Madarosis.

Madarosis may be a presenting feature of a number of vision and life-threatening conditions, including herpes zoster, leprosy, HIV/AIDS, trachoma, malignant eyelid tumors, discoid lupus, scleroderma, and hypothyroidism. It may occur via two broad pathogenic pathways: scarring and non-scarring, which indicates the potential for lash re-growth. Madarosis may occur as an isolated finding or together with loss of other body and scalp hair. The etiology of madarosis can be further divided into dermatological, infection, endocrine, neoplastic, drug-related, congenital, and trauma. This report includes salient points in the clinical history and examination of patients with madarosis, with an emphasis on excluding or diagnosing visual or life threatening disorders associated with madarosis.

Linear basal cell carcinoma: A distinct clinical entity.

The purpose of the study is to describe linear basal cell carcinoma (BCC) as a distinct clinical entity, and highlight its existence to the plastic surgery literature. A Medline and PubMed literature search was conducted, and 33 reported cases of linear BCC were analysed. Of these 33 cases, the most common site for linear BCC was the periocular region, accounting for 49% (n= 16). The most common histologic subtype, was nodular BCC, accounting for 50% (n= 17). Of the 33 reported cases the postoperative defect size was mentioned in five cases only. None of these would have been completely excised if a 2 mm margin was applied, and only one out of five if a 4 mm margin was applied. Linear BCC is a distinct clinical entity. Presence of the tumour along relaxing skin tension lines, increase in subclinical extension, and aggressive tumour behavior are reported observations. Because of these observations it is suggested that margin-controlled excision should be considered for linear BCC.

Cutaneous lip tumours treated with Mohs micrographic surgery: clinical features and surgical outcome.

BACKGROUND: The Australian Mohs micrographic surgery (MMS) database was initiated in 1993 by the Skin and Cancer Foundation Australia (SCFA) with the aim of collecting prospective data, and involved all Mohs surgeons in the country. OBJECTIVES: To present a large series of patients with cutaneous lip tumours treated with MMS in Australia between 1993 and 2002. METHODS: This prospective multicentre case series included all patients with cutaneous lip tumours who were monitored by the SCFA. The main outcome measures were patient demographics, reason for referral, duration of tumour, site, preoperative tumour size and postoperative defect size, recurrences prior to MMS, histological subtypes, perineural invasion and 5-year recurrence after MMS. RESULTS: There were 581 patients (66.1% women and 33.9% men, P < 0.0001) with a mean +/- SD age of 58 +/- 15 years. The upper lip was the most common site involved (81.1%). Basal cell carcinoma (BCC) was diagnosed in 82.3%, squamous cell carcinoma (SCC) in 16.5%, Bowen's disease (BD) in 0.7% and microcystic adnexal carcinoma (MAC) in 0.5% of cases. BCC was more common on the upper lip and in women, whereas SCC was more common on the lower lip and in men (P < 0.0001). Most upper lip tumours occurred in women (75.4%), whereas most lower lip tumours occurred in men (73.6%). SCC was associated with a larger tumour and postoperative defect size compared with the other tumours. The 5-year recurrence for BCC was 3.0%, and there were no cases of recurrence for SCC, BD or MAC. CONCLUSIONS: BCC was the most common cutaneous lip tumour managed by MMS, and was significantly more common on the upper lip and in women. The low 5-year recurrence rate emphasizes the importance of margin-controlled excision.

Periocular keratoacanthoma: can we always rely on the clinical diagnosis?

AIM: To present a series of patients with a clinical diagnosis of periocular keratoacanthoma and assess the incidence of histologically proven invasive squamous cell carcinoma (SCC). METHODS: This retrospective case series included all patients with periocular tumours seen in the authors' unit between 1996 and 2004, and who were initially diagnosed with keratoacanthoma based on the clinical presentation. RESULTS: Twelve patients (eight males, four females) were clinically diagnosed with keratoacanthoma. The final histological diagnosis revealed two cases (16.7%) of invasive SCC, and 10 cases (83.3%) of keratoacanthoma. The lower lid was most commonly involved in cases of keratoacanthoma (50.0%). Six patients (60.0%) underwent Mohs surgery, and four (40.0%) were treated with excision under frozen section control. There were no cases of recurrence during a mean follow up period of 21 (SD 13) months. CONCLUSION: Although the clinical presentation of periocular keratoacanthoma is usually characteristic, a significant percentage of patients will prove to have invasive SCC. Complete excision with margin control offers a definitive diagnosis, as well as tissue conservation and a low recurrence rate.

