Radiotherapy for parotid IgG4-related disease.

We describe the use of radiotherapy for parotid IgG4-related disease (IgG4-RD), initially misdiagnosed as Kimura's disease, with sustained good partial response in a 37-year-old male. To the best of our knowledge, this is the first reported case of radiation for extra-orbital IgG4-RD, albeit inadvertently.

Intravenous lobular capillary haemangioma (pyogenic granuloma): A case report and a review of imaging findings as reported in the literature.

INTRODUCTION: Intravenous lobular capillary haemangioma (IVLCH) is a rare benign lesion with minimal published reports containing a description of its appearance at medical imaging. We present the ultrasound and MRI images of a histologically proven IVLCH and provide the first review of the imaging findings reported in the literature. METHODS: The imaging findings of a case of IVLCH are presented. A PubMed search of English language articles was performed from 1966 to October 2016, and all relevant papers were reviewed. Imaging findings from those papers are summarised. RESULTS: Key features on ultrasound are of a well-defined, usually hypoechoic, mass which is highly vascular. The host vein is usually apparent. MRI imaging usually shows T1 signal isointense to muscle and variable T2 signal, either isointense or of high signal relative to the vein. The lesions enhance with gadolinium. CONCLUSION: Intravenous lobular capillary haemangioma has distinctive ultrasound but less consistent MRI features although radiological diagnosis should usually be possible. Review of reported cases shows that a previously described gender bias is incorrect.

Scalp Basal Cell Carcinoma: Review of 2,202 Cases.

BACKGROUND: Increases in the incidence of basal cell carcinoma (BCC) in women, younger age groups and in aggressive scalp subtypes in younger women have been reported. OBJECTIVE: To describe lesion and patient characteristics in scalp BCC. MATERIALS AND METHODS: Retrospective audit of scalp BCCs from 3 pathology laboratories in Adelaide, South Australia, January 2009-December 2013. RESULTS: Scalp BCC was 2.6% of all BCC. Of 2,202 patients with scalp BCC, 62% were male and 78% were >60 years. Histologic subtypes included nodular (55%), mixed (30%), and superficial (8%). The concordance between biopsy and excision was 83% for division into nonaggressive and aggressive subtypes. The incomplete excision rate was 16%. Aggressive subtypes were larger and had perineural invasion (PNI) in 8.5% and incomplete excision in 26%. CONCLUSION: Basal cell carcinoma on the scalp was less common. Men and the elderly had the majority of cases, with no predilection for women, including aggressive histologic subtypes in younger women. Aggressive subtypes were associated with increased size, incomplete excision, and PNI. A preliminary biopsy assisted division into aggressive and nonaggressive histologic subtypes. Incomplete excision rates were higher and increased in aggressive histologic subtypes and PNI. Mohs surgery or wider margins are suggested in these cases.

Acroangiodermatitis in a lower leg amputee related to suction socket use.

Acroangiodermatitis is a rare condition with numerous causes typically presenting as purple macules on the lower extremities. Although benign, it can mimic the presentation of more serious underlying conditions such as Kaposi's sarcoma. We present a case of acroangiodermatitis in the stump of an amputee related to suction socket use in order to raise awareness of an unusual setting for this vascular proliferation.

Treatment of actinic cheilitis with the Er:YAG laser.

BACKGROUND: Actinic cheilitis is a common condition with the potential to develop into squamous cell carcinoma. Current treatments have varying cure rates and complications. The role of the erbium:yttrium-aluminum-garnet (Er:YAG) laser in the treatment of actinic cheilitis has not been widely published, despite offering theoretical advantages over current treatment modalities. OBJECTIVE: To evaluate the outcome of a series of patients treated with the Er:YAG laser for actinic cheilitis. METHODS: This was a retrospective, interventional, nonrandomized, sequential case series set in a tertiary referral, dermatologic surgery unit. Ninety-nine consecutive patients with actinic cheilitis treated with the Er:YAG laser between January 2001 and June 2008 underwent a case note review, of which 77 went on to a structured telephone interview. The main outcome measures were a subjective improvement in lip symptoms related to actinic cheilitis and objective improvement in the lips at routine follow-up. RESULTS: Mean time to interview follow-up was 65.7 months. Of those interviewed, 92.2% believed there had been an improvement in the cosmetic appearance of their lips; one hundred percent believed the function of their lips had improved or remained unchanged; and 84.8% remained completely disease free at the time of follow-up. The majority of patients (93.5%) were satisfied with the laser treatment. Scarring as a direct result of the laser occurred in 5.1% of patients. LIMITATIONS: Retrospective nature of data collection; inability to interview all patients who underwent treatment. CONCLUSION: The Er:YAG laser is a successful modality for the treatment of actinic cheilitis with good functional and cosmetic results and only a small risk of long-term scarring. It should be considered as a first-line treatment for the disease.

