Peter Pavlidakey

Rosai-Dorfman disease successfully treated with thalidomide

Rosai-Dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic condition of unknown etiology first described in 1969.1 Primary cutaneous manifestation without systemic involvement of Rosai-Dorfman disease is even more uncommon.2 Here we present a case of primary cutaneous manifestation of Rosai-Dorfman disease with excellent response to oral thalidomide.

Cutaneous epithelioid angiomatous nodule: a case with metachronous lesions.

Cutaneous epithelioid angiomatous nodule is regarded as a benign vascular proliferation on the spectrum of epithelioid vascular lesions, probably most closely related to epithelioid hemangioma. Most patients present with solitary lesions. We present an unusual case of an 84-year-old man with multiple epithelioid angiomatous nodules that developed over a 1-year period. Given the multiple lesions, an extensive evaluation for a possible infectious etiology was undertaken. However, no evidence of an infectious agent was identified. The histopathologic differential diagnosis for epithelioid vascular lesions is reviewed.

Chronic Granulomatous Dermatitis Induced by Talimogene Laherparepvec Therapy of Melanoma Metastases

Talimogene laherparepvec (TVEC) is the first oncolytic viral immunotherapy approved by the FDA, for advanced melanoma consisting of genetically modified herpes simplex type 1 virus which selectively replicates causing tumor lysis, expressing granulocyte macrophage‐colony stimulating factor (GM‐CSF) and activating dendritic cells. Intratumoral injection of TVEC produces objective response in 41% of stage IIB‐IV M1a melanoma. However, clinical response assessment can be problematic due to immune‐related inflammation at established tumor sites. Herein, we report 5 cases of granulomatous dermatitis developing at sites of TVEC injection associated with pathologic complete response in 4 of 5 patients. Over 5 months, TVEC injections were administrated in a median of 20 tumors per patient for 9 median doses prior to biopsy of persistent, indurated nodules. Granulomatous dermatitis with melanophages and melanin pigment incontinence was observed in all samples without evidence of melanoma cells in 4 patients. The fifth patient was rendered melanoma‐free by resection of the 1 nodule out of 4 with persistent tumor. Repetitive administration of TVEC or other oncolytic viral immunotherapies mimicking unresolved infection can produce granulomatous inflammation confounding assessment of the degree of tumor response and need for additional TVEC therapy. Tumor biopsies are encouraged after 4 to 6 months of TVEC administration to differentiate melanoma from granulomatous inflammation. Patients with confirmed granulomatous dermatitis replace continued with remained in remission after treatment discontinuation. Inflammatory nodules typically regress spontaneously.

Multifocal epithelial sheath neuroma

were observed; thus, we believe that the cytologic pleomorphism may have been part of a degenerative process. The differential diagnosis of PFS also includes dermatofibroma with atypical or monster cells. However, these tumors are often more cellular with areas indistinguishable from typical dermatofibroma and are associated with an acanthotic and hyperpigmented epidermis. Our case did not possess histological features of a dermatofibroma, including a lack of acanthosis and hyperpigmentation in the overlying epidermis. In addition, the lesional cells were positive for CD34. Local recurrence may occur, but there have been no reports of regional or distal spread, though a case of progression into a myxofibrosarcoma over a 21-month period has been reported. The standard treatment of PFS remains surgical excision followed by histopathological evaluation. To avoid misdiagnosis, PFS should be considered in the differential diagnosis of an elastic hard intradermal nodule.

A case of scurvy associated with nilotinib.

To the Editor , Nilotinib is a second-generation tyrosine kinase inhibitor (TKI) approved to treat adults with Philadelphia chromosome-positive chronic myelogenous leukemia (CML).1 Cutaneous reactions are some of the most common nonhematologic adverse effects secondary to TKI use, and they appear to be dose-related.2 A recent meta-analysis revealed an incidence of 34.3% for all-grade rash with nilotinib use.1 Nilotinib-associated dermatoses have diverse clinical presentations, including milia-like, morbilliform, scaling erythematous, and keratosis pilaris-like morphology.1 Herein, we describe a patient on nilotinib who developed a keratosis pilaris-like eruption and was subsequently diagnosed with scurvy. A 66-year-old white male was diagnosed with chronic-phase Philadelphia chromosome-positive CML in 2014 and was started on nilotinib at 300 mg twice daily shortly thereafter. Although the treatment with nilotinib resulted in complete molecular remission, the patient developed keratotic papules with perifollicular erythema on the chest and abdomen approximately 7 months after initiation of treatment (Fig. 1). The patient reported that the rash felt rough and was pruritic at times. The patient denied any changes in medications around the time when he first noticed the rash and stated that he consumed a balanced diet that included fruits and vegetables. On physical examination, there was follicular prominence with central keratotic cores with a rim of erythema on chest, abdomen and back. There was evidence of a few scattered corkscrew hairs in a similar distribution. There was no evidence of gingival bleeding or other systemic symptoms. Fig. 1. Clinical image shows follicular prominence with central keratotic cores and a rim of erythema on abdomen.

