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Porphyria cutanea tarda (PCT) is characterized by a skin blistering eruption that develops in sun exposed areas of the skin. It is the most common cutaneous porphyria world-wide, and classically associated with hepatic injury but also estrogen use, cigarette smoking, and HIV. In any case of photodistributed persistent blistering skin condition, PCT must be high on the differential. This case of a carpenter diagnosed with PCT not only illustrates a classic case but also the opportunity to achieve significant response to therapy in a motivated patient particularly with improved access to direct-acting antivirals (DAA) for hepatitis C treatment.

Autoimmunity Related Neutrophilic Dermatoses (ARND) is a recently described entity characterized by urticarial papules and plaques with histology showing a neutrophilic perivascular and interstitial infiltrates with leukocytoclasis, along with variable vacuolar interface dermatitis. We report a 38 year old Caucasian female with a ten year history of lupus who presented with pruritic urticarial papules and plaques on the face, trunk, upper extremities and thighs. The onset occurred 10 days after she started a calcium channel blocker (CCB), diltiazem. Histopathology revealed scattered dyskeratotic keratinocytes, vacuolar interface dermatitis, and a sparse perivascular and interstitial mixed infiltrate of neutrophils within the dermis. There was focal leukocytoclasis, dermal edema, and rare eosinophils. The patient initially improved with prednisone taper, but flared again upon starting a different CCB, verapamil. Exposure to certain medications such as CCB in the setting of autoimmune connective tissue disorder may be the inciting trigger for a neutrophilic dermatoses. Future studies may provide further information on the pathogenesis of ARND and cutaneous drug eruption in the setting of SLE.

Diabetes is associated with many complications, including foot ulcers. Individuals with diabetes have a 15% to 25% likelihood of developing a foot ulcer in their lifetime. The pathophysiologic mechanisms are multifactorial but the major etiologic factors are peripheral vascular disease and diabetic neuropathy. Foot examinations are recommended at least annually for patients with diabetes to assess the risk of foot ulcers and to detect, diagnose, and manage them. Management includes avoidance of walking, weight-bearing limitation, use of therapeutic footwear, use of dressings and debridement, and antibiotics. Due to immune dysfunction, diabetic neuropathy, and poor circulation, patients with diabetes are at increased risk of other types of infections. These include erythrasma, candidiasis, paronychia, onychomycosis, necrotizing fasciitis, Fournier gangrene, otitis externa, and nontuberculous mycobacterial skin infections. A high index of suspicion is required to diagnose these conditions. Patient evaluation may include a detailed physical examination, imaging, laboratory tests, and biopsies and cultures. Management may involve mechanical or surgical debridement, topical and oral antibiotics, and abscess drainage.

During summer of 2018, ME to WE organized a dermatology volunteer trip in Masai Mara region of Kenya. Our volunteer group included physicians, physician assistants and medical assistants. We volunteered at the Baraka Hospital along with the local staff there. Our goal was to help treat and triage patients with skin conditions and in the process educate local staff about Dermatology. We saw patients with a variety of skin conditions, including Filariasis, Onchocerciasis, Chromoblastomycosis, Discoid Lupus and others. In addition, we visited local schools to screen students with common skin conditions. We saw approximately 800-100 patients during our time there. On our last day at the hospital we organized lectures to teach the local team about common skin conditions. The key challenges to our work there were limited resources and time available, socio-economic difficulties of the local population, lack of an established dermato-pathology center, etc. The key strengths of the trip were very well organized trip from ticketing to lodging to itinerary, very supportive local staff and how grateful patients were with evaluation of their conditions. Our trip also included local cultural programs that allowed us to learn more about the people there. In a short span of one week, we were able to help in the care of the local patient population and in the process learn about local culture, socio-economic structure and medical practice there.

Background: Perineural invasion (PNInv) is a significant risk factor for metastasis and death in cutaneous squamous cell carcinoma (cSCC). Despite this known association, factors contributing to the presence of PNInv are not well characterized.Aims: To determine risk factors associated with the presence of PNInv using the high-risk cSCC criteria developed by the National Comprehensive Cancer Network (NCCN).Methods: After receiving Institutional Review Board approval for this retrospective review, the presence of NCCN high-risk factors for cSCC were recorded for patients treated at a tertiary referral academic medical center, from January 1, 2010 to March 31, 2012. Stepwise logistic regression was used to identify factors associated with the presence of PNInv.Results: PNInv was present in 34 of 507 cSCCs (6.7%). Moderately or poorly differentiated histology (P < .001, OR 6.6 [95% CI, 3.2-13.7]), acantholytic, adenosquamous, or desmoplastic subtype (P =.01, OR 1.8 [95% CI, 0.8-4.2]), and tumors in areas M (≥10mm) and H ( ≥6mm) (P = .05, OR 5.0 [95% CI, 1.2-21.0]) were significantly associated with the presence of PNInv.Conclusions: This data suggests clinicians should have a higher suspicion and may be able to identify PNInv in high-risk cSCC based on the presence of specific high-risk factors.

