Eccrine porocarcinoma presenting as a recurrent wart.

Eccrine porocarcinoma (EPC) is an exceedingly rare sweat gland tumor most commonly seen in older patients. Diagnosis of EPC is rare, representing a small percentage of cutaneous malignancies. In the absence of established guidelines, wide local excision (WLE) has traditionally been considered the standard treatment. However, there is growing evidence of increased local recurrence and nodal metastasis associated with WLE. More recently, Mohs micrographic surgery (MMS) is emerging as an effective treatment method with higher cure rates. We report a case of EPC presenting as a recurrent wart in a 36-year-old man that was successfully treated with MMS.

Koebner phenomenon: pemphigus vulgaris following Mohs micrographic surgery.

Koebnerization of pemphigus vulgaris (PV) is an infrequently reported reaction. We present a 65-year-old man with Koebnerized pemphigus vulgaris after Mohs microscopic surgery for a basal cell carcinoma. We present this case to heighten awareness of the phenomenon in the dermatological community.

Cadaveric simulation for improving surgical training in dermatology.

Simulation models are rapidly changing medical education, especially the training of dermatology residents. Various models are available, including cadaveric simulations. Our study evaluates the impact of a cadaveric simulation on the training of dermatology residents. Over a period of three years, cadaveric simulation was shown to increase the surgical knowledge of residents. Residents were more confident in their knowledge of surgical anatomy and also surgical skills. Cadaveric simulation may offer a positive impact on resident training in dermatology.

The utility of full-thickness skin grafts (FTSGs) for auricular reconstruction.

BACKGROUND: Full-thickness skin grafts (FTSGs) are a common repair option on the external ear, but there are few large case series examining graft sublocations, dimensions, and outcomes. OBJECTIVE: We sought to report our experience with FTSGs for repair of postsurgical defects of the external ear. METHODS: We conducted a retrospective review of all FTSGs on the ear performed by 2 surgeons (J. C., 2000-2014; B. C. L., 2007-2014) after clearance by Mohs micrographic surgery at a single institution. RESULTS: A total of 1519 FTSGs on the ear were performed between June 2000 and March 2014. The most common sublocations were the superior helix (38.8%), the crura of the antihelix or scapha (18.9%), and the back of ear/back of helix (15.4%). The overall complication rate was 1.6%, and the most common complication was graft failure (1.2%). LIMITATIONS: Data were collected retrospectively from a single institution. Follow-up beyond 3 months was limited. A standardized assessment tool for aesthetic outcomes was not performed. CONCLUSION: By taking advantage of predictable "pincushioning" and combining with local flaps or cartilage grafts, FTSGs can provide more volumetric replacement than previously described. They reliably preserve the height and complex topography of the ear with a low complication rate.

The Bilateral Dufourmentel Flap for Repair of Nasal Dorsum Defects After Mohs Micrographic Surgery.

BACKGROUND: Single-stage repairs of large nasal dorsum defects risk introducing lower nasal distortion. OBJECTIVE: To describe the authors experience with the "birhombic" flap, a bilateral Dufourmentel rhomboid flap, for repair of nasal surgical defects after Mohs micrographic surgery. MATERIALS AND METHODS: The authors performed a retrospective chart review of patients who underwent birhombic flap repair of the nose by a single physician after Mohs micrographic surgery from 2008 to 2013 at the Medical University of South Carolina. RESULTS: Thirty-eight patients were identified on whom the birhombic flap repair was performed on the nasal dorsum over a 6-year period. There were no significant complications. Alar position remained neutral and nasal profile remained unaltered in all cases. Postoperatively, pulsed dye laser was performed in 8 patients (21%) and dermabrasion in 11 patients (29%). All patients achieved very good or excellent final aesthetic results. CONCLUSION: The birhombic flap is a reproducible, one-stage flap for small to large defects of the nasal dorsum that consistently produces topographic restoration with minimal risk of aesthetic or functional complication. The use of 2 opposing flaps redistributes the secondary defect, thus minimizing the potential for lower nasal distortion when closing the flaps' donor sites.

New world cutaneous leishmaniasis.

A 24-year-old Bangladeshi man presented with a 12-week history of a pruritic papule on his left elbow that had enlarged and ulcerated. He was without any constitutional or systemic symptoms. He reported a history of extensive travel in the two years prior to presentation that included Bangladesh, South and Central America, and Mexico. Histopathologic features were consistent with leishmaniasis. Speciation by the Centers for Disease Control showed L. brasiliensis.

Brooke-Spiegler syndrome.

