Itching for progressRees, J.; Murray, C. S.
doi: 10.1111/j.1365-2230.2005.01852.xpmid: 16045669
SummaryThe study of itch remains a neglected field, with a number of barriers limiting satisfactory therapy in the majority of instances. We review recent advances, focusing on the identification of the neural pathways, distinct from pain, that signal itch; methods to measure itch as scratch in humans; and the identification of a role for H4 receptors in mediating itch (in the mouse).
Incontinentia pigmenti case series: clinical spectrum of incontinentia pigmenti in 53 female patients and their relativesPhan, T. A.; Wargon, O.; Turner, A. M.
doi: 10.1111/j.1365-2230.2005.01848.xpmid: 16045670
SummaryA retrospective case series of 53 female patients with incontinentia pigmenti (IP) including 28 secondary cases (female relatives of probands) was reviewed and compared with other series in an attempt to estimate more accurately the true disease burden of patients with IP. We found that, while the frequency of the first three cutaneous stages of IP was comparable with previous studies, none of the secondary cases manifested any serious neurological complications but all displayed stage IV pale anhidrotic reticulate lines on their posterior calves. This important clinical feature of IP in secondary cases has previously been under‐represented in studies that often involved only paediatric probands. Hence, mildly affected cases of IP are often undiagnosed and under‐represented in case series to date, possibly leading to inappropriately high estimates of neurological and eye involvement. With the availability of genetic testing, it is now feasible to confirm the variability of the phenotype and the risk of complications in IP.
Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativaShort, K. A.; Kalu, G.; Mortimer, P. S.; Higgins, E. M.
doi: 10.1111/j.1365-2230.2005.01875.xpmid: 16045671
SummaryWe report a case of vulval squamous cell carcinoma (SCC) arising in chronic hidradenitis suppurativa (HS). The patient had a complex medical history including a 25‐year‐history of Crohn's disease. In addition she had recently received immunosuppressive therapy for nephrotic syndrome secondary to membranous glomerulonephritis. A painful nodule was noted on the vulva that was clinically very suspicious of SCC. An excision biopsy confirmed the diagnosis. There are few publications in the English literature citing association between HS and the development of SCC. The first report in the English literature of vulval SCC arising in chronic HS was published in 1999. We wish to draw attention to the possibility that patients with HS may develop SCC in lesional skin. A painful lump or ulcer could easily be mistaken for an inflammatory lesion and a low threshold for biopsy is warranted. We suggest constant vigilance with regard to malignant change in ano‐genital HS as the diagnosis can be difficult.
Cutaneous calcification following liver transplantationLateo, S.; Charlton, F.; Hudson, M.; Lawrence, C. M.
doi: 10.1111/j.1365-2230.2005.01793.xpmid: 16045672
SummaryEctopic calcification following liver transplantation has been reported to occur in various internal organs but there have been few reports of skin involvement. The pathogenesis is uncertain with previous reports suggesting that the calcifications could be either dystrophic or metastatic. The large amount of intravenous calcium needed to correct hypocalcaemia secondary to blood product transfusion is thought to play a central role. We report a case of calcinosis cutis developing after liver transplantation in a 22‐year‐old woman at sites where no intravenous calcium had been administered. In previously published cases serum calcium and phosphate levels were reported as normal. In our case serum calcium levels were also within or below normal limits with the exception of a transient rise in the immediate post‐operative period. Our case supports earlier hypotheses that short‐lived and often undetected elevations in the calcium‐phosphate product are implicated in this condition.
Antibiotic prophylaxis in patients with valvular heart defects undergoing dermatological surgery remains a confusing issue despite apparently clear guidelinesAffleck, A. G.; Birnie, A. J.; Gee, T. M.; Gee, B. C.
doi: 10.1111/j.1365-2230.2005.01815.xpmid: 16045673
SummaryThe guidelines pertaining to prescription of prophylactic antibiotics to prevent endocarditis during dermatological surgery appear clear and well‐documented. The British Society for Dermatological Surgery, in agreement with the British Society for Antimicrobial Chemotherapy, state that antibiotic prophylaxis for endocarditis is not required for routine dermatological surgery procedures even in the presence of a pre‐existing heart lesion. Pre‐existing cardiac lesions include prosthetic valves, history of bacterial endocarditis, congenital cardiac malformation, rheumatic or other acquired valvular dysfunction, hypertrophic cardiomyopathy or mitral valve prolapse with regurgitation. It is important to distinguish between antibiotic prophylaxis for wound infection and that for bacterial endocarditis. Routine procedures, such as punches, shaves, curettage and simple excisions, performed on clean intact skin have an extremely low risk of wound infection (1–4%). The risk of wound infection increases to 5–15% with clean‐contaminated skin surgery that includes procedures involving eroded or ulcerated skin, respiratory or buccal mucosa, flexural areas and protracted procedures such as Mohs' micrographic surgery. In such cases, antibiotic prophylaxis may be considered in patients with a cardiac lesion because a wound infection may result in bacteraemia and subsequent endocarditis. This should therefore not be considered ‘routine’ dermatological surgery. In contaminated, dirty and/or infected classes of wounds the risk of wound infection is higher (> 25%). Elective skin surgery should be postponed if possible until the wound infection is treated with therapeutic antibiotics.
