Hailey-Hailey disease.

Thompson LD.
Ear Nose Throat J. 2016 Sep;95(9):370.
FIRST PARAGRAPH: Hailey-Hailey disease is a genodermatosis characterized by incomplete penetrance of an autosomal dominant disorder with defects in the ATP2C1 gene. Patients present with well-demarcated, erythematous plaques that often show crusting. The plaques may be vegetative, commonly associated with a distinct malodor. There may be associated burning or itching. While intertriginous areas are most commonly affected, the neck is also frequently involved. There is a natural remission and exacerbation cycle, with heat, moisture, and trauma the most common exacerbating factors. The lesions may be managed by corticosteroids or antibiotics, along with retinoids for severe disease.
PubMed ID: 27657314
Article Size: <1 MB

Merkel cell carcinoma.

Shiffer JD, Thompson LD.
Ear Nose Throat J. 2014 Mar;93(3):100-2.
FIRST PARAGRAPH: Merkel cell carcinoma is a rare and highly aggressive cutaneous neuroendocrine carcinoma that, in most cases, is caused by Merkel cell polyomavirus (approximately 80% of cases). The tumor usually arises on sun-exposed skin of elderly patients, particularly on the head and neck and extremities. There is a slight female preponderance and an increased risk in individuals who are immunosuppressed. Clinically, the tumors are often indistinguishable from other skin cancers and typically present as a firm, painless, rapidly growing nodule. They may be flesh-colored, red, or blue tumors that typically vary in size from 0.5 cm to more than 5 cm (average, 2 cm). When they have a reddish nodular appearance, they may be mistaken for angiosarcoma or granulation tissue.
PubMed ID: 24652557
Article Size: <1 MB

Skin basal cell carcinoma.

Thompson LD.
Ear Nose Throat J. 2010 Sep;89(9):418-20.
FIRST PARAGRAPH: Basal cell carcinoma (BCC) is a low-grade malignancy of basal keratinocytes, the cells responsible for epidermis formation. Melanocytes are seen between the keratinocytes, but they are not responsible for this tumor type. The etiology is multifactorial, related to ultraviolet sun exposure, radiation, and immunosuppression, among other factors. This is one of the most common cancers in humans. The tumors will typically present in older adults, although young adults can also develop this tumor. There is a slight male predilection, but this may be due to differences in sun exposure rather than gender variance. Caucasians and light-skinned people have a higher incidence than dark-skinned people.
PubMed ID: 20859866
Article Size: <1 MB


Thompson LD.
Ear Nose Throat J. 2007 Sep;86(9):542, 545
FIRST PARAGRAPH: Melanoma is a malignancy of melanocytes that show a series of molecular events that result in the melanocytes going through a stepwise progression from dysplasia to invasion to metastasis. Melanomas account for approximately 4.4% of all malignancies. Approximately 62,000 new cases of melanoma are reported annually in the United States, and they are responsible for about 7,900 deaths. The incidence of melanoma worldwide has been increasing steadily.
PubMed ID: 17970142
Article Size: <1 MB

Molluscum contagiosum.

Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Aug;82(8):560.
FIRST PARAGRAPH: Molluscum contagiosum is a virus-induced epithelial hyperplasia produced by a DNA poxvirus. This common disease can be found on the skin and mucosal surfaces. It is acquired by direct contact with an infected individual or, less commonly, by contact with a fomite (e.g., clothing, towels, and toys); autoinoculation is common. Molluscum contagiosum is usually seen in children and young adults; immunocompromised persons are especially vulnerable to infection. Lesions predominantly arise on the skin of the face, neck, eyelids, trunk, and genitalia, as well as on the mucous membranes of these areas (as applicable). The incubation period averages between 2 and 7 weeks, although it can be much longer.
PubMed ID: 14503090
Article Size: <1 MB


Thompson LD.
Ear Nose Throat J. 2012 Jan;91(1):18-20.
FIRST PARAGRAPH: Pilomatricoma, also referred to as pilomatrixoma and calcifying epithelioma of Malherbe, is a benign dermal-subcutaneous tumor derived from the matrix of the hair follicle. Its development is associated with a known mutation in the CTNNB1 gene, the gene that encodes for beta-catenin. Pilomatricomas are relatively common tumors. They usually arise during the first 2 decades of life, and they have no predilection for either sex.
PubMed ID: 22278863
Article Size: <1 MB

Skin keloid.

Thompson LD.
Ear Nose Throat J. 2004 Aug;83(8):519.
FIRST PARAGRAPH: A keloid is a form of excessive scar formation that occurs in response to tissue injury. Keloids arise as a result of abnormal wound healing where there is a disruption of the balance between collagen formation and degradation with a shift toward excess synthesis. Even with injuries that are seemingly minor, the resultant scar formation is disproportionately excessive. The etiology is unknown.
PubMed ID: 15487627
Article Size: <1 MB

Seborrheic keratosis.

Thompson LD.
Ear Nose Throat J. 2006 Feb;85(2):79.
FIRST PARAGRAPH: Seborrheic keratosis is a benign proliferation of the epidermis. Many names are applied to this lesion (senile wart, melanoacanthoma), but seborrheic keratosis is the most widely accepted. Considered one of the most common skin lesions, it usually occurs in older patients; there is no predilection for either sex. The lesions appear “stuck on” the skin, usually on sun-exposed skin. They can be tan, brown, or black. Frequent irritation or friction may cause a papule/nodule to become red and scaly and result in a clinical misdiagnosis. Many variants of seborrheic keratosis are recognized clinically and histologically, but they have no management implications.
PubMed ID: 16579189
Article Size: <1 MB

Melanocytic nevus.

Thompson L.
Ear Nose Throat J. 2006 Dec;85(12):804.
FIRST PARAGRAPH: Nevus is an umbrella term used to describe a host of benign melanocytic growths. Most nevi are small (<0.6 cm), well demarcated, circumscribed lesions with variable pigmentation. Histologically, they are symmetrical.
PubMed ID: 17240699
Article Size: <1 MB

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Cowan ML, Thompson LD, Leon ME, Bishop JA.
Head Neck Pathol. 2016 Jun;10(2):161-6.
Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.
PubMed ID: 26276044
Article Size: 1.4 MB