Incidence of host site complications in periocular full thickness skin grafts.

AIM: To evaluate the complications of periocular full thickness skin grafts (FTSG) in patients treated with Mohs' micrographic surgery (MMS) for periocular malignancy. METHOD: This prospective, multicentre case series included all patients in Australia treated with MMS for periocular malignancy followed by reconstruction with FTSG, who were monitored by the Skin and Cancer Foundation, between 1993 and 1999. The parameters recorded were patient demographics, reason for referral, histological classification of malignancy and evidence of perineural invasion, duration of tumour, site, recurrences prior to MMS, preoperative tumour size, and postoperative defect size. FTSG donor sites included upper lid, preauricular, retroauricular, inner brachial, and supraclavicular. The primary outcome measures were FTSG host site complications (partial/complete graft failure, graft infection, acute bleeding/haematoma, graft hypertrophy, and graft contracture). RESULTS: 397 patients (229 males, 168 females), mean age 60 (SD 15) years (range 20-91 years). 92.7% were diagnosed with basal call carcinoma, 2.0% with Bowen's disease, and 3.3% with squamous cell carcinoma. Medial canthus was involved in 66.5% of patients, lower eyelid in 28.0%, and upper eyelid in 5.5%. Postoperative complications were recorded in 62 patients (15.6% of all patients), and consisted of graft hypertrophy (45.1% of complications), graft contraction (29.1%), and partial graft failure (12.9%). The only statistically significant association found was a higher rate of graft hypertrophy in medial canthal reconstruction (p = 0.007). CONCLUSION: Host site complications of periocular FTSG are not common. Graft hypertrophy accounted for most complications and was more common in the medial canthal area. No other variables such as patient demographics, tumour characteristics, or donor site factors were associated with a higher risk of complications.

Treatment of angiofibromas with a scanning carbon dioxide laser: a clinicopathologic study with long-term follow-up.

BACKGROUND: Facial angiofibromas in tuberous sclerosis have been managed with various treatment modalities, including carbon dioxide (CO(2)) laser resurfacing. OBJECTIVE: Our purpose was to perform a long-term clinicopathologic assessment of CO(2) laser treatment of angiofibromas. METHODS: This was a retrospective case review of 10 patients treated with a scanning CO(2) laser to flatten lesions. Baseline clinical photographs and those taken at 6, 12, and 24 months after the operation were assessed by a blinded observer. Patients also evaluated outcomes. Biopsy specimens taken immediately and at 4 months after the operation were reviewed. RESULTS: Three groups of patients were identified: the first comprised 2 patients with sustained excellent and good outcomes. A second group (3 patients) had excellent outcomes in the early and medium term but then demonstrated partial deterioration. The last group (5 patients) had a range of early results with invariably poor outcomes at 24 months. In contrast, patients' self-assessment at 24 months was good or excellent in 8 of 10 cases. All biopsy specimens taken immediately after the procedure demonstrated ablation extending into the papillary dermis. Residual angiofibromas were present in 6 biopsy specimens. At 4 months, all biopsy specimens showed a band of superficial dermal fibrosis, but distinguishing between this and adjacent angiofibromas was often difficult. Long-term side effects included 2 cases of subtle hypopigmentation. CONCLUSION: The long-term results of CO(2) laser treatment of angiofibromas are unpredictable. The marked improvement obtained at 6 months is sustained in only a minority of cases at 24 months. Despite this, patient satisfaction appears relatively high. Initial clinical improvement may be the result of a combination of destruction of angiofibromas and their sequestration under postoperative fibrosis. The benefits of therapy should be weighed against both early morbidity and the risks of long-term complications such as scarring and hypopigmentation.

Scanned continuous wave CO2 laser resurfacing: a closer look at the different scanning modes.

