Danielle Bouffard

Kerion of the vulva caused by Trichophyton mentagrophytes.

Background: Deep dermatophytosis of genital skin is a rare clinical manifestation of infection by a common group of pathogens. Objective: We emphasize the importance of clinical suspicion and the use of accurate diagnostic methods in the evaluation of deep dermatophytosis. Methods: We report a single case of tinea pubis, kerion type, caused by Trichophyton mentagrophytes in an immunocompetent host. Results: A 54-year-old female presented with a suppurative infection of the vulva and pubis that was unresponsive to empirical antibiotic therapy. T. mentagrophytes was isolated. Oral itraconazole was initiated on the basis of clinical suspicion and continued for a total of 6 weeks. Conclusion: Accurate diagnosis and treatment of deep dermatophytosis of genital skin rests upon proper identification of the pathogen. Prompt initiation of treatment with an oral antifungal agent, such as itraconazole, should be undertaken in order to avoid irreversible scarring alopecia.

Extramammary Paget disease: epidemiology and association to cancer in a Quebec-based population.

Objective This study aimed to further characterize the epidemiology, clinical manifestations, pathology, immunopathology, outcome from therapy, and associated underlying malignancy in extramammary Paget disease (EMPD). Materials and Methods We conducted a retrospective review of patients treated for EMPD in our tertiary care center during a 23-year period ranging from 1985 to 2008. Results Sixty-four cases of EMPD were diagnosed during this period. Mean age at diagnosis was 66.8 years. Of the patients, 79.7% were female. Tumors were mostly localized on the vulvoperineal region. Associated cancers were found in 30% of the patients and included breast cancer and urogenital cancers. Of the patients, 42% had a least 1 recurrence. The risk of recurrence could only be associated to tumor location on the vulvoperineal region. The limitations of this study include its retrospective nature and sample size. Conclusions Extramammary Paget disease is more commonly found on the vulva of older women and frequently recurs. Recurrence was not associated to margin status, which would support a more conservative therapeutic approach.

Plexiform melanocytic schwannoma: a mimic of melanoma

We present a unique dermal tumor for which we propose the term plexiform melanocytic schwanomma. The proliferation consisted of lobules of epithelioid and spindled cells with S100, Melan‐A and HMB‐45 positivity but without obvious melanin pigmentation. The nuclei were moderately pleomorphic in some areas, and in a few areas the mitotic index was elevated. Schwannian differentiation was inferred from the presence of areas with nuclear palisading resembling Verocay bodies, from plexiform architecture and from the presence of a thin rim of EMA positivity around the tumor. Array‐based comparative genomic hybridization showed genomic losses that overlap with those seen in sporadic schwanomma. The differential diagnosis included melanoma, melanotic schwannoma and cutaneous melanocytoneuroma, and we compare and contrast our case with these entities.

Vulvar sarcoidosis: case report and review of the literature.

Background: Sarcoidosis is a multisystemic disorder of unknown etiology that can affect multiple organs, including the lungs, skin, and eyes. Vulvar sarcoidosis has anecdotally been reported. Objective: The aim of this article is to describe a case of vulvar sarcoidosis and review the few cases that have been reported. Methods: We report the case of a 39-year-old woman who presented to the dermatologist with a 2-year history of vulvar pruritus. Results: Examination revealed infiltrated plaques on the vulva and perianal region. The biopsy demonstrated well-defined, nonnecrotizing granulomas in the dermis. Further investigation revealed hilar adenopathy consistent with sarcoidosis. The patient responded well to topical corticosteroids. Conclusion: In the presence of granulomatous lesions of the genital region, infectious causes, foreign body reaction, Crohn disease, and sarcoidosis should be part of the differential diagnosis.

A distinct cutaneous reaction to sorafenib and a multikinase inhibitor

Although a genetic background is essential, it is not, by itself, sufficient to induce an autoimmune response. Indeed, exogenous agents play a major role in triggering pemphigus. They are heterogeneous and numerous, encompassing physical agents (heat, ultraviolet and ionizing rays, surgical and cosmetic procedures), viral infections (especially herpesvirus), certain diet ingredients, emotional stress, and, most importantly, drug intake. Amongst the drugs implicated in the induction or exacerbation of pemphigus, interferons (IFNs) and other cytokines have a pivotal role. Imiquimod [1-(2-methylpropyl)-1Himidazo(4,5-c)quinolin-4-amine] is an imidazoquinoline amine which affects both aspects of the immune system, i.e. the innate and acquired immune responses. These effects are mediated by drug-induced cytokines, including IFN-α, which is produced by keratinocytes and has antiviral, antitumor, and immunomodulatory properties. IFN-α acts directly on human B lymphocytes by modulating immunoglobulin (Ig) synthesis. Long-term treatment with systemic IFN-α often induces the appearance of pemphigus antibodies in nonpemphigus patients. Repeated administration of imiquimod, whilst altering the local immune response, may cause acantholytic lesions as a result of the production of IFN-α, a cytokine commonly identified as a pemphigus-inducing drug. In our case, repeated applications of imiquimod, by altering the local immune response, led to the onset of contact pemphigus as the patient was genetically predisposed (HLA DR14 and DQ1).

