Annular bullous pemphigoid: A case report and review of literature.

Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented. We present a rare case of annular bullous pemphigoid in a 50-year-old male and offer a brief review of the literature. Only five other case reports, including three in adults, have described this unusual presentation, which can mimic other autoimmune blistering diseases, including linear IgA bullous dermatosis and pemphigus herpetiformis. Therefore, histopathology and immunologic studies were essential in properly diagnosing this patient. Our case supports that annular blistering lesions can be a clinical variant of bullous pemphigoid.

A case of cutaneous necrosis due to intra-articular hyaluronic acid and treated with hyaluronidase.

A 66-year-old woman presented to the hospital with cutaneous necrosis of her right ankle and foot. Her symptoms began immediately after an intra-articular injection of hyaluronic acid for ankle osteoarthritis, which was performed 6 days before. Histopathology showed an intra-vascular hyaluronic acid embolus. The initial treatment approach was conservative, but the patient's clinical state degraded. She was thus treated with sub-cutaneous hyaluronidase, the enzyme that degrades hyaluronic acid, which yielded a moderate improvement even though it was administered 22 days after the initial hyaluronic acid injection. Although hyaluronic acid embolism and subsequent cutaneous necrosis are well-known complications of dermal fillers, there are few reported cases of embolism following intra-articular injection. To our knowledge, this is the first time hyaluronidase has been used in this setting.

Acute generalized exanthematous pustulosis simulating toxic epidermal necrolysis: case presentation and literature review.

BACKGROUND: Acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions. These rare conditions differ in clinical presentation, pathological features, treatment and prognosis, but overlap has been described implying a challenging clinical management. CASE PRESENTATION: We describe a case of overlap between TEN and AGEP probably secondary to beta-lactams in a 77-year-old patient treated for a complicated cholangitis. We review the diagnosis and the management of these two conditions. The diagnosis of TEN was suggested by the initial clinical presentation with severe hemodynamic instability, skin detachment, positive Nikolsky sign and mucosal involvement. However, the skin biopsy as well as the rapid improvement of the skin lesions were discriminative for AGEP. This indicated an overlap presentation. Unfortunately, the patient refused allergy investigations in order to find the culprit drug. Medical photographs, proper physical examination and histopathological results are integrated. CONCLUSION: Despite clinical features indicating a diagnosis of TEN, histopathology was conclusive for AGEP thus indicating a possible clinical-pathological overlap between the two conditions, a scarcely described situation in the medical literature. To our knowledge, this is one of the few cases that portrays a TEN-AGEP overlap probably secondary to Piperacillin Tazobactam. Understanding the immunological implications of these conditions can help us better distinguish and manage these severe reactions.

Cutaneous plasmacytosis and multinucleate cell angiohistiocytoma-like lesion in a patient with hepatitis B: A fortuitous triad?

A 28-year-old woman of Chinese descent, with congenital chronic hepatitis B presented with a 7-year history of erythematous-brown papules and plaques on her groins, axillae, and forehead. A first skin biopsy showed findings consistent with two concomitant, yet highly uncommon cutaneous diseases. The presence of lymphoid nodules with germinal centers and clustered polyclonal plasma cells was consistent with cutaneous plasmocytosis. Second, a diffuse proliferation of non-atypical small vessels (CD31+, CD34+, and HHV8-) in a hypercellular stroma peppered with angulated giant cells (CD163+, CD68-) was suggestive of multinucleate cell angiohistiocytoma (MCAH). Interestingly, the second biopsy of a different plaque on the forehead showed only plasmacytosis and the clinical appearance of both plaques and papules alluded to the distinct presence of both concurrent entities. We speculate the immune modulating effects of chronic hepatitis B may have led to a polyclonal plasmacytic proliferation within the dermis. Furthermore, MCAH has been reported in conjunction with other inflammatory skin diseases such as hidradenitis suppurativa and as such we propose that the MCAH lesion in our case may have arisen as a secondary, reactive process to the cutaneous plasmacytosis.

Atypical presentation of adenosquamous carcinoma: A case report.

Cutaneous adenosquamous carcinoma is a rare malignant neoplasm that is more aggressive than conventional squamous cell carcinoma. The typical clinical presentation is an indurated papule or plaque on the head and neck of elderly patients. The authors report the case of a 52-year-old man with a right scrotal and inguinal tumour measuring 10 cm × 15 cm that had progressed over the past 2 years. The histological examination was compatible with adenosquamous carcinoma. Metastatic inguinal and pelvic lymph nodes were identified. This case demonstrates an atypical presentation of a rare tumour. Adenosquamous carcinoma is more aggressive than conventional squamous cell carcinoma, and prompt diagnosis is important.

A secondary syphilis rash with scaly target lesions.

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.

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.

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, non-necrotizing 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.

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.

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.

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 7(1/2)-month follow-up, the patient remained completely asymptomatic.