BACKGROUND: The immediate effects of CO2 laser resurfacing include tissue ablation and residual thermal damage. These laser-tissue interactions are shaped by parameters including fluence, dwell time, and number of passes. OBJECTIVE: To assess the vaporization depth and residual thermal damage following use of the "superficial" or "deep" scanning modes of a 40 W continuous wave CO2 laser using both frozen and paraffin sections. METHODS: Fourteen subjects were divided into two groups for test treatments in the right preauricular area with two passes of the laser. The "superficial" mode parameters were 10 mm2 scan, 200 mm lens, power 36 W, scan time 0.24 seconds, dwell time 0.22 msec, and fluence 5.5 J/cm2. The "deep" mode settings were 9 mm2 scan, 200 mm lens, power 18 W, scan time 0.64 seconds, dwell time 0.28 msec, and fluence 7.0 J/cm2. The deep mode has a greater pattern density than the superficial mode and also has a double pattern of exposure. Biopsies encompassing equal areas of treated and untreated skin were taken immediately postoperatively and processed with both frozen and paraffin-embedded techniques. RESULTS: Vaporization depth was similar in both scanning modes and by both tissue-processing techniques. On frozen sections, residual thermal damage was 20% greater in the deep mode than the superficial mode, but this was not a statistically significant difference. There was no significant difference between the two modes in the depth of thermal injury on paraffin sections. CONCLUSION: In keeping with theoretical expectations, vaporization depth was similar in both treatment groups. No significant difference in residual thermal damage could be demonstrated between the two modes. However, the results on frozen sections suggest that residual thermal damage may be greater in the deep mode than in the superficial mode. In addition, frozen sections may be more sensitive than paraffin sections in the detection of residual thermal damage following laser resurfacing.

Unplanned incomplete Mohs micrographic surgery.

BACKGROUND: Incomplete Mohs micrographic surgery (MMS) is the cessation of MMS while the tumor margins are known to be still positive. OBJECTIVE: Our purpose was to examine the factors behind unplanned incomplete MMS and to identify means of avoiding and managing this situation when it arises. METHODS: We performed a retrospective case review examining clinical presentations, histologic features, and management of incomplete MMS. RESULTS: Fifteen of 10,346 procedures (0.15%) were identified as incomplete MMS. Complete records were available in 14 cases. The age range was 30 to 90 years; the study comprised 10 men and 4 women. The tumors included 9 basal cell and 4 squamous cell carcinomas and 1 dermatofibrosarcoma protuberans. The sites involved were nose, medial canthus, ear, scalp, and lower eyelid. Twelve cases dealt with unresectable disease, whereas two patients were unable to tolerate further surgery. Of the unresectable cases, MMS was terminated because of ongoing multifocal positive skin margins, bony invasion, or extension of tumor to other locations. Surgical defects were repaired, whereas residual disease was managed with a variety of methods. CONCLUSION: Incomplete MMS is a rare problem of either unresectable disease or inability of the patient to tolerate the procedure. Preoperative planning may help to identify both subgroups of patients. A multispecialty approach to managing these cases is often necessary.

Generalised granuloma annulare successfully treated with PUVA.

Disseminated granuloma annulare is an uncommon disorder in which both topical and systemic therapy may have limited success. Anecdotal reports have suggested that PUVA may result in complete clearance of disease; however, maintenance PUVA therapy has usually been required in order to maintain remission. We report the successful treatment of a patient with 5-methoxypsoralen over a 7-month period who remained in remission during a 20-month follow up period.

Malar fat pad elevation: An aid to closure.

BACKGROUND: In closure of defects inferior to the eye, it is important to avoid inducing lower lid ectropion. OBJECTIVE: To describe a new technique for closure of defects inferior and lateral to the eye. METHODS: A case of malar fat pad elevation to close a post-Mohs surgical defect is described and the procedure is detailed. RESULTS: This technique enabled good wound closure with minimal tension and avoided the complication of ectropion. A transient asymmetry was manifest postoperatively. CONCLUSION: Malar fat pad elevation is an effective and elegant means of closing defects inferior and lateral to the eye. This technique minimizes extensive tissue undermining and movement with its consequences of postoperative tissue swelling, bruising, and hematoma formation. The side effect of ectropion is prevented.

Raising eyebrows with botulinum toxin.

BACKGROUND: Brow elevation rejuvenates the facial appearance. OBJECTIVE: To determine if a significant degree of brow elevation could be achieved through selective botulinum toxin treatment of brow depressors. METHODS: Seven women aged 31-42 (mean 37) years old were treated. The distance from lowest eyebrow cilium of the eyebrow to the midpupillary point was measured before and 1 month posttreatment. Botulinum toxin was injected into the glabellar area (7-10U) and the supralateral eyebrow (0-2.5U each side), to a total dose of 10-14 U. RESULTS: Five individuals (71%) showed brow elevation of 1-3 mm with a mean elevation of 1 mm. Two individuals showed no change. Concurrent weakening of the frown response was noted in all patients. CONCLUSION: Botulinum toxin treatment of brow depressors produces a small degree of brow elevation in the majority of patients.

