A. D. Burden

Nail dystrophy due to lichen sclerosus

Lichen sclerosus (LS) affects anogenital skin alone in 80% of cases. When extragenital disease occurs, it usually affects the trunk, neck, axillae and wrist flexures. Nail involvement with LS is rare. In contrast, lichen planus (LP) commonly affects extragenital skin. Mucosal lesions occur in 50% of cases, affecting the mouth and genitalia. Nail disease in LP is common, and, if severe, can lead to destruction of the nail bed. LS and LP can coexist. We report two cases of LS with nail involvement. In the Case 1 disease was confined to the nail, and nail biopsy confirmed LS. In the Case 2, the nail changes formed part of the widespread genital and extragenital LS, confirmed histologically. We review existing literature on nail disease in LS and discuss the possible aetiology of the nail changes.

Treatment of multiple scalp basal cell carcinomas by photodynamic therapy.

The use of surface protoporphyrinu2003IX fluorescence detection to delineate multiple superficial basal cell carcinomas on a patients scalp is described. Photodynamic therapy (PDT) was subsequently performed with clearance of six and partial clearance of the remaining two tumours. The treated lesions have not recurred during 12u2003months of follow‐up. The opportunity to combine diagnostic fluorescence detection with subsequent treatment by PDT offers an effective and practical management option. PDT is a tissue‐sparing modality with low morbidity and good cosmesis, leading us to propose 5‐aminolaevulinic acid–PDT for multiple superficial basal cell carcinomas of the scalp.

Cutaneous actinomycosis presenting as chronic mastitis.

Actinomycosis is a chronic granulomatous suppurative infection caused by anaerobic actinomyces. Primary cutaneous involvement is uncommon because of the exclusively endogenous habitat of the organism. We describe a very unusual presentation mimicking chronic mastitis. A 35‐year‐old woman presented 7u2003months post‐partum with tenderness and induration in the right breast. She was pyrexial and felt systemically unwell. An initial diagnosis of mastitis was made. Treatment with penicillin, imipenem, co‐amoxiclav and metronidazole had no effect. Skin biopsy revealed the characteristic ‘sulphur granules’ of actinomycoses in the deep dermis. Long term oral clindamycin (>u200312u2003months) has produced a very good response clinically, with a concomitant decrease in inflammatory markers. Cutaneous actinomycosis has been described by haematogenous spread from visceral organs or after trauma. The organism is difficult to culture and is often diagnosed histologically by the presence of ‘sulphur granules’. It is very sensitive to penicillin but prolonged treatment is needed.

Human Trichophyton equinum infection treated with terbinafine

A case of tinea capitis caused by Trichophyton equinum is described. This responded to a 6‐week course of treatment with the allyl amine antifungal agent, terbinafine.

Meyerson's phenomenon around a seborrhoeic keratosis.

Sir, A 65-year-old man presented with a 6-month history of an 8-mm diameter crusted lesion with surrounding ring of small red papules on his left shin (Fig. 1) which had become itchy. The rest of his skin was normal and he denied any prior skin problem. He was well, and not taking any systemic or topical medication. There was no history of atopy or hypersensitivity. Patch testing to the European standard series was negative. The central crusted lesion was excised and histology revealed features of an in ̄amed seborrhoeic keratosis. A diagnostic biopsy of the surrounding area carried out at the same time showed marked spongiosis with conspicuous eosinophils in the dermal in®ltrate. A diagnosis of irritated seborrhoeic keratosis with halo dermatitis was made. The itchy dermatitic patch healed very quickly with a moderately potent topical steroid. The appearance of a ring of dermatitis around a melanocytic lesion was described by Meyerson in 1971. This phenomenon has been reported in relation to melanocytic lesions in more than 50 cases but has also been described around nonmelanocytic lesions on a few occasions, and therefore the term Meyersons phenomenon or halo dermatitis (halo eczema) has been suggested. There is no agreement as to the aetiology of halo dermatitis. Meyersons naevi usually affect the trunk of young (average age 25 years) men (male : female ratio 3 : 1). Multiple naevi are involved either simultaneously or consecutively in about two-thirds of cases. Occasionally patches of eczema may appear beyond the melanocytic naevi. The dermatitis resolves spontaneously in many cases within a few months, mostly without involution of the melanocytic naevus. The dermatitis responds to a potent topical steroid but has also been reported to clear when the central naevus is excised. The presence of eosinophils in the dermal in®ltrate along with spongiotic dermatitic changes have been regarded as consistent features by several authors. Tegner et al. reported halo dermatitis around a seborrhoeic keratosis, an irritated seborrhoeic keratosis and a stucco-keratosis. Rosen et al. also noted similar features surrounding two seborrhoeic keratoses. Halo dermatitis has also been described around dermato®broma. Halo dermatitis around nonmelanocytic lesions is noted predominantly in an older age group, perhaps because of the later development of the primary lesions. Some consider the dermatitis that frequently appears around lesions of molluscum contagiosum to be an example of the same process. Histology of dermatitis around Meyersons naevus and halo dermatitis is similar. Unlike a similar patient described by Tegner et al. our patient did not go on to develop widespread nummular dermatitis.

