Neil J. Mortimer

A successful therapeutic trial of rituximab in the treatment of a patient with recalcitrant, high-titre epidermolysis bullosa acquisita

hands of manual workers. Discrimination between occupational dermatitis and MH is difficult; however, lesions appear well demarcated with interface dermatitis in the latter. Therefore, the diagnosis of MH should be considered in patients especially when they present with general signs, interstitial lung disease and/or inflammatory muscle disease and/or polyarthritis requiring a search for anti-Jo-1 antibodies. A close association has been shown between MH and anti-Jo-1 antibodies, but MH has also been reported in polymyositis, dermatomyositis, childhood sclerodermatomyositis and mixed connective disease. In two of seven patients, MH occurred in the course of known idiopathic inflammatory myopathy in whom the skin was not involved initially. In these cases, systemic involvement developed concomitantly or soon after. A skin flare was therefore always associated with deep tissue flare; such an event in patients with antisynthetase syndrome implies deep organ assessment. The patients with MH became clear of their lesions together with clearance of the deep tissue disease. When MH was associated with idiopathic inflammatory myopathy in our series, it never appeared as an isolated skin sign. This suggests that when MH is present in a patient free of systemic signs, the diagnosis cannot be antisynthetase syndrome; the hand lesions belong rather to a classical occupational dermatitis and a search for anti-Jo-1 antibodies need not be done in this situation. Prognosis of the antisynthetase syndrome is poor as its mortality rate is 40%, with 62% in patients with interstitial lung disease, but could be improved by an early diagnosis. Therefore, in view of the frequent occurrence of this sign in antisynthetase syndrome, and the importance of early management, careful examination of the hands is mandatory in patients with systemic signs such as interstitial lung disease, myositis and arthritis.

Full‐thickness skin grafts for surgical defects of the nasal ala – a comprehensive review, approach and outcomes of 186 cases over 9 years

Skin cancers of the nasal ala are commonly encountered in dermatological surgery. Various techniques have been described to reconstruct surgical defects of the ala, including melolabial transposition flaps, interpolated melolabial flaps, bilobed and trilobed transposition flaps, island pedicle flaps and spiral flaps. Full‐thickness skin grafts have received little attention in the reconstructive literature.

Muscular hinge flaps: utility and technique in facial reconstructive surgery.

&NA; The authors have indicated no significant interest with commercial supporters.

Sclerosing Squamous Cell Carcinoma of the Skin, An Underemphasized Locally Aggressive Variant: A 20-Year Experience

BACKGROUND Desmoplastic (sclerosing) responses to a variety of neoplasms have been documented but rarely evaluated in association with primary cutaneous squamous cell carcinoma (SCC). We report a distinctive variant of SCC demonstrating an infiltrative growth pattern and stromal desmoplasia. METHODS Cases were identified through a retrospective review of our dermatopathology and dermatologic surgery databases. After initiation of the study, additional cases were identified prospectively. Neoplasms were scored microscopically for specific histopathologic parameters and reactivity with selected histochemical and immunohistochemical stains. Clinical follow‐up data were obtained through a review of medical records or contact with the patients referring physicians. RESULTS Seventy‐three carcinomas from 72 patients were identified (46 men, 26 women; median age 76, range 45–91). The original pretreatment biopsies were available in 69 of 73 cases. All lesions developed on sun‐damaged skin, with the cheek constituting the most common site. The clinical presentation was typically as a sclerotic plaque. All neoplasms extended into the reticular dermis or subcutaneous fat, and perineural invasion was identified in 53 cases (73%). Patients who underwent standard excisional surgery experienced a recurrence rate of 80%; 9% of those treated with micrographic surgery experienced postoperative recurrences. Metastasis or carcinoma‐related death was not observed in any patient during the follow‐up period (median 36 months). CONCLUSIONS Our results suggest that desmoplasia is uncommonly found in association with cutaneous SCC but helps define a locally aggressive variant of carcinoma. In light of the infiltrative nature of desmoplastic SCC of the skin and the high incidence of perineural invasion, micrographic surgery is the surgical modality of choice. The authors have indicated no significant interest with commercial supporters.

The Nasal Sidewall Rotation Flap: A Workhorse Flap for Small Defects of the Distal Nose

BACKGROUND Skin cancers of the nasal tip present a challenge for the dermatologic surgeon. The bilobed flap has been widely used as the “workhorse” flap for such defects but requires meticulous design and may be complicated by a tendency toward pin‐cushioning. OBJECTIVE To describe the use of the nasal sidewall rotation (NSR) flap for reconstructing defects on the nasal tip. METHODS A retrospective analysis of the Mohs micrographic surgery database over a 4‐year period was performed. All cases in which the NSR flap was used were identified. Defect location and size and any postoperative complications were noted. All patients were reviewed at the time of suture removal and at 6 weeks. RESULTS There were 65 cases (19 men and 46 women). Age ranged from 39 to 86 (mean 60.5, median 59). Defect size varied from 0.4 to 2.0 cm in diameter, with 63% measuring 1.0 to 1.4 cm. Good to excellent results were seen in all patients, and postoperative complications were uncommon and minor. CONCLUSION The NSR flap is a versatile and useful alternative for reconstructing surgical defects of the nasal tip. The authors have indicated no significant interest with commercial supporters.

