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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 26, Issue 8, 2020

Commentary

Urgent safety considerations for dermatologic surgeons in the COVID-19 pandemic

Dermatologic surgeons are at increased risk of contracting SARS-COV-2. At time of writing, there is no published standard for the role of pre-operative testing or the use of smoke evacuators, and personal protective equipment (PPE) in dermatologic surgery. Risks and safety measures in otolaryngology, plastic surgery, and ophthalmology are discussed. In Mohs surgery, cases involving nasal or oral mucosa are highest risk for SARS-COV-2 transmission; pre-operative testing and N95 masks should be urgently prioritized for these cases. Other key safety recommendations include strict control of patient droplets and expanded pre-clinic screening. Dermatologic surgeons are encouraged to advocate for appropriate pre-operative tests, smoke evacuators, and PPE. Future directions would include national consensus guidelines with continued refinement of safety protocols.

Acne related to dietary supplements

Multiple prescription medications may cause or aggravate acne. A number of dietary supplements have also been linked to acne, including those containing vitamins B6/B12, iodine, and whey, as well as "muscle building supplements" that may be contaminated with anabolic-androgenic steroids (AAS). Acne linked to dietary supplements generally resolves following supplement discontinuation. Lesions associated with high-dose vitamin B6 and B12 supplements have been described as monomorphic and although pathogenesis is unknown, a number of hypotheses have been proposed. Iodine-related acne may be related to the use of kelp supplements and has been reported as monomorphic, inflammatory pustules on the face and upper trunk. Whey protein supplements, derived from milk and used for bodybuilding, are associated with papulonodular acne involving the trunk and sometimes the face. Finally, AAS-induced acne has been described as acne fulminans, acne conglobata, and acne papulopustulosa. With studies indicating that about half of US adults report using dietary supplements, it is important that dermatologists directly ask acne patients about their supplement use and educate them on the potential risks of even seemingly innocuous dietary supplements.

Dermatology and anatomy laboratory: comparing three formats of integration

Background: Medical schools rarely offer exposure to clinical dermatology in the first-year curriculum. Objective: We describe and report student satisfaction results of three novel teaching methods for integrating basic dermatology concepts into gross anatomy laboratory. Methods: During the first year of the intervention, 180 students attended an hour-long anatomy laboratory session during which skin lesions were examined. One attending and three resident dermatologists spent time at all tables of students, then circulated to answer questions. During the second year, 189 students participated in the same teaching session preceded by a 30-minute in-class lecture. During the third year, 172 students were given the option to view a supplemental online video module before or after the teaching session. Each year following the teaching session students were sent an optional online survey regarding the impact of the teaching session on their understanding of skin lesions and their cadaver experience. Results: Overall, students believed the intervention helped them develop a better understanding and appreciation for dermatology. Preceding the laboratory session with a lecture or educational video yielded higher satisfaction scores. Conclusions: This brief teaching intervention illustrates an approach to introducing dermatologic entities within the foundational science curriculum of the first year of medical school.

Case Report

Case report and review of solitary cutaneous focal mucinosis: a unique primary cutaneous mucinosis unrelated to mucinosis-associated systemic diseases

Localized deposition of mucin in the upper dermis is referred to as cutaneous focal mucinosis. Patients with this condition either present with a single skin lesion (solitary cutaneous focal mucinosis) or numerous skin lesions (multiple cutaneous focal mucinosis). A man with solitary cutaneous focal mucinosis is described and the features of this condition are reviewed. Solitary cutaneous focal mucinosis has a slight male predominance and typically presents in adults, ranging in age from 29 years to 60 years, as a nodule or papule that is flesh-colored or white and most commonly located on an extremity or the trunk. Microscopic examination shows deposition of mucin in the upper dermis; the overlying epidermis can be normal, atrophic or hyperplastic. The skin lesion is often removed at the time of biopsy. However, recurrence has not been observed when the mucin deposition is present at the edge of the biopsy or excision specimen. Although the pathogenesis of this condition remains to be established, in contrast to individuals with multiple cutaneous focal mucinosis, solitary cutaneous focal mucinosis is a unique primary cutaneous mucinosis unrelated to mucinosis-associated systemic diseases.