A 50-year-old woman presented with a long history of multiple, flesh-colored papules and nodules on her central face, neck, and upper shoulders, as well as pink papules on her scalp. They were neither pruritic nor tender, but the patient desired treatment for cosmesis. Histopathologic examination of a representative facial lesion was consistent with a trichoepithelioma and an upper forehead papule was consistent with cylindroma. She reported a positive family history of similar lesions.

Colonic ganglioneuromatous polyposis and metastatic adenocarcinoma in the setting of Cowden syndrome: a case report and literature review.

Cowden syndrome is a rare, autosomal-dominant, multisystem disorder characterized by hamartomatous tissue overgrowth and an increased risk of breast, thyroid, and endometrial cancers. Most of the cases arise from germline mutations of the phosphatase and tensin homologue tumor suppressor gene. An association with colon cancer remains unproven but has been suggested in previous reports. We present the case of a 42-year-old man with colonic ganglioneuromatous polyps and an adjacent colonic adenoma giving rise to a signet-ring adenocarcinoma with lymph node metastases in the setting of Cowden syndrome. Although gastrointestinal polyps are a common feature of Cowden syndrome, reports of ganglioneuromatous polyps and malignant degeneration are rare. Cutaneous features of Cowden syndrome in our patient include "cobblestone" lesions of the tongue and oral mucosa, facial trichilemmomas, multiple acral keratoses, and a storiform collagenoma.

Familial multiple pilomatrixomas as a presentation of attenuated adenomatosis polyposis coli.

Pilomatrixomas are benign follicular tumors that occur most commonly in children. Rare multiple or familial pilomatrixomas have been associated with myotonic dystrophy and other disorders. Although sporadic pilomatrixomas and hybrid cutaneous cysts with pilomatrixoma-like features have been observed in some kindreds with Gardner syndrome, an autosomal dominant form of familial adenomatous polyposis, no definitive association has been made with multiple or familial pilomatrixomas. Here we describe two siblings with multiple pilomatrixomas who were also found to have a family history of colonic adenocarcinoma. Genetic testing revealed a mutation in the 5' portion of the adenomatous polyposis coli (APC) gene, in a region associated with an attenuated APC phenotype. These findings show that multiple pilomatrixomas may be the presenting symptom of patients with APC gene mutations.

A transient epidermolysis bullosa simplex-like phenotype associated with bexarotene treatment in a G138E KRT5 heterozygote.

Basal keratinocyte lysis is the hallmark histopathological finding of epidermolysis bullosa simplex (EBS), a group of rare heritable mechanobullous disorders characterized by intraepidermal blister formation and skin fragility. Over 100 mutations, found predominantly in the genes encoding keratins 5 and 14 (KRT5, KRT14), have been described to account for a variety of clinical subtypes. EBS with mottled pigmentation (EBS-MP) is a rare variant featuring childhood-onset reticulate hyperpigmentation and focal palmoplantar keratoderma, typically associated with a P25L KRT5 mutation. In this report, we present the case of a 77-year-old woman with a history of palmoplantar keratoderma who developed a transient EBS-MP-like phenotype associated with bexarotene treatment for cutaneous T-cell lymphoma. Genetic sequencing revealed a heterozygous G138E KRT5 variant, present in approximately 10% of the European population and only rarely associated with pathology. Bexarotene, which has been reported to alter keratin synthesis, caused vesiculobullous reactions with similar frequency in clinical trials. We propose that the cumulative effect of drug treatment and underlying G138E polymorphism resulted in transient basal keratinocyte lysis in our patient and provides a plausible explanation for this unusual bexarotene side effect.

Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism.

Melanotic schwannoma is a rare markedly pigmented peripheral nerve sheath tumor comprising cells with prominent melanization and schwannian features. The psammomatous variety is associated with Carney complex, a multiple neoplasia syndrome with spotty skin pigmentation. We present the first 2 reported cases of melanotic schwannoma arising in patients with a history of nevus of Ota, a rare dermal melanosis believed to represent a failure of melanocyte migration to the epidermis during embryogenesis. Case 1 involves a 40-year-old woman with a 1.8-cm, deeply pigmented, trigeminal nerve mass and pigmentation of the maxillary sinus mucosa and bone. Case 2 involves a 53-year-old woman with a 1.5-cm mass adjacent to the clavicle. Microscopically, both masses consist of partially encapsulated epithelioid and spindle cells with abundant melanin pigment, arising in association with peripheral nerves. Morphological, immunohistochemical, and ultrastructural features support a diagnosis of melanotic schwannoma. No psammoma bodies are noted, and neither patient exhibits any additional features of Carney complex. Melanotic schwannoma is most often benign but has been associated with malignant behavior in some cases. Distinguishing this nerve sheath tumor from malignant melanoma can be difficult but is of great clinical importance due to differences in prognosis and treatment.