Lentigines in psoriatic plaques: are they unique?

Sir, Pantoprazole is one of the proton pump inhibitors (PPIs) widely used to treat peptic diseases. Pantoprazole has a lower affinity than omeprazole or lansoprazole for the hepatic cytochrome P450 enzyme system and it has been assumed to have lesser drug interactions and side-effects. As a family, PPIs have been considered to be well tolerated, with minor side-effects, involving mainly the gastrointestinal tract, the central nervous system and the skin. An autoimmune syndrome with the characteristics of lupus or bullous pemphigoid has been rarely associated with omeprazole. A 73-year-old woman was treated with pantoprazole (Pantocw) 40 mg/day for oesophagitis and gastritis. Eight days later she developed an erythematous maculopapular eruption, with burning sensation, over the face, scalp, ears and anterior V of the chest, that gradually worsened within the next 2 weeks. She stopped pantoprazole and presented to us with residual blistering and crusting (Fig. 1). She had also been taking indapamide, atenolol and fosinopril for 3 years, and pravastatin and hydroxytryptophan for 1 year. Past medical history included hypertension and a mastectomy 10 years previously. A scalp biopsy was compatible with a phototoxic dermatitis, with a negative direct immunofluorescence. Blood abnormalities included a positive anti-SSA (28 EU/mL; normal , 20 EU/mL) and rheumatoid factor (47.4 UI/mL; normal , 35 UI/mL). She was treated with oral antihistamines and prednisolone for 2 weeks, along with emollients, topical steroids and a sunscreen. Pantoprazole was discontinued, but the other medications were continued. One month later clear clinical improvement was evident, with only a defluvium, while the anti-SSA positivity disappeared. Four months later, patch and photopatch tests (5 J/cm UVA) to pantoprazole and all the components of Pantocw (kindly provided by Byk Gulden), were all negative. Six months after the reaction, an alopecia remained apparent along with some cicatricial areas, and slight atrophic lesions over the ears and face, but no medication was instituted. Fourteen months after the described phototoxic reaction, the patient presented several erythematous and atrophic lesions with fine scaling and telangiectasia, localized on the scalp, face and ears (Fig. 2), and anterior upper chest, in the areas where the apparent phototoxic reaction had been most intense. We performed biopsies over the left lobule of the ear and right eyebrow areas: both were compatible with the clinical diagnosis of discoid lupus with positive direct immunofluorescence to IgM at the dermoepidermal junction. Rheumatoid factor was still positive but all other autoantibodies (ANA, anti-DNA, anti-SSA, and anti-SSB) and complement were negative or normal. She was treated during summer time with hydroxychloroquine (200 mg/day), topical steroids, and sunscreen. Now, more than 2 years after the initial reaction, the disease is well controlled. PPIs are benzimidazole derivatives that may induce a variety of skin disorders: oedema, pruritus, urticaria, xerostomia and myalgia have been reported with a frequency from 1 to 10%. Blistering eruptions, including toxic epidermal necrolysis and bullous pemphigoid (either exacerbated or induced), along with lichen planus and pityriasis rosea have also been suggested.