The association between guttate psoriasis and infection with group A Streptococcus (GAS) has been well established in the medical literature. However, responses to treatments aimed at GAS eradication such as systemic antibiotics or tonsillectomy are inconsistent. Further complicating treatment recommendations for a disease with a suspected microbial trigger, the standard therapy for severe psoriasis is with systemic immunosuppressant medications. This case report illustrates the role of GAS as a trigger for acute onset severe psoriasis in a child whose skin disease initially worsened with a trial of methotrexate. An immune evaluation confirmed a co-existing selective antibody deficiency. Subsequent treatment with intravenous immune globulin dramatically improved his underlying immune function and decreased GAS infections. This improvement in overall immune function and decrease in GAS infections cleared his skin disease. An interval change in formulation to subcutaneous immune globulin was not as effective.

Background : Cutaneous squamous cell carcinoma (cSCC) is the most common cancer capable of metastasis. Due to its high incidence and lack of inclusion in national databases it has been difficult to identify high-risk factors associate with metastasis. The development of a cSCC metastatic risk model would help physicians identify patients who are at risk for metastasis, and would allow for the initiation of early aggressive management to improve outcomes. Aim : Explore different statistical approaches to develop a model to predict cSCC metastasis that is accurate and reflects routine clinical practice. Methods : All cSCCs diagnosed and treated at Saint Louis University from January 2010 to March 2012 were included. Three statistical approaches were studied: multivariable logistic regression (MLR), pattern classification (PC) and sum score method (SSM). Two models using the SSM were created with a different number of factors used to merit assignment to the metastatic cohort: 2 factors (S2) or >2 factors (S2+). For each model, sensitivity (SN), specificity (SP) and positive predictive value (PPV) were calculated. Results : SN, SP, and PPV for each model were: MLR: SN 4.3%, SP 97.4%, PPV 16.0%; S2: SN 78.3%, SP 83.7%, PPV 12.5%; S2+: SN 60.9%, SP 96.5% PPV 34.1%; PC: SN 73.9%, SP 95.9%, PPV 34.7%. Conclusions : The PC model was the most accurate. The S2+ model had a lower SN, but would be easier to implement as clinicians would only have to sum high-risk factors.

Wet wrap therapy is a well-known, effective short-term treatment for inflammatory skin conditions such as atopic dermatitis and psoriasis. The traditional wet wrap technique involves application of a corticosteroid ointment and emollient followed by a 2-layer mummy-style wrap using roll gauze and vinyl wrap. However, this method is time consuming and labor intensive. On the other hand, commercially-available eczema clothing are expensive. Ordering difficulties and barriers to maintaining a bulk supply resulted in frequent stock depletion, leaving patients and staff no choices other than roll gauze and vinyl wrap. With a grant from the Cardinal Glennon Foundation we designed cost-effective, allergen-free wet wrap garments for pediatric patients. The inner layer garments were made from white cotton, chosen for its natural abundance, folding endurance, conduction of heat, cost effectiveness and low allergenicity risk. Nylon was used for the outer layer, chosen for its light weight and moisture retention. All the garments were made without dye, metal, elastic or other allergens. Plain cotton strings were used to secure the waist. The garments were half the cost of branded eczema clothing, based on internal online market research.Hospital staff members involved in directly providing wet wrap treatments have appreciated these garments for ease of use, patient comfort, and durable supply. Future Quality Assessment and Quality Improvement studies to compare the advantages of this treatment with traditional wraps will allow better evaluation of this custom designed clothing

We present the case of a 62-year-old African-American woman with medical history of hypertension and hyperlipidaemia who presented to dermatology clinic for ‘bug bites’. Skin examination showed resolving bullae on the shins and postinflammatory pigment changes. Histopathology showed eosinophilic spongiosis and direct immunofluorescence (DIF) was negative for IgG, IgM, IgA and C3. After returning to clinic with recurrent severe bullous eruptions, the patient presented with anaemia, lymphocytosis, posterior cervical lymphadenopathy and weight loss. An exuberant bite reaction in the setting of lymphoma was suspected. Further workup with haematology revealed elevated IgG level and total protein levels. Flow cytometry showed a B cell lymphoma subtype. Extensive imaging was positive for diffuse lymphadenopathy, with accompanying evidence of Ebstein-Barr virus infection. Our case highlights the importance of considering exuberant arthropod bite reaction in the setting of undiagnosed lymphoma in a patient with bullous eruption and negative DIF.