Leishmaniasis Panamensis Masquerading as Myiasis and Sporotrichosis: A Clinical Pitfall

We report a case of cutaneous leishmaniasis panamensis in nonendemic Costa Rica. A 19-year-old female presented with nonhealing, unilateral eruption of erythematous papules with superficial central ulceration in a sporotrichoid pattern on right upper arm and back. Given the clinical picture and geographic locale, the patient was initially diagnosed with myiasis or human botfly infestation; however, the sporotrichoid pattern of the bites is an unlikely finding in myiasis. Peripheral blood smear, Giemsa stain, and polymerase chain reaction (PCR) were consistent for Leishmania spp. Ulceration resolved with 20-day course of IV sodium stibogluconate.

Keloidal Scleroderma: Case Report and Review

Objective. We report a rare case of keloidal scleroderma and provide an analysis of similar cases. Results. A 41 year-old woman presented with dark brown, indurated, exophytic nodules over the chest along with smaller hyperpigmented plaques scattered over the abdomen, with concomitant sclerodactyly. The clinical, laboratory, and pathological findings were consistent with a diagnosis of keloidal scleroderma. The patient was treated with methotrexate, resulting in reduced firmness of her plaques and no new lesions. A literature review of previously reported cases was performed using keywords including keloidal morphea, keloidal scleroderma, nodular morphea, and nodular scleroderma. In our review, the majority of patients were African American and female. 91% of cases had nodular lesions with distribution on the trunk. The majority of patients exhibited sclerodactyly and pulmonary involvement was reported in 28%1. The majority of patients were ANA positive (63%) and only 10% demonstrated anti-SCL-70 positivity. Conclusion. Keloidal scleroderma is a rare presentation, which can often be clinically confused with keloid and scar formation. Due to this being a rare variant, our knowledge of treatment options and efficacy is limited. Methotrexate could be considered as an initial treatment option for patients with progressive keloidal scleroderma.

Inflammatory melanoma in transit metastases with complete response to talimogene laherparepvec

TVEC: Talimogene laherparepvec INTRODUCTION Inflammatory melanoma was first described in 1984 in 2 patients presenting with nodular cutaneous melanomas resected from the back followed 4 to 12 months later by recurrence in the form of diffuse, indurated, erythematous skin 20-25 cm in diameter with scattered nodularity. Microscopic examination found melanoma cells in the dermis and dermal lymphatics. Both patients died of hematogenous metastases within 6 months of their inflammatory melanoma recurrences. Although histopathologic examination of melanoma frequently finds an associated inflammatory cell infiltrate, gross clinical inflammation is extremely rare. A search of PubMed and Google Scholar performed on August 5, 2016 found 3 additional case reports of inflammatory melanoma, 2 of which involved direct dermal lymphatic extension from grossly involved lymph nodes. Here we report the first, to our knowledge, successfully treated case of surgically incurable inflammatory melanoma with emphasis on molecular and immunophenotypic characterization.

Two Cases and a Review of Graft-Versus-Host Disease and the Role of Hepatitis C Treatment in Liver Transplant Patients

Graft-versus-host disease (GvHD) is a rare but fatal complication after solid organ transplantation arising in 1% to 2% of cases. We report 2 cases of GvHD after orthotopic liver transplantation. Both patients had a history of hepatitis C virus (HCV) infection prior to transplantation. Both cases presented between 1 and 4 months after transplantation with rash, pancytopenia, and/or diarrhea. Our second case also developed oral and ocular manifestations after liver transplantation, which are more commonly described after stem cell transplantation. Diagnosis in both cases was made by clinical presentation in conjunction with histopathology and flow cytometry. Both patients were treated by increasing immunosuppression with tacrolimus and high-dose steroids. Response to treatment differed based on the degree of pancytopenia. Our case report is distinguished by several factors such as the context of GvHD presentation and the role of HCV treatment. Diagnosis of GvHD is difficult and often delayed due to nonspecific presentation that overlaps with other conditions. Furthermore, the relation between HCV treatment and potential initiation of GvHD in solid organ transplant patients is unclear.

Pediatric ocular lichen planus and lichen planopilaris: One new case and a review of the literature

When lichen planus involves the scalp, it is known as lichen planopilaris, and when it involves the eye, it is known as ocular lichen planus; both are rare. Early detection and targeted therapy are crucial in preventing hair loss and scarring conjunctivitis. Little is known regarding appropriate treatment for lichen planopilaris. The objective of this case study is to present a new case of pediatric ocular lichen planus and lichen planopilaris and to identify all reported cases of pediatric lichen planopilaris, highlighting disease involvement, treatment, and response to treatment.