Fatal cytophagic panniculitis and haemophagocytic syndrome.

We present two fatal cases of cytophagic panniculitis (CP) and haemophagocytic syndrome (HPS). In the first of these, there was an underlying T-cell lymphoma but in the second no associated disease was found. HPS is a frequently fatal disorder of immune regulation, characterized by fever, histiocytic haemophagocytosis, hepatosplenomegaly, pancytopenia, hypertriglyceridaemia and coagulopathy; CP is a less common manifestation. A number of benign and malignant conditions may present with HPS, the clinical findings and investigations aiding in determining an underlying disorder. Therapy is both supportive and directed at any associated illness, but often very difficult as diagnosis is delayed.

Cytokeratins in primary cutaneous amyloidosis.

The expression of keratins was investigated immunohistochemically on formalin-fixed and snap-frozen primary cutaneous amyloidosis tissue with a panel of monospecific and polyspecific antikeratin antibodies, with recognized keratins K1, K5, K6, K7, K8, K10, K14, K16, K17, K18 and K19. Amyloid deposits in frozen sections of seven cases of macular amyloidosis and lichen amyloidosus always reacted with antibodies LP34 (labelling K5, K6 and K18), MNF 116 (labelling K5, K6, K8, K10, K17 and K18), and RCK 102 (labelling K5 and K8); frozen sections in one case each of the seven cases also reacted with antibodies LL001 (labelling K14), LP1K (labelling K7 and K17), and LP2K (labelling K19). In formalin-fixed sections of 13 cases of macular amyloidosis and lichen amyloidosus, amyloid deposits were labelled with LP34 in three sections, MNF 116 in four sections, LL020 (labelling keratins K5 and K6) in one section, and LP2K in two sections. In nodular primary cutaneous amyloidosis, amyloid deposits were not labelled with any antikeratin antibodies. These data confirm that amyloid in macular amyloidosis and lichen amyloidosus contains keratin epitopes, and suggests derivation of the fibrillar component from keratin intermediate filaments. Several different keratins appear to undergo conversion to amyloid. LP34, MNF 116 and RCK 102 antibodies, which have in common the labelling of keratin K5, may be useful in the diagnosis of macular and papular amyloidosis with frozen tissue sections.

Failure of pulsed dye laser therapy for resistant verrucae.

Treatment with the 585-nm pulsed dye laser was performed in seven patients with viral warts (six with plantar warts and one with a periungual wart) who had failed to respond to conventional therapeutic modalities, including keratolytics, formalin soaks, cryotherapy, curettage, CO2 laser and systemic retinoids. Each patient was and treated on six occasions, separated by intervals of 3 weeks, with fluences of 8.5-9.5 J/cm2. Final follow-up was performed 3 weeks after the final treatment. All patients showed some decrease in the thickness of their warts, this being of a small degree in five of the patients. In the remaining two, there was a moderate reduction in both thickness and wart size. None of the patients experienced complete resolution of their lesions. symptomatic improvement was obtained in all three patients who had previously complained of pain. These data indicate that treatment of resistant viral verrucae of the periungual and plantar skin with the pulsed dye laser is not curative although partial resolution and symptomatic improvement may occur.

Management of the immunobullous disorders. II. Pemphigus.

In the second of our reviews on the management of the immunobullous disorders, we review the therapy of pemphigus disorders, including pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, drug-induced pemphigus, IgA pemphigus and paraneoplastic pemphigus.

Generalized granuloma annulare sparing vaccination sites.

Generalized granuloma annulare (GA) is a chronic and widespread form of GA, less common than the localized type, in which the primary papular lesions may be arranged in either predominantly annular or non-annular configurations. Associations with numerous systemic disorders, including malignancies of the haematopoietic system, have been reported. There is a marked tendency to show the isomorphic or Koebner phenomenon. We report a most unusual case of generalized non-annular GA associated with chronic myelomonocytic leukaemia and myelodysplasia, in which the eruption showed striking sparing of vaccination sites.

Immunofluorescence of the immunobullous disorders part one : Methodology.

In the past two decades the immunofluorescence techniques have greatly contributed to the diagnosis, treatment and understanding of the immunobullous disorders. The methodology of direct and indirect immunofluorescence as applied to the immunobullous disorders is described in detail.

Immunofluorescence of the immunobullous disorders Part two: The clinical disorders.

The immunofluorescence findings in the immunobullous disorders are reviewed together with a summary of clinical and histopathological findings.