Acinic cell carcinoma on the lower lip resembling a mucoceleCho, J. H.; Yoon, S. Y.; Bae, E. Y.; Lee, C. N.; Lee, J. D.; Cho, S. H.
doi: 10.1111/j.1365-2230.2005.01814.xpmid: 16045674
SummaryA 64‐year‐old woman presented with a 2‐week history of an asymptomatic mass involving the lower lip. Histopathological examination showed a well‐circumscribed tumour composed of many lobules separated by thin, fibrous connective tissue. Individual lobules were composed of round or polyhedral tumour cells, which had a characteristic finely granular and vacuolated cytoplasm and eccentric hyperchromatic nuclei. Positive staining was observed with Periodic acid–Shiff, and immunohistochemistry for cytokeratin, α‐1 antitrypsin, and S‐100 protein resulting in a final diagnosis of acinic cell carcinoma. Acinic cell carcinoma represents a well‐established, although uncommon, entity in the classification of neoplasms of salivary gland origin. The parotid salivary gland is the most frequent site of acinic cell carcinoma, whereas the lip is a particularly unusual site. The unusual presentation of this tumour may lead to confusion with a mucocele. Given these findings, we suggest that acinic cell carcinoma should be considered in the differential diagnosis of any mucocele‐like mass on the lower lip.
Dyschromatosis universalis hereditaria: two cases in a Chinese familyWang, G.; Li, C.‐Y.; Gao, T.‐W.; Liu, Y.‐F.
doi: 10.1111/j.1365-2230.2005.01823.xpmid: 16045675
SummaryTwo cases of dyschromatosis universalis hereditaria (DUH) from a Chinese family are presented. Case 1 was a 62‐year‐old woman who had a generalized and progressive hyper‐ and hypopigmentation of the skin from the age of 8 years. Her brother had also developed a similar skin pigmentary defect from about the same age. Histopathological and ultrastructural examination of lesional skin showed increased melanin content in epidermal keratinocytes but no changes in the appearance or number of melanocytes. Although hereditary defects may influence melanogenesis resulting in a pigmentary anomaly, the pathogenesis of DUH remains unclear.
Two cases of atypical membranous aplasia cutis with hair collar sign: one with dermal melanocytosis, and the other with naevus flammeusFujita, Y.; Yokota, K.; Akiyama, M.; Machino, S.; Inokuma, D.; Arita, K.; Kimura, C.; Shimizu, H.
doi: 10.1111/j.1365-2230.2005.01821.xpmid: 16045676
SummaryWe report two atypical cases of membranous aplasia cutis surrounded by a rim of hairs, one case associated with dense dermal melanocytosis and the other with naevus flammeus, with characteristic clinical features. A rim of hypertrichosis, ‘hair collar’ sign, is proposed to have a close association with neuroectodermal defects. A failure of the normal closure of the cranial neural tube might have affected foetal skin development, including melanoblast migration and capillary network formation. The changes in the present cases, as well as the hair collar sign may suggest a complex hamartomatous nature of membranous aplasia cutis.
Persistent severe amiodarone‐induced photosensitivityYones, S. S.; O'Donoghue, N. B.; Palmer, R. A.; Menagé, H. du p.; Hawk, J. L. M.
doi: 10.1111/j.1365-2230.2005.01820.xpmid: 16045677
SummaryAmiodarone, a benzofuran derivative, has been used therapeutically as an antiarrhythmic and coronary vasodilator in Europe since 1964. One of its commoner side effects is cutaneous photosensitivity; more rarely, after ingestion of the drug for around 12 months, a slate‐grey or violaceous discoloration of sun‐exposed sites may gradually develop. Both of these side effects usually resolve within 2 years of discontinuation of the drug. We now present a woman who developed both photosensitivity and a slate‐grey discoloration whilst taking amiodarone; on discontinuation of the drug, the dyspigmentation gradually resolved, but the photosensitivity has persisted and the patient remains symptomatic more than 17 years later.
A case of bullous pemphigoid associated with autoantibodies targeting antigenic sites other than the NC16a domain of BP180Shinojima, Y.; Ochiai, T.; Kawamura, A.; Arakawa, H.; Fukuda, K.; Hashimoto, T.
doi: 10.1111/j.1365-2230.2005.01819.xpmid: 16045678
SummaryBullous pemphigoid (BP) is associated with autoantibodies to the 180‐kDa BP antigen (BP180), and the antigenic site exists on noncollagenous 16a (NC16a) domain of BP180. We now report a male BP patient whose IgG autoantibodies did not react against the NC16a domain of BP180 by either immunoblotting or ELISA, whereas they did react with BP180 extracted from normal human keratinocytes. Anti‐BP180 cicatricial pemphigoid was ruled out due to the lack of conjunctival mucosal involvement and the absence of scarring in the oral cavity. Our findings indicate that there is an antigenic reactive region other than NC16a on the extracellular domain of BP180. There have been few reports describing detailed clinical features of BP caused by autoantibodies targeting antigenic sites other than the NC16a domain. We conclude that it is difficult to differentiate their clinical features from those associated with autoantibodies targeting the NC16a domain of BP180.