Acute blistering diseases on the burn ward: Beyond Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

OBJECTIVE Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis are on a spectrum of rare reactions primarily attributable to drugs. Timely diagnosis, cessation of the offending drug and burn center care are associated with favorable outcomes. Acute blistering disease has a wide differential diagnosis, including autoimmune bullous disease and other drug reactions. The aim of our study was to identify the final diagnosis in patients transferred for widespread blistering disease and to identify clinical features at admission predicting final diagnosis. METHODS We performed a 5-year retrospective chart review (2006-2011) of the clinical features at admission of patients transferred to a burn ward with widespread blistering disease. Clinical features at admission were compared between patients. RESULTS 12 patients had a final diagnosis of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis and 7 patients had an alternative final diagnosis. Skin detachment surface area at admission was superior in the Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis group. Presence of tense bullae and pustules was associated with an alternative final diagnosis. CONCLUSION Extensive skin detachment surface and morphological features (tense bullae, pustules) were statistically significant clinical clues to final diagnosis. Patients transferred for widespread blistering disease should be thoroughly evaluated in order to exclude other causes of acute blistering disease.

Canadian case report of erythema nodosum leprosum successfully treated with prednisone and thalidomide.

Background: Erythema nodosum leprosum (ENL) is a disease rarely encountered in Canada. It is characterized by multiple remissions and recurrences, often requires long-term treatment, and can result in debilitating sequelae. Objective: To promote rapid recognition and adequate therapy for ENL. Methods: Case report of a 39-year-old man diagnosed with an ENL. The clinical and histopathologic features, treatment provided, and response to treatment are detailed in this article. Results: ENL presented itself as painful cutaneous lesions on the face and limbs, bilateral paresthesia of the fourth and fifth fingers, and systemic symptoms. Prednisone 40 mg daily for a week and then 60 mg daily for another week reduced the lesions by 80% and the pain by 50%. Although prednisone 60 mg daily was continued for one more week and then stopped, thalidomide was started at a dose of 300 mg daily for 4 weeks and then reduced gradually, which led to complete resolution. Conclusion: At the 7½-month follow-up, the patient remained completely asymptomatic.

A secondary syphilis rash with scaly target lesions

Abstract A 40-year-old man reported a 5-day history of fever and malaise, followed by a pruritic generalized rash. He had well-demarcated erythematous papules and plaques with scaling. The patient was diagnosed with secondary syphilis. The skin biopsy showed a psoriasiform lichenoid dermatitis with plasma cells. The anti-T. pallidum antibody confirmed the presence of spirochetes. He was also found to be hepatitis C virus and human immunodeficiency virus positive. The characteristic rash of secondary syphilis may appear as maculopapular, evolving initially from macules to small reddish-brown papules with minor scaling later. When the scaling is prominent, lesions can be difficult to differentiate from guttate psoriasis. Typical target lesions are most often associated with erythema multiforme, but they can rarely occur in secondary and congenital syphilis. Syphilis should be suspected in high-risk patients presenting a variety of atypical syndromes such as neurologic symptoms, uveitis or cholestatic hepatitis, especially if palmoplantar lesions are present.

Helicobacter cinaedi bacteremia mimicking eosinophilic fasciitis in a patient with X-linked agammaglobulinemia

XLA: X-linked agammaglobulinemia INTRODUCTION Bruton agammaglobulinemia (XLA) is an X-linked genetic disorder characterized by severe antibody deficiency. The cornerstone of treatment is immunoglobulin replacement therapy, but patients remain at risk for recurrent sinopulmonary, gastrointestinal, and skin infections. XLA has also been associated several inflammatory and autoimmune diseases such as arthritis, inflammatory bowel disease, and, more recently, eosinophilic fasciitis. Helicobacter cinaedi is reportedly involved in several cutaneous manifestations ranging from cellulitis to superficial ulcers on erythematous, eroded plaques. We report a case of H cinaedi bacteremia mimicking eosinophilic fasciitis in a patient with XLA.

A rare cause of blanching red legs: cutaneous collagenous vasculopathy

“The redness just won’t go away,” described a 55-year-old Caucasian woman known for diabetes, hypertension, hypercholesterolemia, and gastroesophageal reflux. Medications for these illnesses included metformin, sitagliptin, dapagliflozin, ramipril, atorvastatin, aspirin, and pantoprazole. The patient denied any recent changes in this therapeutic regimen. For the past 2 years, the lower aspect of both of her legs had been affected by petechiae and diffuse nonpruritic and non-urticating macules that were otherwise asymptomatic. There was no involvement of the nails or mucosal tissue. Upon examination, the erythema blanched with diascopy. Some of the first diagnoses we considered were that of pigmented purpuric dermatoses, generalized essential telangiectasia or leukocytoclastic vasculitides among others. A serum workup including complete blood count, C-reactive protein, antinuclear antibodies (ANA), rheumatoid factor, and complement levels were entirely within normal limits. Hence, skin biopsy of the right leg was obtained. The epidermis displayed a slight hyperpigmentation, and the superficial reticular dermis showed dilated vessels with a thickened hyalinized vessel wall and absence of perivascular hemosiderin deposition. There was no evidence of frank vasculitis nor inflammation. These histologic findings were consistent with cutaneous collagenous vasculopathy (CCV) (Fig. 1). CCV is a distinct and rare microvascular disease affecting superficial dermal blood vessels and was only first described in 2000. It is a largely underdiagnosed microangiopathy, frequently mistaken for other more common disease entities. Notably, CCV is associated with hypertension, diabetes, dyslipidemia, and the metabolic syndrome. The precise etiology of CCV is not fully understood; however, several mechanisms were suggested such as microangiopathy related to diabetes mellitus, trauma, and genetic defects affecting collagen production or vascular collagen deposition during the repair process of damaged vessels. Finally, as for clinical management, pulsed dye laser was reported as an effective treatment for the telangiectatic lesions in CCV. Several other treatment modalities have been described including sclerotherapy, intense pulsed light therapy, and compression stockings. Our patient, in particular, was prescribed 20–30 mmHg compression stockings. In conclusion, CCV should be considered in the differential diagnosis of asymptomatic acquired diffuse telangiectatic lesions, and a skin biopsy is essential for the diagnosis.