Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion and prominent epidermal involvement: A case report.

Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion (CMCT) is a recently described entity with only 13 cases reported in the literature. Histopathologically, the neoplasm consists of atypical epithelioid to spindled cells that form a well-circumscribed nodule usually confined to the dermis and subcutis with cytological features including large vesicular nuclei with prominent nucleoli and abundant eosinophilic cytoplasm. Immunohistochemistry shows variable expressivity of melanocytic markers. Currently, there are limited data regarding long-term outcomes of this newly described entity. Most cases have done well, but there is one case reported with an adverse event. Hence, further studies are needed to accurately classify this tumor. Definitive diagnosis is made by laboratory evidence of CMCT. Herein, we report the first case of CMCT with epidermal involvement in the youngest patient known to be affected to date.

A case of idiopathic acquired leukonychia totalis in a 17-year-old boy.

Whitening of the nail, or leukonychia, can have a wide range of etiologies including genetic disorders, trauma, poisoning, autoimmune disorders, and infections. Here we detail a case of idiopathic acquired leukonychia totalis in a 17-year-old boy. This condition has been reported 13 times in the literature previously, with only young boys being affected. Proper diagnosis may help minimize unnecessary investigations and prevent additional psychological stress over whether an underlying disease is present.

A case of primary cutaneous Ewing sarcoma in a neutropenic patient.

Primary cutaneous Ewing sarcoma is a rare clinical presentation of Ewing sarcoma, usually occurring as a small, localized tumor on the extremities of young adults and associated with favorable prognosis. We report a case of primary cutaneous Ewing sarcoma, which presented on the sole of the foot of a 27-year-old patient with relapsed acute myeloid leukemia and neutropenia. Diagnosis was determined through histological features and staining, as well as fluorescence in situ hybridization and molecular testing. The patient underwent wide-local excision with plan to begin targeted chemotherapy, but unfortunately died from adenovirus pneumonia while neutropenic before targeted chemotherapy was initiated.

Hypocellular Plaque-Like CD34-Positive Dermal Fibroma (Medallion-Like Dermal Dendrocyte Hamartoma) Presenting as a Skin-Colored Dermal Nodule.

Plaque-like CD34-positive dermal fibromas, also known as medallion-like dermal dendrocyte hamartomas (MDDHs), are a recently recognized group of congenital and acquired spindle cell neoplasms that may appear histologically similar to dermatofibrosarcoma protuberans. Recognizing the clinical heterogeneity of this neoplasm and the subtle pathologic differences are crucial to making the correct diagnosis and avoiding the aggressive surgical intervention required to treat a dermatofibrosarcoma protuberans. We present the case of a 9-year-old girl with an acquired variant of a plaque-like CD34-positive dermal fibroma without clinical epidermal change. Our case expands the clinical spectrum to include an acquired variant of a plaque-like CD34-positive dermal fibroma without clinical epidermal change. Examination of more cases is needed to determine whether all clinical variants are truly subtypes of the same neoplasm or represent distinct CD34-positive spindle cell proliferations.

Primary Cutaneous Polymorphic EBV-Associated Posttransplant Lymphoproliferative Disorder After a Renal Transplant and Review of the Literature.

IMPORTANCE: Posttransplant lymphoproliferative disorder (PTLD) is an uncommon complication after solid organ transplants and hematopoietic stem cell transplants. Extranodal involvement in PTLD can involve several organ systems, including the central nervous system, bone marrow, lungs, gastrointestinal tract, and skin. Isolated involvement of the skin without systemic involvement in PTLD is rare. Primary cutaneous PTLD is generally categorized as either cutaneous T-cell lymphomas or cutaneous B-cell lymphomas, with variable Epstein-Barr virus (EBV) positivity. Herein, we describe an exceedingly uncommon case of a polymorphic variant of primary cutaneous PTLD. OBSERVATIONS: A woman in her 30s, who received an EBV+ deceased donor kidney transplant, presented with a 2-week history of 2 indurated patches over the lower abdomen. A skin biopsy revealed an atypical lymphoid proliferation with immunohistochemical stains demonstrating a mixed population of both B and T cells that stained strongly positive for EBV-encoded RNA. A bone marrow biopsy and positron emission tomography/computed tomography were negative for systemic involvement. The patient was treated with immunosuppression reduction and rituximab infusions. CONCLUSIONS AND RELEVANCE: This case highlights a rare polymorphic variant of primary cutaneous EBV-associated PTLD and increases awareness of this uncommon posttransplant complication. Cutaneous PTLD is reviewed, therefore dermatologists are aware of this uncommon disorder.

Histiocytoid Sweet syndrome with haloed myeloid cells masquerading as a cryptococcal infection.

: Histiocytoid Sweet syndrome is a histopathologic variant of Sweet syndrome characterized by an infiltrate of mononuclear cells that have a histiocytic appearance and represent immature granulocytes. The primary histopathologic differential diagnosis for histiocytoid Sweet syndrome includes leukemia cutis and true histiocytic dermatoses. However, it does not usually include a deep mycotic infection. Herein, we describe a case of histiocytoid Sweet syndrome in a 75-year-old man in which histopathologic examination showed a dense dermal infiltrate composed of mature neutrophils and numerous yeast-like mononuclear cells with a surrounding halo, suspicious for cryptococcal organisms. Immunohistochemical and histochemical studies demonstrated expression of myeloperoxidase by the yeast-like forms and an absence of periodic acid-Schiff and mucicarmine staining. Microbiologic culture studies were negative for fungal organisms. The case met all the criteria for Sweet syndrome and, given the cytomorphology of the mononuclear cells with vesicular irregularly rounded nuclei and myeloperoxidase expression, the case was most consistent with histiocytoid Sweet syndrome. The perinuclear haloes in this case most likely represent cytoplasmic vacuolization of the immature, histiocytoid appearing neutrophils secondary to a novel nonapoptotic caspase-independent death pathway. This case highlights the histopathologic similarities that may be present between cryptococcal infection and histiocytoid Sweet syndrome, and the utility of immunohistochemical markers and histochemical staining to differentiate between the two entities.

