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Background: Abrocitinib, an oral Janus kinase 1 inhibitor, provided significant itch relief by week 2 in patients with moderate-to-severe atopic dermatitis (AD) in the phase III JADE COMPARE trial. Objectives: This post-hoc analysis of JADE COMPARE aimed to further characterize itch response and determined whether early itch relief could predict subsequent improvements in AD severity. Methods: JADE COMPARE was a randomized, double-blind, double-dummy, placebo-controlled trial. Adult patients (aged ≥ 18 years) with moderate-to-severe AD were randomly assigned to receive oral abrocitinib 200 mg or 100 mg once daily, subcutaneous dupilumab 300 mg every other week (after a 600-mg loading dose), or placebo, plus medicated topical therapy for 16 weeks. Assessments were ≥ 4-point improvement in Peak Pruritus Numerical Rating Scale (PP-NRS4) from days 2 to 15, Eczema Area and Severity Index (EASI), Investigator’s Global Assessment (IGA) response, and Dermatology Life Quality Index (DLQI) scores at week 12. Association between week 2 PP-NRS4 and efficacy at week 12 was evaluated by chi-squared tests. The predictive value of early response for later efficacy was assessed by area under the receiver operating characteristic curve. Results: As early as day 4 after treatment, a significantly greater proportion of patients achieved PP-NRS4 response with abrocitinib 200 mg (18.6%) versus dupilumab (5.6%; p

A number of neutrophilic dermatoses are associated with malignancies and their treatment. These rarely occur together in the same patient. A Caucasian 72-year-old male was treated for acute myeloid leukemia (AML) with chemotherapy including daunorubicin and cytarabine. Within 48 hours of commencing treatment, he developed pyrexia and, two days later, disseminated non-tender pink plaques on the limbs and trunk. A skin biopsy showed a dermal interstitial infiltrate of lymphocytes, histiocytoid cells and predominantly neutrophils. This extended into the subcutis, where a neutrophilic lobular panniculitis was seen. These findings are consistent with Sweet’s syndrome. In addition, a neutrophilic and lymphocytic infiltrate was also present around eccrine coils and lower ducts. The eccrine epithelium showed squamous metaplasia with dyskeratosis and sloughing into the lumen. These latter findings are consistent with neutrophilic eccrine hidradenitis (NEH). These two histologically distinct entities form part of the neutrophilic dermatoses that have been described in oncology patients with reports of concurrent or sequential occurrence of various neutrophilic dermatoses in the same patient. Ours, however, is only the second reported case of simultaneously captured Sweet’s and NEH in the setting of AML. The most likely explanation is that of an epiphenomenon, whereby the neutrophilic infiltrate extended around the sweat glands in the context of the neutrophilic dermatosis.

Summary We present the case of a 54-year-old patient with renal transplant who developed unusual vascular changes on the forearm distal to a functioning arteriovenous fistula, as well as a painful ulcerated lesion on her anterior abdominal wall. We believe that the diffuse dermal angioendotheliomatosis variant of reactive angioendotheliomatosis had a role in the pathogenesis of this patient's lesions.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a condition that was first described in 1990 in a group of children, but has since been described in adults. We present the cases of identical twin patients aged 40 years. The first brother presented with an 8-year history of itchy lesions over the left ankle and the insteps of both feet. After a diagnostic biopsy, he was treated with potent steroids under occlusion for 8 weeks, which resulted in flattening of the lesions and resolution of the pruritus. The second twin had a 20-year history of a very similar presentation but the lesions were less pronounced; he chose not to have treatment. No other family members were affected. Skin biopsies from both patients showed similar changes. Within an overall hyperkeratotic and acanthotic epidermis, there were focal areas of lichenoid change and epidermal thinning. Beneath these areas, there was oedema and nodular aggregates of dense inflammatory cell infiltrate, predominantly lymphocytic infiltrate. APACHE has not been previously described in twins.

We report a case of idiopathic hypereosinophilic syndrome (HES) presenting with cutaneous infarction and subsequent extensive deep vein thrombosis. The eosinophilia improved dramatically with systemic corticosteroid therapy. A variety of skin disorders have been associated with HES, although there are no previous reports of HES associated with cutaneous infarction. HES is a rare disorder characterized by a sustained overproduction of eosinophils and multisystem disease. The aetiology of the eosinophilia remains uncertain but clonal populations of abnormal T-cells producing interleukin 5 may be implicated.