Eccrine porocarcinoma of the upper eyelid.

Eccrine porocarcinoma is an unusual, locally aggressive tumor with a significant risk of metastasis and recurrence after surgical excision. Eyelid involvement is rare. We describe a 70-year-old man who was examined for right upper eyelid eccrine porocarcinoma that was treated with Mohs surgery. Eccrine porocarcinoma should be considered in the differential diagnosis of malignant eyelid tumors.

Metastatic Bowen carcinoma.

A 72-year-old man presented with basaloid squamous cell carcinoma metastatic to both lungs that was morphologically similar to recurrent, invasive moderately to poorly differentiated carcinoma arising from persistent Bowen's disease of the right cheek. The original lesion of invasive moderately differentiated squamous cell carcinoma arising in Bowen's disease had been excised 11 years previously. Mohs' surgery was performed for the recurrent lesion on the cheek 12 months prior to detection of lung metastases. He died of respiratory failure 19 months following diagnosis of his metastatic disease. Our case reflects the potentially aggressive nature of invasive squamous cell carcinoma arising from Bowen's disease and highlights the importance of approaching this entity with a view to complete surgical excision with adequate margins.

An unusual presentation of periocular Merkel cell carcinoma.

AIM: Merkel cell carcinoma (MCC) is an uncommon but potentially aggressive tumor. We describe a rare presentation of MMC of the upper lid. METHODS: A 63-year-old male presented with a 3-month history of right upper lid fullness. Examination revealed a large multinodular mass protruding into the conjunctival aspect of the eyelid. An enlarged right submandibular lymph node was also noted. RESULTS: An incisional biopsy was suggestive of MCC. Staged surgical excision with rapid paraffin sections was performed. The tumor cells were seen invading the tarsal plate and adjacent to the palpebral conjunctiva but not into the epidermis. MCC was confirmed using immunohistochemical stains and neuroendocrine markers (cytokeratins Cam 5.2, AE1/3, CK20, chromogranin, synaptophysin, and Leu 7). The lymph node biopsy was also confirmed as metastatic MCC. The patient was treated with postsurgical chemotherapy and radiotherapy, with no recurrence during a 6 months follow-up period. CONCLUSION: Although uncommon, periocular MCC may present as an atypical conjunctival multinodular mass.

Intraepithelial sebaceous carcinoma of the eyelid misdiagnosed as Bowen's disease.

BACKGROUND: Sebaceous carcinoma (SC) is well known for its ability to masquerade clinically and histologically as a variety of periocular conditions resulting in a delayed diagnosis. We present a series of periocular SC cases and discuss the difficulties in histopathological diagnosis when this tumor presents with a Bowenoid pattern of intraepithelial spread. METHODS: A retrospective case study of all patients with SC of the eyelid treated in our Hospital, from 1997 to 2004, was conducted. RESULTS: Eight patients were identified (four females and four males). Seven cases involved the upper eyelid. Initial clinical diagnoses included blepharitis (three cases), blepharoconjunctivitis (one case), cicatrizing conjunctivitis (one case), and lid lesions (two cases). Histopathologically, 87.5% of cases were misdiagnosed as Bowen's disease (BD) on the initial biopsy. Six of these cases showed no invasive disease on the initial biopsy and were eventually found to be invasive SC on subsequent excisions. In one case, the tumor was wholly in situ. Delay in diagnosis ranged from 0 to 56 months. CONCLUSIONS: SC should always be considered in the histological differential diagnosis of any eyelid lesion which resembles BD, particularly if the upper eyelid is involved or if multivacuolated cytoplasmic clear cell changes are seen.

Orbital eosinophilic angiocentric fibrosis case report and review of the literature.

OBJECTIVES: To report a patient with a rare case of orbital eosinophilic angiocentric fibrosis (EAF) and to review the literature. DESIGN: Interventional case report. METHODS: A 61-year-old man presented with a 6-week history of right periorbital edema and painless proptosis. Examination revealed a nonaxial proptosis, lateral globe displacement, and mild limitation in right eye adduction. MAIN OUTCOME MEASURES: Clinical course and radiological and histological findings. RESULTS: Orbital imaging revealed a right medial orbital mass with involvement of middle ethmoidal air cells. An orbital biopsy of the mass demonstrated an inflammatory infiltrate with a marked eosinophilic component, onion skinning of vessels, and surrounding fibrosis. The diagnosis of orbital EAF was made. There was no response to a 3-month treatment course with systemic steroids, but the patient did not want any further surgical interventions. CONCLUSION: Although orbital EAF is rare, ophthalmologists need to be aware of this entity, as it may invade the orbit from the sinonasal tract or present as a localized orbital mass. The presence of even minimal sinus involvement and the characteristic histopathology are useful in establishing the correct diagnosis.

