including 5‐fluorouracil cream, cryotherapy, carbon dioxide laser ablation and excision. Each patient was treated once with a fluence of 150 J cm−2 using a broad‐band light source (580–740 nm) 4 h after topical application of 20% 5‐ALA. Patients were reviewed clinically at 1 month and 6 months after treatment. Results All of the patients developed initial erythema of treated sites, three with subsequent erosions. All patients had clinically evident persistent VIN III at 1‐month review. Five patients have subsequently undergone surgical treatment and one is regularly reviewed. Conclusions This small uncontrolled study indicates that, as currently administered, a single episode of topical PDT is not effective in the management of treatment‐resistant VIN III.

Psoriasis associated with vulval scarring

Psoriasis is a chronic inflammatory skin disorder which is generally not associated with scarring. We report two patients with long‐standing severe anogenital psoriasis, that was associated with loss of the labia minora, thus clinically mimicking the scarring associated with lichen sclerosus. Histopathological finding were however, consistent with psoriasis with no evidence of lichen sclerosus. Elastic fibres were present and there was no evidence of abnormal collagen or fibrous tissue. The association of vulval psoriasis with scarring has not been reported previously.

Clinical patterns of lichen planopilaris in patients with vulval lichen planus

dilator was used several times a day. The chronic back pain was no longer noticeable. A successful relocation of the sigmoid colon was performed without any complication 13 weeks after the protective stoma had been made. During follow-up visits the patient presented shrinking nonirritated wounds. Defecation was normal, manometry was positive and continence normal. Moreover she reported that she had gained weight and had reached her original body weight again. Anorectal necrosis after paracetamol abuse is rare, only a few cases have been described before. The pathological mechanism leading to anorectal necrosis after paracetamol abuse is still unknown. Paracetamol is widely used as an antipyretic and analgesic drug, however it also has vasoconstrictive properties. Therefore it is applied in neonatology for closure of the ductus arteriosus. It is highly probable that the vasoconstrictive properties are responsible for the perianal necrosis in our patient. In the present case, the correct diagnosis at an earlier time point could have avoided the invasive surgical treatment and shortened the duration of the patient’s disease. This case underlines the importance of a detailed medical history and the clinical experience of the treating physician in reaching the correct diagnosis.

Fixed drug eruption of the vulva secondary to fluconazole

1 Dalghous AM, Freysdottir J, Fortune F. Expression of cytokines, chemokines, and chemokine receptors in oral ulcers of patients with Behçet s disease (BD) and recurrent aphthous stomatitis is Th1-associated, although Th2 association is also observed in patients with BD. Scand J Rheumatol 2006; 35: 472–5. 2 Akdeniz N, Esrefoglu M, Keles MS et al. Serum interleukin2, interleukin-6, tumour necrosis factor-alpha and nitric oxide levels in patients with Behçet s disease. Ann Acad Med Singapore 2004; 33: 596–9. 3 Eedy DJ. Imiquimod: a potential role in dermatology? Br J Dermatol 2002; 147: 1–6. 4 Chakrabarty AK, Mraz S, Geisse JK, Anderson NJ. Aphthous ulcers associated with imiquimod and the treatment of actinic cheilitis. J Am Acad Dermatol 2005; 52 (Suppl.): S35–7. 5 Zalaudek I, Petrillo G, Argenziano G. Aphthous ulcers and imiquimod. J Am Acad Dermatol 2005; 53: 360–1.

A first case of lichen sclerosus with Turner's syndrome.

A 32-year-old woman with Turner s syndrome (TS) had a 6-month history of an intractable vulval itch. Her only medication was hormone-replacement therapy (Prempak-C; Wyeth Pharmaceuticals, Maidenhead, Berkshire, UK) and there was a family history of type 1 diabetes mellitus and thyroid disease. On physical examination, there was whitening and sealing of the clitoral hood over the glans, and pallor on the inner side of the labia majora and on the perianal area. The labia minora were short and pale and there was introital narrowing. Vaginal examination was normal. A clinical diagnosis of anogenital lichen sclerosus was made and the patient was given clobetasol propionate ointment (Dermovate; GlaxoSmithKline, Brentford, Middlesex, UK) to use regularly during the first 3 months and then as required for symptom control. At follow-up, the patient still had mild pruritus. Patch tests to exclude a contact allergic process gave negative results. The patient continued to use the topical ointment intermittently, and the disease was well controlled over a follow-up of 7 years. After this time, a new symptom of vaginal discharge was noted, and on examination the labia minora was found to have flattened into the surrounding skin (Fig. 1a). The introital narrowing was unchanged but lower vaginal adhesions, which were easily divided digitally, were present. An oral examination was normal. A vaginal swab gave negative results. Histological examination of a skin biopsy from the right inner labium majus showed epidermal atrophy, dermal oedema and early dermal hyalinization with a patchy inflammatory cell infiltrate in the subepidermal stroma, suggestive of lichen sclerosus (LS) (Fig. 1b), Direct immunofluorescence to exclude mucous membrane pemphigoid and lichen planus was negative. A reducing course of hydrocortisone foam (Colifoam; Meda Pharmaceuticals, Bishops Stortford, Hertfordshire, UK) applied to the vagina for a month stopped the vaginal discharge. Two years later, the patient developed hypothyroidism. LS affects both sexes but is most commonly seen in the anogenital area in premenarchal girls and postmenopausal women. Its aetiology is unknown but there is a strong familial association and patients usually have a personal history of autoimmune disease. (a)