The aim of this study was to compare the efficacy and tolerability of twice-daily vs. once-daily regimes of dithranol (anthralin) in Lassar's paste. Over a 4-year period, 61 inpatients with stable plaque psoriasis gave informed consent and entered a randomized controlled trial, having twice or once-daily application of dithranol in Lassar's paste as part of otherwise standard Ingram's regime. Primary outcome measurements were time required in hospital, nursing time, changes in total body surface area affected by psoriasis and thickness of a target plaque and in some patients, an assessment of the recurrence of psoriasis. Doctors were blinded as to the regime being used. At entry, mean patient age, lesional surface area and target plaque thickness were comparable in both groups and no patient had received systemic therapy in the preceding 3 months. Forty-two patients completed the study, two (11%) in the twice-daily group withdrawing due to skin irritation or 'burning'. Mean lesional surface area and target plaque thickness were similar in both groups at hospital discharge. Mean (+/- SD) time spent in hospital was not significantly different in each group, being 13.3 (+/- 6.2) days and 13.9 (+/- 4.5) days for the twice-daily and once-daily groups, respectively (P = 0.36). Duration of hospitalization did not correlate with surface area or plaque thickness on admission. Mean (+/- SD) nursing time spent on treatment was significantly greater in the twice-daily group, at 0.82 (+/- 0.33) hours per day compared with 0.51(+/- 0.25) hours per day in the once-daily group. Relapse rate at 6 months was not different between the two groups.

CD56+ lymphomas derived from natural killer (NK) cell lineage are rarely encountered in Western populations and their clinical and pathological features have not been fully defined. The majority of reported cases are lymphomas of the nasal cavity, which are most commonly seen in Asia. A subtype of CD56+ lymphoma has recently been described (blastoid NK-cell lymphoma) which characteristically presents in older patients with cutaneous infiltrates and disease at other nodal and extranodal sites. We describe a case that correlates well with the clinicopathological features of blastoid NK-cell lymphoma. An unusual feature in our patient was that the cutaneous features of the lymphoma showed complete resolution shortly following commencement of oral steroid therapy.

Objective To evaluate a novel coconut-derived emulsion (CDE) shampoo against head lice infestation in children. Design A school trial in which pupils were treated on days 0 and 7 and checked on days 8 and 15 and a family trial where product was applied by parents three times in 2 weeks or used as a cosmetic shampoo and checked on days 14 and days 70. Setting UK schools in Bristol and Western-super-Mare and families in Northern Somerset. Main outcome measure Numbers of children free from infestation after treatment. Results In the school trial, percentage cures at day 8 were 14% (permethrin, n = 7) and 61% (CDE, n = 37). In the family trial where all family members were treated, cure rate was 96% (n = 28), and if the shampoo was subsequently used as a cosmetic shampoo, only 1 of 12 children became re-infested after 10 weeks. Conclusion CDE shampoo is a novel effective method of controlling head lice and used after treatment as a cosmetic shampoo can aid in the reduction of re-infestation.

We report a missense mutation in the gap junction protein beta-3 (encoding Connexin 31), which was detected in only the affected members of a family in which the autosomal dominant skin disease erythrokeratoderma variabilis was segregating. The nucleotide change results in an arginine to proline substitution in codon 42. This residue is positioned on the first transmembrane/first extracellular domain of the gap junction protein with the mutation replacing a negatively charged residue with a nonpolar residue. This change may disrupt the conformation of the protein and voltage gating polarity leading to impaired channel function.

The tricyclic antidepressant, doxepin, is known to have H1 and H2 antihistaminic effects. Recently, 5% doxepin cream has been marketed in the U.S.A. for treatment of eczematous dermatoses. We investigated the effects of topical doxepin treatment on histamine-, substance P- and prostaglandin E2- (PGE2) induced responses in the skin of normal and atopic subjects. We compared the effects of topical doxepin with those of the oral antihistamine terfenadine. The weal volume and flare area responses to histamine were significantly reduced by treatment with topical doxepin or oral terfenadine in both normal and atopic subjects (P < 0.05). The mean +/- SEM percentage reduction in flare area for 10 micrograms/site of histamine in non-atopics and atopics was 48 +/- 8% and 60 +/- 17% with terfenadine, and 54 +/- 12% and 81 +/- 4% with topical doxepin, respectively. The mean percentage reduction in weal volume for the same dose of histamine in non-atopics and atopics was 70 +/- 9% and 63 +/- 16% with terfenadine, and 96 +/- 2% and 89 +/- 6% with topical doxepin, respectively. The flare but not the weal response to substance P was inhibited by both treatments in all subjects (P < 0.05). The mean +/- SEM percentage reduction in flare area for 200 pmol/site of substance P in non-atopics and atopics was 53 +/- 10% and 73 +/- 4% with terfenadine, and 74 +/- 7% and 75 +/- 4% with topical doxepin, respectively. The cutaneous responses to PGE2 were not affected by either drug. The inhibitory effects of doxepin were as great as those of terfenadine, and doxepin had a significantly greater effect than terfenadine in inhibiting the weal response to histamine and flare response to substance P in normal volunteers (P < 0.05). There was no significant difference between atopics and non-atopics in the percentage reduction of cutaneous responses by oral terfenadine or topical doxepin. Marked sedation occurred in three of the first 10 subjects treated with topical doxepin, necessitating a reduction in dosage for the remaining six subjects. In summary, topical doxepin was as effective as, and sometimes more effective than, a standard dose of oral terfenadine in the inhibition of histamine-induced and axon-reflex-mediated cutaneous responses. The marked sedative effect may limit its clinical use in some patients.

