BASCULE syndrome: A report of three new cases with various associations.

Bier anemic spots, cyanosis, and urticaria-like eruption comprise the recently defined BASCULE syndrome. This rare and benign condition has been reported in approximately 15 individuals in the literature. We present three new cases of BASCULE syndrome occurring in adolescents with interesting associations.

The distinctive histopathology of cicatricial alopecia caused by IgG4-related disease.

IgG4-related disease (IgG4-RD) is characterized by masses at multiple sites, a dense lymphoplasmacytic infiltrate containing numerous IgG4+ plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We present a third case of alopecia (in this instance, cicatricial) caused by IgG4-RD. Based on our findings combined with those seen in two other cases, the histopathologic features of IgG4-RD alopecia include: sparing of the epidermis, cicatricial (scarring) alopecia with a markedly decreased number of hairs, miniaturization of residual hairs, and total loss of the sebaceous glands. Groups of follicles with their associated sebaceous glands (follicular units) are replaced by an extremely dense infiltrate of lymphocytes and especially plasma cells. Histiocytic aggregates, both foamy and non-foamy, may also be present. Variable degrees of fibroplasia may be present but are not an important feature in this type of alopecia.

Recurrent melanoma development in a Caucasian female with CDKN2A+ mutation and FAMMM syndrome: A case report.

Melanoma is a form of skin cancer originating from melanocytes that has an increasing incidence over the past few decades. From 1992 to 2010, the overall incidence of melanoma was 12.29 cases per 100,000 person-years in Canada. Approximately 10% of cases are attributed to a hereditary component, with one of the most common being familial atypical multiple mole melanoma syndrome. In this case report, we highlight the atypical case of a middle-aged Caucasian female with familial atypical multiple mole melanoma syndrome, who has developed dozens of primary melanomas over the past decade. We highlight the management of her case, as well as the importance of monitoring by multiple other subspecialists given the propensity for the development of alternate malignancies.

A Case of Hailey-Hailey Disease Managed With Oral Magnesium Citrate and High-Dose Vitamin D3.

Hailey-Hailey disease, or benign familial pemphigus, is a rare blistering disease originally described in 1939. The disease is due to an autosomal dominant mutation in the ATP2C1 gene on chromosome 3, which encodes for an adenosine triphosphate-dependent calcium pump in the Golgi apparatus whose function is to maintain intercellular calcium homeostasis. Common treatments for Hailey-Hailey disease involve calcineurin inhibitors, topical corticosteroids, topical or systemic antibiotics, topical antifungals, ablative lasers, or botulin toxin. In this case report, we highlight a unique case of Hailey-Hailey disease that was resistant to many conventional therapies and ultimately managed with oral magnesium citrate and high-dose vitamin D3.

Structured assessment and followup for patients with hereditary kidney tumour syndromes.

INTRODUCTION: Optimal clinical assessment and subsequent followup of patients with or suspected of having a hereditary renal cell carcinoma syndrome (hRCC) is not standardized and practice varies widely. We propose protocols to optimize these processes in patients with hRCC to encourage a more uniform approach to management that can then be evaluated. METHODS: A review of the literature, including existing guidelines, was carried out for the years 1985-2015. Expert consensus was used to define recommendations for initial assessment and followup. RESULTS: Recommendations for newly diagnosed patients' assessment and optimal ages to initiate followup protocols for von Hippel Lindau disease (VHL), hereditary papillary renal cancer (HPRC), hereditary leiomyomatosis with renal cell carcinoma (HLRCC), Birt-Hogg-Dubé syndrome (BHD), familial paraganglioma-pheochromocytoma syndromes (PGL-PCC), and tuberous sclerosis (TSC) are proposed. CONCLUSIONS: Our proposed consensus for structured assessment and followup is intended as a roadmap for the care of patients with hRCC to guide healthcare providers. Although the list of syndromes included is not exhaustive, the document serves as a starting point for future updates.

Effect of calcipotriene plus betamethasone dipropionate topical suspension on the hypothalamic-pituitary-adrenal axis and calcium homeostasis in subjects with extensive psoriasis vulgaris: an open, non-controlled, 8-week trial.