Staphylococcal scalded skin syndrome as a complication of septic arthritis.

1 Grover RW. Transient acantholytic dermatosis. Arch Dermatol 1970; 101: 426–34. 2 Davis MD, Dinneen AM, Landa N, Gibson LE. Grover’s disease: clinicopathologic review of 72 cases. Mayo Clin Proc 1999; 74: 229–34. 3 Zelickson BD, Tefferi A, Gertz MA et al. Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy. Acta Derm Venereol 1989; 69: 445–8. 4 French LE, Piletta PA, Etienne A et al. Incidence of transient acantholytic dermatosis (Grover’s disease) in a hospital setting. Dermatology 1999; 198: 410–11. 5 Economopoulos T, Papageorgiou S, Dimopoulos MA et al. Non-Hodgkin’s lymphomas in Greece according to the WHO classification of lymphoid neoplasms. A retrospective analysis of 810 cases. Acta Haematol 2005; 113: 97–103.

Optimizing Outcomes in Trilobed Flap Reconstruction for Nasal Tip Defects

We have found it most useful for the repair of medium-sized defects involving the very distal nasal tip, where the defect extends onto the soft triangle or infra-tip and columella area. Furthermore, we have, like the authors, found the flap of particular use in men with rhinophymatous change in whom the thick sebaceous nasal skin is particularly immobile and in whom a bilobed flap does not facilitate enough tissue movement to allow tension-free closure of the distal nasal tip.

Childhood sarcoidosis presenting with extensive cutaneous lesions, bilateral hilar lymphadenopathy and severe hypercalcaemia

A 9‐year‐old boy with a history of atopic dermatitis presented with a 4‐month history of an asymptomatic papular eruption. This was predominantly perioral in distribution with lesser involvement of the neck, arms and trunk. Investigations revealed severe hypercalcaemia of 3.77 mmol/L (normal range 2.10–2.60) and bilateral hilar lymphadenopathy. The diagnosis of sarcoidosis was supported by granulomata in skin and lymph node biopsies on histopathological examination. Prednisolone (2 mg/kg/day) rapidly normalized serum calcium.

The utility of the 'temporary marginal lid suture' in facilitating Mohs surgery for tumours involving the eyelid.

MADAM, The application of temporary sutures placed through the tarsus of the lower eyelid is well documented in the oculoplastic and dermatological surgery literature. They are generally used as suspension sutures (Frost sutures) to reduce the risk of ectropion due to wound contraction after reconstructive procedures involving the lower eyelid. The use of temporary sutures to facilitate Mohs surgical extirpation of eyelid tumours has not previously been described. Mohs surgical excision of skin cancers involving the eyelids presents unique challenges to the surgeon. The very thin, mobile nature of eyelid skin and the need to evert the lid to incise the conjunctiva for marginal lid tumours makes the incision of tissue difficult and the harvesting and orientation of an optimal Mohs surgery specimen technically demanding. Methods to facilitate stabilization of the tissue include the use of chalazion clamps, jaeger plates and manual stretching of the skin by the surgical assistant. We describe the use of a marginal eyelid suture to stabilize the eyelid, stretch the tissue and evert the lid when incising the conjunctiva, which in our experience is a useful tool to ensure highquality Mohs surgery specimens for frozen section tissue processing. After the periphery of the tumour has been marked and the area anaesthetized, a 6 ⁄0 monofilament suture of the surgeon’s choice is passed through the tarsal plate at the lid margin (Fig. 1a). In this particular example, the suture is passed through both the tumour and the tarsal plate. The needle is then removed and the suture left in place. During Mohs extirpation of the tumour, the surgical assistant controls the suture. Traction can be applied in such a way as to stabilize and stretch the eyelid skin and also to apply vertical traction when required (Figs 1b, 2a). We have found this technique useful for the first stage of Mohs micrographic surgery when treating marginal lid tumours of either the lower Table 2 The reported effects of stopping nicorandil Site Number of responses No improvement (%) Some improvement (%) Full improvement (%)

The use of dietary manipulation in patients referred to a contact dermatitis clinic

reflects the activation of peripheral T lymphocytes. In summary, to our knowledge, we are the first group to have shown a positive correlation between telomerase activity in PBMC and disease severity of psoriasis, but not duration. Telomerase activity in PBMC may serve as an objective indicator of psoriasis severity, and inhibiting telomerase activity may become a new target for psoriasis therapy.