Coexistent granulomatous vasculitis and leukaemia cutis in a patient with resolving herpes zoster

An 80‐year‐old man presented with a 6‐month history of indurated tender purple papules. These had coalesced to form plaques with some central scarring and a dermatomal distribution on the left arm, immediately following herpes zoster (HZ) infection at this site. The patient had a 5‐year history of small lymphocytic lymphoma (SLL), which was being managed conservatively under a ‘watch and wait’ protocol. On histological examination of a skin biopsy, marked interstitial granulomas and prominent granulomatous vasculitis were seen, supporting the clinical impression of a post‐HZ granulomatous reaction. In addition, there was a dense monoclonal small B‐cell lymphocytic infiltrate indicating koebnerization by SLL (a finding that has not been reported previously with concurrent postherpetic granulomatous vasculitis). Although benign pseudolymphomas occur in postherpetic cases, this case shows that even in association with benign vasculitic features true lymphomas can occur. Furthermore, this case highlights the importance of immunocytochemistry, molecular studies and clinicopathological correlation.

Psoralen ultraviolet A-induced melanonychia.

A 53-year-old white man presented with a 10-year history of psoriasis affecting the dorsa of both hands. There were no associated nail changes, and the patient’s feet were unaffected. His mother had psoriasis, which was being treated with methotrexate. He had failed to respond to topical treatments, including superpotent corticosteroids, combined potent corticosteroid with calcipotriol, and dithranol. Topical hand psoralen ultraviolet A (PUVA) photochemotherapy was commenced, but after 3 weeks of twice-weekly treatment, there was little benefit. Thus, the treatment was changed to oral 8-methoxypsoralen (8-MOP) PUVA. Twice-weekly treatments were given for 5 weeks (cumulative dose 61.5 J/cm), but the patient was unable to tolerate treatment due to irritation and blister formation. At follow-up, the patient was found to have symmetrical longitudinal bands of nail pigmentation affecting all fingernails, but most prominently, his thumbs (Fig. 1a,b). The patient reported onset of these bands within the first week of starting oral PUVA treatment. A number of speckled lentigines were seen over sites of previous plaques of psoriasis on the patient’s fingers. New pigmentation was not seen at any other sites, and specifically, the toenails were spared. Laboratory investigations, including full blood count, urea and electrolytes, liver and thyroid function tests, bone profile, haematinics and glucose all gave normal results. Four months after treatment, the bands of melanonychia had completely resolved. The development of pigmentary changes on the skin during PUVA photochemotherapy is well documented, with lentigines being the most frequent change. Development of melanonychia is much rarer, with only occasional cases reported in the literature. The mechanism of melanonychia development is not clear. Perria found that it is the distal, rather than the proximal matrix of the nail that contains an active melanin synthesis compartment. It is suggested that on exposure to UVA, the melanocytes in this compartment are stimulated to increase melanin production, resulting in melanonychia. Some reports suggest that the UVA dosage is the key factor, as PUVA-induced lentigines are seen after a cumulative dose of 440 J/cm. In our case, the melanonychia occurred after a much lower cumulative dose (61.5 J/cm), which is similar to other reports, although the dose varies, with incidences of 42–430 J/cm cumulative UVA doses reported. A more widely accepted theory is that the type of psoralen is of greater importance, with almost all reports of PUVAinduced melanonychia occurring in patients who were given 8-MOP. The course of the melanonychia is variable, but in most cases, it seems to resolve after treatment is discontinued. The melanin that has been transferred onto the (a)