BACKGROUND: The two-compound topical suspension/gel containing calcipotriene plus betamethasone dipropionate is effective and safe in the treatment of psoriasis on the body and scalp within the general psoriasis patient population. OBJECTIVE: To evaluate the systemic effects of once-daily use of two-compound topical suspension/gel on the hypothalamic-pituitary-adrenal (HPA) axis and calcium homeostasis in subjects with extensive psoriasis vulgaris. METHODS: An open-label, single-group, 8-week trial in 43 subjects with extensive psoriasis covering 15-30% of the body surface area. Blood and 24-hour urine samples were collected and a standard-dose adrenocorticotropic hormone (ACTH) stimulation test was performed at baseline, weeks 4 and 8. Primary endpoints were serum cortisol 30 minutes after ACTH injection (HPA axis response abnormal at serum cortisol ≤18 µg/dL) and changes from baseline in albumin-corrected serum calcium (sCa), 24-hour urinary calcium excretion (24hCa) and urine calcium:creatinine ratio (Ca:Crea). RESULTS: Two (4.7%) subjects showed signs of adrenal suppression based on the ACTH stimulation test results at week 4; both were withdrawn from treatment and had normal serum cortisol 30-minute values at follow-up 4 weeks later. None of the subjects who continued treatment to week 8 showed signs of adrenal suppression. There were no clinically relevant mean changes from baseline to weeks 4 and 8 in sCa, 24hCa or Ca:Crea and no subject had sCa above the reference range. CONCLUSION: The two-compound topical suspension/gel containing calcipotriene plus betamethasone dipropionate may be applied once daily to extensive psoriasis vulgaris without generally causing adrenal suppression or disturbance of calcium homeostasis, consistent with previous findings. In a small number of patients with extensive psoriasis treated with large volumes of topical suspension, adrenal suppression may be observed. In the real-world setting, it is anticipated that systemic side-effects would occur in only a few cases within the general psoriasis patient population. ClinicalTrials.gov Identifier:

The man who got too close to his cows.

Trichophyton verrucosum is one of the more common chronic zoophilic dermatophytes. Its presentation is diverse, including aggressive inflammatory skin lesions, and it can be accompanied by constitutional symptoms, such as fever and lymphadenopathy. As such, it may be misdiagnosed as bacterial cellulitis and undergo multiple antibiotic courses unsuccessfully, without consideration of a fungal skin infection. We present a cattle farmer presenting with an extensive erythematous plaque that failed to respond to antibacterial treatment. History revealed that his cows were being treated for "ringworm". His diagnosis was confirmed by skin biopsy demonstrating fungal elements and culture yielding T. verrucosum. The farmer was successfully treated with itraconazole.

Topical tacrolimus in the treatment of erosive pustular dermatosis of the scalp.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is a rare condition characterized by chronic, sterile, pustular erosions leading to scarring alopecia. Although the etiology is unknown, it appears to be associated with ultraviolet light exposure and trauma. Histologic findings include nonspecific atrophy of the epidermis and chronic inflammation. CASE HISTORY: A 71-year-old female presented with a 1-year history of a boggy, erythematous, friable plaque on the vertex of her scalp. A diagnosis of EPDS was made based on presentation, negative cultures, and histologic findings. Initial therapy with intralesional and topical steroids and oral antibiotics resolved the inflamed plaques; however, steroid-induced atrophy became prominent after 5 months of use. The treatment was discontinued, resulting in recurrence of disease. Topical tacrolimus 0.1% ointment was initiated, which has been successfully controlling the lesions with reversal of skin atrophy and clinical evidence of hair growth. CONCLUSION: This is the fourth reported case of the successful treatment of EPDS with topical tacrolimus for the resolution of atrophy and the prevention of relapse of inflammation. Although its long-term use warrants close follow-up for side effects, tacrolimus may constitute a novel therapeutic option for the treatment of EPDS.

Allergic contact dermatitis to propolis.