Twenty-five cases of distinctive ear nodules on the helices of the ears in elderly males are presented. We believe the clinicopathological features of these lesions allow them to be defined as a distinct entity. They are probably more common than is currently recognized. Histologically, a typical nodule comprises a spur of fibrous tissue with a focus of cartilage metaplasia. While the pathogenesis of weathering nodules remains unclear, there is an association with age and chronic UV exposure.

Summary Aims To study sun-protective behaviors among skiers and snowboarders on the South Island of New Zealand and to identify associations with personal characteristics or weather conditions. Methods Two hundred twenty-six skiers and snowboarders completed an interviewer-administered questionnaire during September and October 2002. Reported behaviors were used to derive a composite sun-protection index, which was used to divide the sample into “protected” and “unprotected” groups. Odds ratios of being unprotected were calculated by logistic regression. Results Forty-eight percent (95% CI 42–54%) of interviewees recalled being sunburned while skiing or snowboarding in the past. Sixty-eight percent (95% CI 62–74%) were unaware of any educational messages specific to sun protection while skiing or snowboarding. Women were more likely to be protected from the sun (OR 0.47; 95% CI 0.27–0.81). Having a skin type resistant to burning (OR 1.93; 95% CI 0.92–4.06) and reported awareness of education messages (OR 1.66; 95% CI 0.92–2.99) were associated with not using sun protection. Conclusions Sunburn is common and sun protection not used by all. Men are less likely to report use of sun-protection measures. There is no evidence from this study that current strategies are effective in promoting skin protection while skiing or snowboarding.

The term orofacial granulomatosis was introduced for orofacial lesions which resemble Crohn's disease clinically and histologically in patients without gastrointestinal disease. 1 The Melkersson-Rosenthal syndrome and its oligosymptomatic form Meischers granulomatous cheilitis are usually felt to be part of the same spectrum of disease. 2 Oedema usually involves tissues of the lower half of the face. 2 We describe a patient with histological changes consistent with those found in orofacial granulomatosis, but with isolated localized oedema of the right periorbital tissues. The patient also had syringomyelia which has not previously been described in association with orofacial granulomatosis.

Summary Background There is strong evidence that the incidence and prevalence of atopic diseases is increasing. However, estimates of the prevalence of atopic dermatitis (AD) have varied greatly in the U.K. and most parts of the developed world. Objectives The aim of the study was to estimate the prevalence and incidence of AD between the ages of 0 and 42 months in children born in the 1990s in a defined population in the U.K. Design We used data from the Avon Longitudinal Study of Pregnancy and Childhood (ALSPAC), a large population-based study in the U.K. that enrolled all pregnant mothers who were resident in Avon and had their delivery date falling between 1 April 1991 and 31 December 1992. Since then ALSPAC has collected a wide range of data from the newborns and their parents. Data reported here were collected at 6, 18, 30 and 42 months using parental reports in a postal questionnaire. Of the 14 009 children originally enrolled 8530 provided information on AD in each of the four follow-up questionnaires. We defined AD as a report of rash in at least two of the four questionnaires. Incidence risk was defined as the percentage of new cases of AD between follow-up questionnaires, out of the total number of children whose parents had not reported that they had AD by the time of the previous follow-up. Results Period prevalence of 21·0%, 25·6%, 23·2% and 19·9% were observed at ages 0–6, 6–18, 18–30 and 30–42 months, respectively. The corresponding incidence risks were 21·0%, 11·2% and 3·8%, at 0–6, 6–18 and 18–30 months, respectively. There were no gender differences in either the incidence or prevalence of the disease. Conclusions Results from this large, prospective study are consistent with recent reports of increased incidence and prevalence of AD. Health planners can use our estimates of incidence and prevalence to project the number of children likely to suffer from AD during infancy and early childhood, and thus to determine the human and financial resources required.