Toxic epidermal necrolysis occurring with immune checkpoint inhibitors

Nivolumab and ipilimumab are immune checkpoint inhibitors (ICIs) used in the management of advanced malignancies including malignant melanoma. Although several cutaneous adverse events have been reported with these immunotherapy agents, toxic epidermal necrolysis (TEN) secondary to ICIs is rare. We report a 67-year-old man with TEN occurring during nivolumab and ipilimumab co-therapy and review published cases to highlight the challenges in recognizing and managing these patients. ICI-induced TEN can present atypically with delayed onset in comorbid, immunosuppressed patients with an associated high mortality rate. Prompt recognition and drug withdrawal are essential to improve outcomes. High dose systemic corticosteroid has also been recommended for the management of ICI-induced TEN, unlike other drug-induced TEN for which optimal immunomodulatory treatment is still debated.

Kikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus?

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disorder that must be distinguished from systemic lupus erythematosus (SLE). Although a minority of patients with KFD develop SLE, most patients have a self-limited disease. Importantly, KFD can have skin manifestations resembling cutaneous lupus. Therefore, the diagnosis of SLE should be predicated on a complete rheumatologic workup and not on the constellation of skin disease and lymphadenitis. Nonetheless, as our exceedingly rare case illustrates, patients who do not initially meet diagnostic criteria for SLE require dermatologic follow-up. We present a young adult woman who had a remote history of KFD and later presented with combined features of discoid lupus and lupus erythematosus panniculitis (LEP). On subsequent rheumatologic workup, she fulfilled criteria for SLE. We discuss the differential diagnosis of both KFD and LEP and emphasize how strong communication among dermatologists and other healthcare providers is essential in the management of patients with KFD.

Case Presentation

Intravascular lymphoma presenting with paraneoplastic syndrome

Intravascular lymphomatosis (IVL) is a rare type of B-cell non-Hodgkin lymphoma (BCNHL), which can mimic many other diseases. Fever, neurological symptoms, and skin findings are the most frequent clinical findings. Intravascular lymphomatosis may be associated with genetic factors and infection with human immunodeficiency virus (HIV), human herpesvirus 8 (HHV-8), human T-lymphotropic virus 1 (HTLV1), Epstein-Barr virus (EBV), and hepatitis B virus (HBV). A 50-year-old man was hospitalized with recalcitrant hyponatremia of unknown cause. He had also telangiectatic, indurated, slightly erythematous plaques on his trunk for the last 10 days. His past medical history was unremarkable, although he was a carrier of hepatitis B. Multiple skin biopsies were performed and were considered to be diagnostic of IVL. The hyponatremia was unresponsive to water restriction and hypertonic solution support but it resolved with B cell directed chemotherapy. A final diagnosis was made as syndrome of inappropriate antidiuretic hormone (SIADH) in the setting of IVL. This case had a relatively early diagnosis with just 10-days of skin lesions. Intravascular lymphomatosis is a very rare disease and is usually difficult to diagnose. An even more uncommon presentation is IVL complicated by a paraneoplastic syndrome. There are prior reported cases of SIADH in the setting of IVL. However, this case underscores the importance of evaluating patients with SIADH for potential IVL.

Dermatomyositis associated with nivolumab therapy for melanoma: a case report and review of the literature

We present a rare case of dermatomyositis associated with nivolumab therapy for melanoma. Nivolumab is an immune checkpoint inhibitor that blocks the programmed death-1 (PD1) receptor and has a number of associated immunotherapy related adverse events. Although most are T-cell mediated, some are antibody mediated mimics of classical autoimmune diseases. We review the characteristics of other cases of anti-PD1 associated dermatomyositis and the recent literature to better understand how to classify and treat this challenging immunotherapy related adverse event.

