Faculty Bibliography

Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory disease characterized by tender, erythematous, indurated breast plaques with associated edema, drainage, and scar formation.1 IGM is often mistaken for breast carcinoma or infectious mastitis.1,2 Histopathology readily distinguishes IGM from breast carcinoma, as the primary finding in IGM is granulomas centered around mammary lobules.3 Nevertheless, differentiating IGM from bacterial mastitis and other mimickers, such as atypical mycobacterial infections or sarcoidosis, can be more difficult.4 Herein, we report the largest case series of concurrent IGM and erythema nodosum (EN)

The evaluation of the vulva should always begin with a detailed clinical history. The clinician should be very familiar with vulvar anatomy and the changes it undergoes depending on the patient's age and hormonal status. A systematic approach should be developed when examining the vulva so as to not leave out any parts. Finally, there is a wide array of ancillary tests and diagnostics procedures that can be pursued to arrive at the correct diagnosis and begin proper management.

The vulva is an under-studied area of the female genitourinary tract which is prone to maceration, overgrowth of organisms, and atypical presentations of common dermatologic conditions. In current practice, dermatologists must recognize and manage vulvar infections and infestations beyond the more commonly recognized sexually transmitted infections. Herein, this article reviews the literature on a selection of under-recognized viral, bacterial, fungal, and parasitic vulvar infections and infestations.

Vulvar dermatoses are common, potentially debilitating conditions that can be seen by a variety of medical specialists. Lichenoid vulvar diseases, namely lichen sclerosus (LS), lichen planus (LP), and lichen simplex chronicus (LSC), can all negatively impact patients' quality of life and LS and LP also have an association with squamous cell carcinoma. It is essential that dermatologists are familiar with the unique features of each of these conditions to ensure the appropriate management and follow up. Herein, we provide an update on the epidemiology, clinical presentation, histopathology, and treatment of patients with vulvar LS, LP, and LSC.

Changes in melanocytic nevi during pregnancy are frequently attributed to the new hormonal milieu and are dismissed without concern for malignancy. Recent studies suggest that pregnancy itself does not induce significant change in nevi, and delays in the assessment of changing moles may contribute to the often more advanced nature of melanomas diagnosed during or soon after pregnancy. Nevi on the breasts and abdomen can grow as a result of skin expansion, but studies have found no significant changes in nevi located in more stable areas such as the back or lower extremities. There is also insufficient evidence to support the notion that nevi darken during pregnancy. As such, any changing nevus that would raise concern for malignancy in a nonpregnant patient should do so in a pregnant patient as well. Pregnancy can, however, induce physiologic pigmentary changes that are often worrisome to both patients and physicians. These benign changes include melasma, pigmentary demarcation lines, secondary areola, and linea nigra as well as other less common findings. It is important for physicians to recognize these changes as physiologic to provide adequate reassurance to their patients and avoid unnecessary stress.

Cutis verticis gyrata that involves only the face isa rare presentation of this even rarer cutaneousanomaly. We present a 61-year-old man, whodeveloped primary essential progressive cutis verticisgyrata of the face.

We present a 58-year-old woman with bipolardisorder and with a longstanding history of yellowbrown,hyperkeratotic papules in a seborrheicdistribution and nail changes. Her father andpaternal grandmother had similar eruptions and alsohad psychiatric disease. Histopathologic examinationshowed acantholysis and dyskeratosis, which wasconsistent with Darier-White disease. Darier-Whitedisease is a rare, hereditary disorder that is inheritedin an autosomal dominant manner and that usuallypresents in adolescence or early adulthood dueto mutations in the SERCA2 calcium pump. Theresultant alterations in calcium homeostasis affectdesmosome assembly and lead to acantholysis andapoptosis, which creates the characteristic eruption.Genetic linkage analysis of affected families showsthat a susceptibility locus for bipolar disorder cosegregateswith genetic markers that are found inthe Darier-White disease region. Treatments, whichinclude topical emollients, mild keratolytics, andtopical or oral retinoids, are aimed at improvingthe appearance of skin, relieving symptoms, andpreventing or treating infectious complications.

Cowden syndrome (CS) is a genetic cancerpredisposition syndrome that is associated withgermline mutations in the phosphate and tensinhomologue deleted on chromosome ten (PTEN)tumor suppressor gene. It is characterizedby the formation of benign and malignanttumors. Characteristic benign tumors includetrichilemmommas, acral keratoses, mucocutaneousneuromas, and oral papillomas. The most commonmalignant condition include breast, thyroid, andendometrial cancers. We present a case of a30-year-old woman with CS, who initially presentedwith trichilemmomas that were misdiagnosed ascomedonal acne. Recognition of the presentingfeatures of CS is important to ensure proper referral,management, and treatment for these patients.

Chronic arsenic exposure is known to inducepunctate keratoses with an increased risk ofprimary squamous-cell carcinoma. Drinking wateris currently the major source of arsenic exposureworldwide and is considered one of the mostsubstantial environmental carcinogens. We describethe case of a 61-year-old Hungarian woman withscattered, acral, hyperkeratotic papules and a historyof five palmoplantar squamous-cell carcinomasas well as two other extremity non-melanomaskin cancers. Prior to immigration, she had livedin a county of Southern Hungary that is known tohave elevated concentrations of inorganic arsenicin the drinking water above the World HealthOrganization's current maximum threshold forsafety. To date, this report is the first to describethe phenomenon of palmoplantar squamouscellcarcinomas in a patient from this region andunderscores the importance of vigilant screening inthose individuals who have spent substantial time inhigh-risk regions internationally and domestically.

Lymphangioma circumscriptum (LC) is anuncommon, benign, cutaneous disorder thatinvolves lymphatic dilatation to cause vesicularand papular lesions on the trunk, extremities, andanogenital areas. It may be a diagnostic challengewhen appearing in the anogenital region andoften is misdiagnosed and mistreated as infectiousetiologies, which include molluscum contagiosumand condylomata accuminata. We report a healthy40-year-old man with a six-month history of aneruption of multiple, asymptomatic, scrotal papulesthat were diagnosed as lymphangiomas. Awarenessof scrotal LC in adult men without prior disease orsymptoms is necessary for proper diagnosis andavoidance of unnecessary treatment.