Pyodermatitis-pyostomatitis vegetans of the eyelids case report and review of the literature.

OBJECTIVES: To present the first reported case of eyelid involvement in pyodermatitis-pyostomatitis vegetans (PDPSV) leading to the diagnosis of ulcerative colitis, and to review the literature. DESIGN: Interventional case report. METHODS: A 29-year-old man presented with a 4-week history of severe bilateral upper and lower eyelid margin ulceration and pustules unresponsive to topical and systemic broad-spectrum antibiotic treatment. Further questioning revealed the existence of skin and oral lesions. MAIN OUTCOME MEASURES: Clinical course, histological findings, and response to treatment. RESULTS: The histological and immunofluorescence studies were suggestive of PDPSV. Colonoscopy showed significant chronic active ulcerative colitis. Treatment with systemic steroids and sulfasalazine resulted in complete resolution of eyelid, oral, and skin lesions. However, stopping the steroids resulted in recurrence of eyelid and oral lesions and required recommencement of treatment. CONCLUSION: It is important to be familiar with this pustular skin condition, as correct diagnosis may lead to the diagnosis of inflammatory bowel disease. Although periocular involvement is probably rare, the combined typical skin and oral lesions and the characteristic histological and immunofluorescence tests should suggest the correct diagnosis.

Malignant fibrous histiocytoma of the eyelid: differential diagnosis and management.

PURPOSE: Malignant fibrous histiocytoma (MFH) is a pleomorphic soft tissue sarcoma that occurs rarely in the periocular region. The purpose of this study was to present a case series of periocular MFH and to discuss the differential diagnosis and management. METHODS: This is a retrospective case review of patients diagnosed with periocular MFH from tertiary hospitals and private practices. RESULTS: Four patients, two women and two men with periocular MFH, had a mean age of 81 years (range, 72 to 85 years). All tumors were <5 cm in diameter and of storiform pleomorphic histologic subtype. One was located deep and 3 presented superficially. The initial diagnoses were sarcomatoid carcinoma, atypical fibroxanthoma, and leiomyosarcoma that were subsequently reclassified as MFH. One patient had a frozen section, 1 had fast track paraffin section margin control, and 2 had no frozen section margins at the initial excisions. Histology showed 2 negative margins, 1 close margin, and 1 positive margin that were cleared with wide local excision. Local recurrence occurred in 3 cases, and of these, 1 required orbital exenteration. Adjuvant radiotherapy was given to 3 patients. One had regional node metastases. There were no distant metastases or tumor-related deaths. Mean follow-up period was 36 +/- 35 months (median, 30; range, 1 to 84 months). CONCLUSIONS: This study highlights the difficulties in the clinicopathologic diagnosis of periocular MFH and in particular the distinction of more superficial tumors from atypical fibroxanthoma. The mainstay of treatment is complete surgical excision with wide margins, and consideration should be given to histologic margin control in addition to adjuvant radiotherapy.

The Australian Mohs database: periocular squamous intraepidermal carcinoma.

OBJECTIVE: To present the findings and report the outcomes of all patients with periocular squamous intraepidermal carcinoma (IEC) treated with Mohs micrographic surgery (MMS) in Australia between 1993 and 1999. DESIGN: Prospective, noncomparative, multicenter, interventional case series. METHODS: A prospective series of 53 patients undergoing MMS for periocular IEC over a 7-year period (1993-1999). MAIN OUTCOME MEASURES: Recurrence, site, size of IEC, prior recurrence, and size of final defect. RESULTS: Fifty-three patients had 27 (51%) lower eyelid, 17 (32%) medial canthus, and 9 (17%) upper eyelid IEC. Most (85%) of the IECs were present for less than 5 years. Fifty-eight percent were primary IECs, and 42% were recurrent IECs. There were no differences in the clinical features of primary and recurrent IECs. Significant subclinical tumor extension was found, whereby the defect exceeded tumor size by at least 2 size groups (> or =2 cm) in 25% of cases. Follow-up to date was available in 68% (36 of 53) of cases (19 primary and 17 recurrent IECs). With a mean follow-up of 77.4 months (median, 75; range, 44-120 months), 3 (8.3%; exact 95% confidence interval 1.8%-23.1%) cases recurred. Hence, the recurrence rate was 5.3% (1 of 19) for primary periocular IEC and 11.8% (2 of 17) for recurrent periocular IEC. CONCLUSIONS: The Australian MMS database provides data for the first prospective series of periocular IEC managed by MMS. Periocular IEC demonstrates significant subclinical tumor extension, with no significant differences in the clinical features of primary and recurrent lesions. Compared with other published studies, the recurrence rate of 5% and 12% for primary and recurrent lesions, respectively, with more than 5-years of follow-up for most cases emphasizes the importance of margin-controlled excision for periocular IEC.