Various skin changes have been described in algodystrophy, but the association of hypertrichosis with this condition is poorly documented. We describe a patient who developed algodystrophy after a mild inflammatory arthritis, in association with a number of skin manifestations, including hypertrichosis. We suggest a mechanism to explain this particular association.

Summary The effectiveness of EMLA® cream in relieving the discomfort associated with percutaneous infiltration of lignocaine was assessed in a double-blind, placebo-controlled study. Patients undergoing minor skin surgery were divided into two groups, according to the number of lesions requiring surgery. The first group had a single lesion and were randomized to apply either EMLA or placebo cream 1 h prior to infiltration, whilst the second group received EMLA to one and placebo to the other of two lesions treated at the same clinic attendance. In neither group was there a clinically useful reduction of discomfort, probably due to inadequate dermal anaesthesia, and we would not, therefore, recommend the routine use of EMLA for this purpose. The most recent evidence suggests that a 2-h application rime may give more effective dermal anaesthesia, hut this would probably limit its use to occasional problematical cases.

We present a 14-year-old girl with self-induced areas of hypo- and hyperpigmenation on her forearm as a result of applying 10 blasts of an asthmatic aerosol inhaler directly to her skin. We emphasize the importance of being aware of potential danger associated with the common metered-dose inhaler when it is misused.

A 61-year-old woman presented with a 2-year history of intense itching of her palms and soles. The irritation was relieved only by plunging her hands and feet into cold water. She would awaken at least 4 times per night to rub her hands and feet for 15 minutes before falling asleep again. Her symptoms were more marked in the summer compared with the winter. Her medical history included chronic obstructive pulmonary disease, hiatal hernia, nasal polyps, and 3 previous deep vein thrombi. She took sulfalene tablets and ipratropium bromide, albuterol, and beclomethasone dipropionate aerosol inhalers during the winter for her bronchitis. On examination, her hands and feet appeared healthy. There were no color changes, dryness, or abnormal neurologic symptoms. Investigation showed that the results of a complete blood cell count, electrolyte levels, liver function tests, immunoglobulin profile, total IgE level, and autoantibody profile all were within normal limits. A biopsy of the palm showed a mild chronic inflammatory infiltrate of the upper dermis, mild acanthosis, and no spongiosis. These changes were nonspecific and consistent with rubbing of the skin. A psychological assessment by a clinical psychologist showed no evidence for a psychosomatic cause of her pruritus. The following treatments were tried for several months per treatment, one after another: 0.5% to 2% menthol in aqueous cream (BP cream, Hillcross Pharmaceuticals, Briercliff, England), 30% emulsifying ointment in purified water, various emollients, oral antihistamines, 10% crotamiton cream twice per day (Emla cream, Astra, Westboro, Mass), 2.5% lignocaine hydrochloride and 2.5% prilocaine hydrochloride cream twice per day, 0.05% clobetasol propionate cream under occlusion twice per day, oral doxepin hydrochloride, 75 mg/d, carbamazepine, 200 mg twice per day, and topical 0.07% capsaicin cream twice per day, all without beneficial effect. She also underwent a twice-weekly course of local UV-B phototherapy for 6 weeks and a twice-weekly course of UV-A phototherapy with topical psoralen paint for 10 weeks, but both of these treatments were ineffective. THERAPEUTIC CHALLENGE

We present a 3-year-old boy with scleroatrophic syndrome of Huriez, a rare autosomal dominant condition with only seven affected families worldwide. Although assumed to occur from birth, an evolving case has not previously been documented. Infants do not possess the focal areas of keratoderma on the palms or soles; these develop in adult life. Of particular interest is the high incidence of squamous cell carcinomas that arise from the scleroatrophic skin.

A 49-year-old woman presented with a 1-year-history of a widespread eruption which proved to be due to leukaemia cutis. Subsequently, she developed pancytopaenia and a bone marrow biopsy revealed refractory anaemia with excess blasts in transformation (RAEB-T) with a high monoblastic component. This transformed into acute myeloid leukaemia. Leukaemia cutis in this context is well described but in this patient it became manifest 1 year prior to referral to the dermatologist. When occurring with a myelodysplastic syndrome, leukaemia cutis often heralds malignant transformation to acute myeloid leukaemia. Prompt diagnosis in this situation may identify a group of high-risk patients with myelodysplastic syndrome for whom chemotherapy and allogenic bone marrow transplantation, rather than the more conventional approach of supportive treatment, could be a more appropriate management strategy.