The Woronoff ring and fumaric acid ester flush

A 46-year-old man with early-onset sporadic psoriasis was treated with fumaric acid esters (FAEs) and oral methotrexate. One of the common side-effects of FAEs is flushing, which the patient experienced during a clinic visit. On physical examination, typical psoriasis lesions were seen on the trunk, with a surrounding pale halo representing the Woronoff ring (Fig. 1). In 1926, Woronoff described a pale area of normal skin surrounding a treated area of psoriasis. This phenomenon is now known as a Woronoff ring, although the sign is not commonly noted and the pathogenesis is unclear. It has been described after treatment with ultraviolet light, topical tar, topical corticosteroids and dithranol treatment, and also in patients with untreated psoriasis. The nature of the Woronoff ring has had little investigation. Woronoff first reported characteristic histological features in the rings such as epidermal acanthosis, which had defined demarcation between lesional and nonlesional skin. However, parakeratosis was not seen. More recently, it has been shown that the only abnormalities in the Woronoff ring were minimal acanthosis and a decrease in the amount of epidermal melanin present in the halo. In 1976, Penney et al. reported that the area of whiteness was approximately the same width around the psoriatic plaque, regardless of the size and shape of the plaque. Furthermore, the pale ring around the psoriatic lesion does not develop erythema when coal tar or dithranol is directly applied, or during ultraviolet therapy. This suggested that the inability of the Woronoff ring to redden was due to local deficiency of prostaglandin (PG)E2. To investigate this further, PG was injected into the skin outside the Woronoff ring in two patients with an injection of normal saline used as a control. The injection of prostaglandin produced redness in the Woronoff ring, whereas that of saline did not. PGE2 levels were found to be reduced in the Woronoff ring compared with adjacent nonlesional skin. This suggests that an inhibitor of prostaglandin synthesis may be present possibly diffusing outwards from the psoriatic plaque. Studies of the cutaneous and subcutaneous blood flow in psoriatic skin showed that the colour of the Woronoff ring could not be ascribed to local cutaneous vasoconstriction. Van de Kerkhof et al. found that the glycoprotein endoglin was decreased in the marginal zone of psoriatic plaques. Endoglin is a scavenger of transforming growth factor-b, which inhibits the extravasation of peripheral white blood cells. Therefore, decreased endoglin could lead to reduced inflammation and thus account for the pale ring seen. FAEs were introduced for treatment of chronic plaque psoriasis in 1959. Fumaderm , (Almirall Hermal, GmbH, Reinbek, Germany) the commercially available form, is licensed in Germany for psoriasis but not as yet in the UK. However, FAEs are being increasingly used to treat patients

Buccal prochlorperazine causing perioral fixed drug eruption.

factor as the probable culprit. The subject had used a galenic topical preparation prepared by a pharmacist, containing ascorbic acid 1%, 40 g fluprednidene acetate 0.1% and miconazole nitrate 2% cream, and 20 g of an emollient base containing various compounds such as cystine, lipids, D-panthenol and vitamin E, which he had obtained as an over-thecounter medication to prevent onychomycosis . He had applied it to all fingernails and toenails for 3 months before he had noticed the yellow hue. He denied use of any other topical or systemic agents. No clinical signs of onychomycosis were present at the time of the examination. Ascorbic acid was implicated as the probable culprit, as prolonged exposure to the environment of this substance in liquid form causes oxidation and a gradual change in colour from white to yellow. The shape of the discoloration, which followed the shape of the proximal nail fold, suggested that the nail matrix was intact, and that the cause was not systemic. Nail staining is often seen with dissolved rather than suspended pigments, and it is commonest with deep red nail polishes that contain the dyes D & C reds numbers 6, 7 or 34, or 5 lake. The nail plate is stained yellow after approximately 7 days of continuous wear of such cosmetics. Lake pigments are manufactured by precipitating a dye with an inert binder, usually a metallic salt. Ascorbic acid for galenic preparations is available in powder form (or tablets pulverized to powder) containing magnesium stearate and silicon dioxide. It is possible that interaction between the ascorbic acid and the magnesium salt might result in the final product acquiring a staining capability similar to lake pigments. Oxidation of ascorbic acid also results in the substance acquiring a yellow hue, and gradual deposition of the oxidized product on the nail plate layers could result in the yellow chromonychia. Although ascorbic acid has been used for treatment of a number of skin diseases, we could not find any references to topical treatment or reported adverse events after application to the nails in the available medical literature. We advised our patient to discontinue use of the preparation. At a follow-up visit 2 months later, there was complete resolution of the disorder (Fig. 1b). This case emphasizes that although such preparations are safe, an embarrassing outcome might result after application of non-evidenced-based, over-the-counter remedies for the prevention of cutaneous disorders.