Acquired epidermodysplasia verruciformis in a child with atopic dermatitis.

A 4-year-old girl with an established diagnosis of atopic dermatitis, previously severe and treated with cyclosporine, developed widespread papules that demonstrated changes consistent with epidermodysplasia verruciformis on biopsy. Human papilloma virus (HPV) typing was performed and was consistent with epidermodysplasia verruciformis-type HPV (type 5). These lesions rapidly resolved with a 2-week course of imiquimod. Rapid resolution and no family history of epidermodysplasia verruciformis make this most consistent with acquired epidermodysplasia verruciformis. This case is the first reported case of acquired epidermodysplasia verruciformis in a child without the human immunodeficiency virus and may be linked to cyclosporine use, which also has never been previously reported.

Botryomycosis of the vulva: a case report.

OBJECTIVE: Vulvar ulcers may be caused by various etiologies including infection, trauma, dermatosis, and cancer. We report a case of a vulvar ulcer caused by botryomycosis. CASE: An 85-year-old woman presented with vulvar itching, pain, bleeding, and ulcerations suspicious for cancer. Biopsies of the ulcers returned without dysplasia or malignancy. She was referred to the tertiary care vulvar vaginal disease clinic. Dermatopathologic reevaluation of pathologic slides diagnosed lichen sclerosus and botryomycosis. The patient was treated with ciprofloxacin for 7 weeks with complete resolution of vulvar ulcerations. CONCLUSION: Botryomycosis should be included in the differential diagnosis of infectious etiology of vulvar ulcers.

Lymphoplasmacytic plaque in children: a report of two new cases with review of the literature.

Lymphoplasmacytic plaque in children has been proposed as a rare, emerging clinicopathologic entity characterized by solitary, extratruncal, asymptomatic papules and plaques that are typically found in healthy young Caucasian females. Biopsy of these lesions reveals a dermal lymphoplasmacytic infiltrate with or without epithelioid granulomas. Two unique patients with lymphoplasmacytic plaque in children are presented in this report, including a 26-month-old female with a lesion on her finger, who represents both the youngest described patient and the first documented with a finger lesion, as well as a 17-year-old young woman with a left thigh lesion, who represents the patient with the longest clinically and histopathologically observed lesion to date. These two additional patients corroborate the experience of lymphoplasmacytic plaque in children in the six previously reported cases and further expand the clinicopathologic spectrum of the disease. Recognition of lymphoplasmacytic plaque in children is important to facilitate distinction from potential differential considerations, including lymphoproliferative disorders and infectious conditions, particularly as the experience to date appears to suggest that lymphoplasmacytic plaque in children represent a reactive (pseudolymphomatous) condition.

Atypical intravascular CD30+ T-cell proliferation following trauma in a healthy 17-year-old male: first reported case of a potential diagnostic pitfall and literature review.

Herein, we present the first report of a reactive atypical intravascular CD30+ T-cell proliferation. Our patient developed the condition after trauma, and he has followed a benign clinical course. This observation represents a potential diagnostic pitfall for intravascular lymphoma and adds to the list of reactive conditions that may be associated with an atypical CD30+ T-cell infiltrate.

Sclerotic bodies in nephrogenic systemic fibrosis: a new histopathologic finding.

Nephrogenic systemic fibrosis (NSF--known previously as nephrogenic fibrosing dermopathy) is a systemic disorder observed exclusively in patients with a history of kidney disease associated with renal failure. Reported histopathologic findings of NSF include spindle-shaped fibroblast-like cells with fibrosis, thickened collagen bundles with surrounding spindled and epithelioid cells, increased number of elastic fibers, sparse inflammatory infiltrate and increased stromal mucin. Two populations of multinucleated giant cells (Factor XIIIa and CD68 positive) have also been observed. We observed the presence of sclerotic bodies with entrapped elastic fibers in two cases of NSF, which we interpreted to be collagenous in nature, a finding not previously reported. These bodies should not be confused with osseous metaplasia previously seen in association with NSF, which show lacunae and cells within the osseous bodies that may or may not be calcified. We did not observe lacunae or cells within the sclerotic bodies in our cases. Furthermore, the sclerotic bodies in our cases stained blue on Masson trichrome, whereas previous investigators observed the osseous metaplasia to be red. We suggest that sclerotic bodies may be an additional clue to the diagnosis of NSF.

A neutropenic child with a purple lesion on the thigh.

Zygomycosis is an aggressive infection by an angioinvasive fungus seen in patients with defects in phagocytosis. We describe a neutropenic child with primary cutaneous Rhizopus infection presenting with a bull's-eye appearance. The patient was treated with aggressive surgical debridement along with amphotericin B followed by skin grafting, with full recovery.