Surgical margins for lentigo maligna and lentigo maligna melanoma: the technique of mapped serial excision.

OBJECTIVES: To assess the margins required for excision of lentigo maligna (LM) and lentigo maligna melanoma (LMM) by the technique of mapped serial excision (MSE), and to assess the efficacy of MSE. DESIGN: An interventional, prospective, noncontrolled case series. SETTING: Tertiary referral, dermatologic surgery unit. PATIENTS: Consecutive patients with head and neck LM or LMM who underwent MSE between March 1, 1993, and October 31, 2002. INTERVENTION: The MSE of LM or LMM. MAIN OUTCOME MEASURES: The number of 5-mm levels for excision of LM and LMM and recurrence. RESULTS: One hundred sixty-one LMs or LMMs in 155 patients were treated. Thirty percent (37 of 125) of LMs required more than 5-mm margins. For LMMs less than 1 mm in Breslow thickness, 12% (4/32) required more than 10-mm margins. For primary tumors, 20% of LMs (18 of 91) required more than 5-mm margins, while 10% of LMMs less than 1 mm in Breslow thickness (2 of 21) required more than a 10-mm margin. For recurrent tumors, 56% of LMs (19/34) required more than a 5-mm margin. Mean follow-up of 38 months (range, 5-100 months) showed 4 recurrences (2%) after MSE. The extrapolated recurrence at 5 years was 5.0%. CONCLUSIONS: The current recommendations of 5-mm margins for LM and 10-mm margins for LMM less than 1 mm in Breslow thickness are often insufficient. Our results demonstrate the importance of margin-controlled excision, particularly in recurrent lesions. The use of MSE offers a high cure rate, in conjunction with tissue conservation.

Superficial malignant fibrous histiocytoma presenting as recurrent atypical fibroxanthoma.

Atypical fibroxanthoma (AFX) and malignant fibrous histiocytoma (MFH) are neoplasms of probable fibrohistiocytic lineage. The precise relationship between AFX and MFH remains controversial. We present two cases in which a lesion was excised from the face of an elderly person and reported as AFX. Both cases recurred within 12 months and were reclassified as MFH. They were excised with slow Mohs' micrographic surgery, with adjuvant radiotherapy in one case. In the other, a further recurrence was treated with orbital exenteration and radiotherapy. We review the difficulties in differentiating between AFX and MFH and the implications for treatment.

Eyelid sebaceous carcinoma masquerading as in situ squamous cell carcinoma.

BACKGROUND: Sebaceous carcinoma (SC) accounts for 1% to 5.5% of all eyelid malignancies. Diagnosis is often delayed because of its ability to masquerade as other periocular lesions both clinically and histologically. OBJECTIVE: To promote a high index of suspicion among Mohs surgeons for SC when managing biopsy-proven in situ squamous cell carcinoma (SCC) of the eyelid, particularly the upper eyelid. METHODS: This is a retrospective case review. RESULTS: A 77-year-old woman and a 71-year-old man were referred for Mohs micrographic surgery with a diagnosis of upper eyelid in situ SCC on biopsy. Intraoperatively, the presence of clear cell differentiation, in addition to extensive conjunctival involvement, leads to the correct diagnosis of SC. One case was subsequently found to have Muir Torre syndrome. CONCLUSION: Early diagnosis of eyelid SC leads to a better outcome and a higher survival rate. Hence, when managing in situ SCC of the eyelids, particularly the upper eyelid, Mohs surgeons should be aware of the possibility of SC and actively look for sebaceous cell differentiation or extensive conjunctival spread.

Giant keratoacanthoma arising in hypertrophic lichen planus.

A 45-year-old man presented with a rapidly enlarging tumour in an area of long-standing hypertrophic lichen planus of the lower leg. Histological examination of the resected specimen showed it to be a giant keratoacanthoma measuring 37 x 57 mm. Neoplastic change is a rarely reported complication of chronic variants of cutaneous lichen planus. To date there have been only two reports of keratoacanthoma development in association with lichen planus.

Discoid lupus erythematosus presenting as madarosis.

PURPOSE: Discoid lupus erythematosus (DLE) is an autoimmune disorder that usually affects the sun-exposed skin. Periocular involvement occurs uncommonly and may progress from eyelid erythema to scarring and madarosis. METHODS: Observational case report. RESULT: A case of DLE that presented with madarosis alone in the absence of preceding skin erythema and scarring. CONCLUSION: Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Biopsy should be considered in the presence of clinical features such as erythematous scale on the face and alopecia and sent for direct immunofluorescence staining.