Secukinumab-associated localized granuloma annulare (SAGA): a case report and review of the literature

Granuloma annulare (GA) is a benign, usually self-limited inflammatory skin dermatosis characterized clinically by pink-red to brown dermal papules or annular plaques. The main histologic feature is the presence of palisading or interstitial granulomas composed of necrobiotic collagen, elastic fibers, and mucin surrounded by a lymphohistiocytic infiltrate. Granuloma annulare is commonly associated with trauma, infections, diabetes mellitus, dyslipidemia, malignancy, thyroid disease, and a variety of medications. Two cases of GA have been reported in association with the use of secukinumab, a monoclonal antibody directed against interleukin 17A (IL17A), for the treatment of moderate-to-severe plaque psoriasis. We report the third case of secukinumab-associated GA in a 52-year-old woman with a history of diabetes mellitus type II, dyslipidemia, and non-alcoholic steatohepatitis. After four months of therapy with secukinumab, she presented with pink papules coalescing to plaques involving the antecubital fossae. Histology demonstrated a lymphohistiocytic palisading granuloma with central necrobiotic collagen and mucin, consistent with GA. Physicians should be aware of the possibility of GA developing in patients receiving secukinumab, especially in those with predisposing factors for GA. A better understanding of secukinumab-associated GA may lead to discoveries in GA pathogenesis and reveal broader immunomodulatory effects of secukinumab.

An evolving presentation of cutaneous tuberculosis

Tuberculosis is a fairly common disease in the United States and around the world, newly infecting ten million people throughout the world per year. Despite the pervasiveness of tuberculosis, cutaneous tuberculosis (CTB) rarely manifests worldwide. Tuberculous infections of the skin arise in several distinct variants that can be classified as either multibacillary or paucibacillary; each subtype within these categories presents with its own morphological and histological findings. The diagnosis of CTB can prove clinically challenging as its variants mimic many conditions dermatologist encounter on a daily basis. Additionally, tissue confirmation is difficult. We report a case of CTB which evolved from a lupus vulgaris presentation to the metastatic tuberculous abscess variant.

Recurrent angiolymphoid hyperplasia with eosinophilia during several pregnancies

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign vascular tumor. Although its etiology is not clearly known, infection, hormonal factors, and trauma are the suspected reasons. There are few cases considered to be related to pregnancy described in the literature. Granulomas are rarely seen in the histopathology and only four patients with granulomas have been described. Herein, we will present a 28-year-old woman who has been pregnant three times; in every pregnancy, she developed pink nodular lesions on the back of her ear. In the first two pregnancies, the lesions had appeared during the pregnancy and regressed completely in the postpartum period. In her third pregnancy, the condition emerged again and lasted 1.5 years after birth of her child. Histopathology exhibited ALHE with granulomas. Although the relationship between ALHE and pregnancy is already known, there are few cutaneous ALHE cases associated with pregnancy reported in the literature. This association is further supported by this case having recurrent ALHE during each pregnancy period. Also, the presence of naked granuloma in histopathology, unlike most of the cases, contributes to the current histopathological data.

Photo Vignette

A novel CDKN2A in-frame deletion associated with pancreatic cancer-melanoma syndrome

Pancreatic cancer-melanoma syndrome (PCMS) is an inherited condition in which mutation carriers have an increased risk of malignant melanoma and/or pancreatic cancer. About 30% of PCMS cases carry mutations in CDKN2A. This gene encodes several protein isoforms, one of which, known as p16, regulates the cell-cycle by interacting with CDK4/CDK6 kinases and with several non-CDK proteins. Herein, we report on a novel CDKN2A germline in-frame deletion (c.52_57delACGGCC) found in an Italian family with PCMS. By segregation analysis, the c.52_57delACGGCC was proven to segregate in kindred with cutaneous melanoma (CM), in kindred with CM and pancreatic cancer, and in a single case presenting only with pancreatic cancer. In the literature, duplication mapping in the same genic region has been already reported at the germline level in several unrelated CM cases as a variant of unknown clinical significance. A computational approach for studying the effect of mutational changes over p16 protein structure showed that both the deletion and the duplication of the c.52_57 nucleotides result in protein misfolding and loss of interactors' binding. In conclusion, the present results argue that the quantitative alteration of nucleotides c.52_57 has a pathogenic role in p16 function and that the c.52_57delACGGCC is associated with PCMS.

Giant pilomatrixoma: a distinctive clinical variant: a new case and review of the literature.