Summary Polymyositis and dermatomyositis are idiopathic inflammatory myopathies characterized by subacute symmetrical weakness of proximal limb and trunk muscles. Dermatomyositis is distinguished from polymyositis by the presence of rash.1,2 We describe an adult patient with treatment-resistant childhood-type dermatomyositis who made a good response to high dose intravenous immunoglobulins. Additionally, there was evidence of panniculitis which is an unusual histopathological finding in dermatomyositis.

Summary Scleredema of Buschke is a rare disorder characterized by the development of areas of skin induration which usually resolve spontaneously. It is occasionally associated with a benign gammopathy, and rarely with myelomatosis. We describe B 60-year-old woman with extensive skin changes, who developed IgA myeloma. Unusually, her skin disease did not respond to conventional myeloma therapy. Death occurred as a consequence of the progressive skin disease.

The efficacy and safety of calcipotriol solution in the treatment of scalp psoriasis was compared with placebo (vehicle solution), in a multicentre double-blind, randomized, parallel-group study of 49 adult patients. Calcipotriol solution (50 micrograms/ml), or placebo, was applied twice daily over a 4-week period. At the end of the study period 60% of patients on calcipotriol showed clearance or marked improvement of their psoriasis compared with 17% on placebo. Overall assessment of treatment response showed that calcipotriol was superior to placebo in both investigator (P < 0.001; 95% confidence interval for difference 19.0-67.6) and patient (P < 0.001; 95% confidence interval for difference 18.3-68.0) assessments. Total sign score for psoriasis (i.e. the sum of the scores for redness, thickness and scaliness) decreased by 48.9% in the calcipotriol group, and by 18.6% in the placebo group (P = 0.005). Calcipotriol was significantly superior to placebo in reducing redness, thickness, scaliness and extent of psoriasis, and in the patients' assessment in reducing scalp flaking and itching. No statistically significant changes in blood biochemistry were detected during the study, and the solution was generally well tolerated.

Objective: To assess the clinical outcomes of 45 cases of harlequin ichthyosis and review the underlying ABCA12 gene mutations in these patients. Design: Multicenter, retrospective, questionnaire-based survey. Setting: Dermatology research institute. Participants: Patients with harlequin ichthyosis for whom we had performed ABCA12 mutation analysis. Main Outcome Measures: Referring physicians were asked to complete a questionnaire using the patients' notes, detailing the clinical outcome of the affected child. In each case, the causative ABCA12 mutation was identified using standard polymerase chain reaction and sequencing techniques. Results: Of the 45 cases, the ages of the survivors ranged from 10 months to 25 years, with an overall survival rate of 56%. Death usually occurred in the first 3 months and was attributed to sepsis and/or respiratory failure in 75% of cases. The early introduction of oral retinoids may improve survival, since 83% of those treated survived, whereas 76% who were not given retinoids died. Recurrent skin infections in infancy affected one-third of patients. Problems maintaining weight affected 44%. Three children developed an inflammatory arthritis, and developmental delay was reported in 32%. Mutation analysis revealed that 52% of survivors had compound heterozygous mutations, whereas all deaths were associated with homozygous mutations. Conclusions: Harlequin ichthyosis should be regarded as a severe chronic disease that is not invariably fatal. With improved neonatal care and probably the early introduction of oral retinoids, the number of survivors is increasing. Compound heterozygotes appear to have a survival advantage.

Palmar fasciitis and polyarthritis syndrome is a rare, disabling, paraneoplastic condition of unknown pathogenesis. There is no known effective treatment, although the condition may be halted by control of the cancer. Previously reported cases have mostly been in patients with advanced ovarian malignancies. We present the case of a 69-year-old woman with this condition in association with bladder carcinoma, together with a review of the literature and discussion of possible therapeutic options.

Necrobiosis lipoidica (NL) has distinctive clinical and histological appearances. The legs are the commonest site of involvement, and 85% of cases involve the legs exclusively. We describe a patient in whom NL did not involve the legs, but was found to occur on unusual areas: the back and nipple. This is possibly the first report of NL affecting the nipple.

Vulvitis chronica plasmacellularis (Zoon's vulvitis) is rare. We describe a case in whom all the typical features, as delineated by Zoon, are demonstrated.

A 26-year-old woman developed partial lipoatrophy 12 years after juvenile dermatomyositis was diagnosed. Renal function was normal, but she had other features typically associated with partial lipoatrophy, including hepatomegaly, acanthosis nigricans, hypertrichosis, and hyperinsulinemia.

We present a case of eruptive collagenomas of the skin in a young girl. The typical clinical features and histology of this rare condition are described. The differential diagnosis and other types of cutaneous collagenomas are briefly discussed. A biopsy is required to confirm the excessive mature collagen which predominates in these lesions.