A 35-year-old Asian woman was referred to the dermatology clinic with a 2-week history of enlarging, fluid-filled, pruritic lesions on the right foot. The affected area had a recent history of minor trauma for which the patient applied an over-the-counter propolis ointment. At presentation, the patient was also noted to have been using the following, as prescribed by her primary care physician: valacyclovir, ciprofloxacin, terbinafine cream, mupirocin ointment, and 2% hydrocortisone cream. No clinical improvement was observed with these agents. Examination revealed grouped erythematous papules progressing into vesicles and bulla on the lateral side of the right foot. A KOH scraping was negative. We diagnosed the patient with allergic contact dermatitis to propolis.

Kikuchi-Fujimoto's necrotizing lymphadenitis in association with discoid lupus erthematosus: a case report.

BACKGROUND: Kikuchi-Fujimoto's necrotizing lymphadenitis (KFNL) is a rare, benign, self-limited condition characterized by constitutional symptoms, lymphadenopathy, and skin lesions. OBJECTIVE: We report a case of KFNL in a 43-year-old East Indian woman with a ten-year history of discoid lupus erythematosus (DLE) of the scalp and a three-month history of a erythematous plaque on the left nasal bridge, cervical lymphadenopathy, and fever. Skin biopsy samples were taken from the face and lymph node. RESULTS: Histopathological examination of the skin revealed a mixed infiltrate of inflammatory cells, nuclear dust, and histiocytes phagocytosing nuclear debris in the reticular dermis. The lymph node showed interfollicular liquefactive necrosis, immunoblasts, and a similar cellular infiltrate as the skin. The non-necrotic areas demonstrated follicular hyperplasia. These pathological changes are associated with a diagnosis of KFNL. CONCLUSIONS: KFNL is reported in association with systemic lupus erythematosus, but only two other cases of systemic KFNL in association with DLE exist in the literature. This case is unique in that the patient presented with cutaneous and systemic KFNL in the setting of longstanding DLE.

Fatal squamous cell carcinoma arising from transplant-associated porokeratosis.

Porokeratosis is a disorder of epidermal keratinization characterized by variably sized plaques with central depression and a well-demarcated keratotic border. Associations of porokeratosis with immunosuppression and of porokeratosis with malignancy have been observed. The authors report a case of fatal metastatic squamous cell carcinoma arising from porokeratosis in an immunosuppressed patient.

Intraepidermal neutrophils--a clue to dermatophytosis?

BACKGROUND: Dermatophyte infections are occasionally diagnosed by histopathology. Spongiotic or psoriasiform features are typical but non-specific, and neutrophils may be present within the stratum corneum. Traditionally, this latter finding has been felt to be a diagnostic clue for dermatophytosis, and usually precipitates a periodic acid Schiff (PAS-D) stain to confirm the presence of hyphae in the stratum corneum. Our objective was to evaluate whether the presence of neutrophils within the stratum corneum is a sensitive or specific test for dermatophytosis. METHODS: We performed a retrospective case-control study on 303 cases of spongiotic or psoriasiform dermatitides over a 35-month period. Hematoxylin and eosin (H&E) and PAS-D stains were utilized to identify intraepidermal neutrophils and fungi. RESULTS: The sensitivity and specificity for diagnosing dermatophyte infection based upon neutrophils within the stratum corneum were 62 and 59%, respectively. The positive and negative predictive values in our population were 4 and 98%, respectively. CONCLUSION: The histologic feature of neutrophils within the stratum corneum is neither sensitive nor specific in the diagnosis of dermatophytosis.

The skin in pregnancy.

BACKGROUND: Physiologic skin changes are common during pregnancy due to a temporary shift in hormonal, metabolic, and immunologic factors. Physicians may mistake normal skin changes in pregnancy as pathologic change within the skin, and so an appreciation of the common and less common skin manifestations will assist in appropriate patient care. OBJECTIVE: This review highlights the normal physiologic skin changes in pregnancy, which include pigmentary changes, changes to the hair and nails, alterations in glandular activity, vascular and hematological changes, cutaneous tumors, and mucous membrane changes. CONCLUSION: Physiologic changes to the skin in pregnancy can be complex and confusing. Pregnancy can be a stressful time for women so it is imperative that physicians be aware of the normal skin changes during pregnancy in order to prevent unnecessary investigations and treatments.