Pilomatrixoma is a benign adnexal tumor very common in pediatric age and in young adults that derives from follicular matrix cells. Although clinically it usually presents as a subcutaneous nodule of bluish color less than 3cm in size, multiple clinicopathological variants have been described in the literature. Among these we can find the giant pilomatrixoma, a rare clinical variant that reaches a size greater than or equal to 4cm and can simulate the clinical presentation of a malignant neoplasm. We report a 59-year-old man with an exophytic and ulcerated nodule in the left parotid region that was removed with the suspected diagnosis of a cutaneous squamous cell carcinoma. Histopathological analysis showed a proliferation of basaloid cells with areas of transition to ghost cells, under granulation tissue, hemorrhage, and an ulcerated epidermis. Thus, the diagnosis of giant pilomatrixoma was made. We reviewed the literature and found a total of 53 articles that report a total of 71 cases of giant pilomatrixoma. It is important to recognize this clinical subtype of pilomatrixoma because, apart from the possibility of being clinically confused with malignant lesions, the clinicopathological differential diagnosis must be made with the proliferating pilomatrixoma and pilomatrixcarcinoma.

Hand-foot skin reaction with primarily dorsal involvement in a patient with metastatic renal cell carcinoma on cabozantinib

A 61-year-old man with metastatic renal cell carcinoma on cabozantinib developed hand-foot skin reaction with predominantly dorsal involvement including painful violaceous plaques over the joints and keratotic yellow plaques on the palmar fingers. The medication was discontinued with resolution of the plaques and later reinitiated at a lower dose uneventfully.

Giant pigmented apocrine hidrocystoma of the scalp

Hidrocystomas are benign cysts of sweat duct epithelium that can present as single or multiple lesions, with or without pigmentation. The size is typically 1-3mm in diameter. Although hidrocystomas commonly occur in most parts of the head and neck region, occurrence on the scalp is rare. Herein, we present a 29-year-old woman with a giant pigmented apocrine hidrocystoma of the scalp, which, to our knowledge, represents the largest of its kind reported to date.

Primary cutaneous adenoid cystic carcinoma of the abdomen: a rare entity

Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excision.

Circumscribed palmoplantar hypokeratosis: a case report and review of the literature

We describe an 84-year-old man presenting with a solitary, well-circumscribed, chronic erosion of the sole. Histopathologic examination confirmed diagnosis of circumscribed palmoplantar hypokeratosis. Circumscribed palmoplantar hypokeratosis is a rare and benign condition of unknown etiology presenting as an erosion on the palms or soles. Although lesions are typically asymptomatic, the entity is important for dermatologists and providers in other specialties to recognize, especially considering a differential diagnosis that includes neoplasia.

Surgical Pearl

Revisiting the Rintala advancement flap for nasal tip reconstruction

The approach to nasal reconstruction is based on the nature of the defect and the donor tissue. The Rintala flap, first described by Rintala and Asko-Slejavaara, is a surgical technique used to close midline nasal defects. It is a simple, one-stage procedure and is most effective for defects ranging from 1.5 to 2.5 centimeters. In addition, the Rintala flap is an excellent tissue match and maintains the natural contour of the nose. Other flaps employed in nasal defect closure include the paramedian forehead flap and the Rieger flap. The authors discuss the Rintala flap technique and utility with comparison to alternative flaps used for nasal reconstruction.

Letter

Association between pemphigus and psoriasis: a systematic review and meta-analysis

Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus with psoriasis, but this association is lacking larger scale investigation. With this in mind, we conducted a systematic review and meta-analysis to evaluate the association between pemphigus and psoriasis. In doing so, we found an association between the two conditions. Pemphigus was more common in patients with psoriasis than in controls (OR 2.64, 95% CI 1.24-5.59, P=0.01), with heterogeneity (I2=94%). We go on to propose pathophysiologic mechanisms and its relevance for diagnostic and management considerations.

Erosive pustular dermatosis of the scalp

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory condition commonly associated with antecedent iatrogenic insult. EPDS may be diagnostically challenging owing to a lack of pathognomonic histologic findings and cutaneous manifestations that overlap with alternative dermatologic conditions. Therefore, EPDS may be more common than previously recognized. We present a 60-year-old woman with a four-year history of non-healing scalp erosions, progressive skin atrophy, and scarring alopecia despite intravenous antibiotics and intraoperative debridement who improved with systemic glucocorticoids. Our report emphasizes the importance of early recognition of EPDS when delayed wound healing and erosive disease occur in the setting of iatrogenic injury to the scalp. Timely treatment with systemic anti-inflammatory agents is paramount to prevent cicatricial alopecia and mitigate further scalp insult in EPDS.