The molecular basis of disease heterogeneity in autoimmune conditions such as Pemphigus vulgaris is poorly understood. Although desmoglein 3 (Dsg3) has been well established as a primary target of immunoglobulin (Ig) autoantibodies in PV, there remain several questions regarding the overall distribution of anti-Dsg3 Ig subtypes among patient subsets and considerable controversy regarding whether an isotype switch can be observed between phases of disease activity. To systematically address the outstanding questions related to Ig-isotype specificity in PV, we analyzed IgA, IgM, IgG1, 2, 3 and 4 anti-Dsg3 levels by ELISA in 202 serum samples obtained from 92 patients with distinct clinical profiles based on a set of defined variable (activity, morphology, age, duration) and constant (HLA-type, gender, age of onset) clinical parameters, and 47 serum samples from HLA-matched and -unmatched controls. Our findings provide support for earlier studies identifying IgG4 and IgG1 as the predominant antibodies in PV with significantly higher levels in active than remittent patients. We do not see evidence for an isotype switch between phases of disease activity and remission, and both IgG4 and IgG1 subtypes remain elevated in remittent patients relative to controls. We do, however, find IgG4 to be the sole subtype that further distinguishes PV patient subgroups based on different disease morphologies, disease duration, and HLA-types. These data provide further insight into the immune mechanisms responsible for phenotypic expression of disease, and contribute to the broader effort to establish comprehensive immunoprofiles underlying disease heterogeneity to facilitate increasingly specific and individualized therapeutic interventions.

Hidradenitis suppurativa (HS) is a chronic skin disorder of the terminal follicular epithelium of apocrine sweat glands, manifesting as painful and exudative papules, pustules, cysts or nodules. This inflammatory condition often presents with other systemic and cutaneous disorders. We present the case of an African-American man with HS who was also diagnosed with neutrophilic dermatoses and diverticular disease. Neutrophilic dermatosis was identified based on histopathology findings. Our patient underwent multiple surgeries for flaring of his skin condition. Colchicine and doxycycline were started, but the patient was not able to tolerate them. Humira was planned for treatment of HS and neutrophilic dermatosis but could not be pursued because of the pericolic abscess. Colonoscopy and radiological investigation revealed multiple colonic diverticuli, for which he initially underwent percutaneous drainage followed by surgical removal of sigmoid mass and colocutaneous fistula. Culture from the specimen revealed abnormal growth of Actinomyces.

Recent guidelines from the American Joint Committee on Cancer (AJCC) and National Comprehensive Cancer Network (NCCN) have been proposed for the assessment of “high-risk” cutaneous squamous cell carcinomas (cSCCs). Though different in perspective, both guidelines share the common goals of trying to identify “high-risk” cSCCs and improving patient outcomes. Thus, in theory, both definitions should identify a similar proportion of “high-risk” tumors. We sought to evaluate the AJCC and NCCN definitions of “high-risk” cSCCs and to assess their concordance.Methods. A retrospective review of head and neck cSCCs seen by an academic dermatology department from July 2010 to November 2011 was performed.Results. By AJCC criteria, most tumors (n=211,82.1%) were of Stage 1; 46 tumors (13.9%) were of Stage 2. Almost all were of Stage 2 due to size alone (≥2 cm); one tumor was “upstaged” due to “high-risk features.” Using the NCCN taxonomy, 231 (87%) of tumors were “high-risk.”Discussion. This analysis demonstrates discordance between AJCC and NCCN definitions of “high-risk” cSCC. Few cSCCs are of Stage 2 by AJCC criteria, while most are “high-risk” by the NCCN guidelines. While the current guidelines represent significant progress, further studies are needed to generate a unified definition of “high-risk” cSCC to optimize management.

In this report, we present an African-American female patient who presented with haematuria to her primary care physician. The symptoms persisted and coexistent metastatic spindle cell-type melanoma of the kidney and renal cell carcinoma were discovered on further evaluation. No primary site for melanoma was identified. Despite aggressive treatment with ipilimumab, the patient's disease progressed quickly. The patient opted for palliative care and was referred to a hospice. Coexistent melanoma and renal cell carcinoma is exceedingly rare. Melanoma itself is rare in the African-American population. However, when it does present, it usually is at an advanced stage, as was the case in our patient. Since no primary tumour site for melanoma was found and the diagnosis was made after the tumour had metastasised, we think this case highlights the importance of cutaneous cancer surveillance in the non-Caucasian population. Earlier identification of melanoma in this population will help to improve outcomes.

A 60-year-old African-American male patient with a history of seizures, developmental delay, long history of behavioural issues with psychotic episodes, heart, liver, thyroid and kidney diseases presented for evaluation of a right neck skin lesion. Physical examination revealed a shiny purplish-red plaque on the right neck and a thin pink plaque on the posterior neck. The lesions were similar in appearance, but different enough to warrant skin biopsy of each. Pathology demonstrated mycosis fungoides (MF) on the right neck and dermatofibrosarcoma protuberans (DFSP) on the posterior neck. The identification of two rare conditions made us reconsider our diagnosis. After further review, the right neck skin lesion was thought to be anticonvulsant-induced cutaneous lymphoid hyperplasia, not MF. This case demonstrates how insufficient skin biopsy can have significant clinical consequences. Biopsy of the right neck only would have overlooked a DFSP and incorrectly given the patient a diagnosis of MF.