Jeong Hee Cho-Vega

Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases

Adult T-cell leukemia/lymphoma, an aggressive T-cell neoplasm, is causally linked to human T-cell lymphotropic virus type 1 and based on this association has a distinct geographic distribution. In our United States-based practice, whose population is enriched for immigrants from human T-cell lymphotropic virus type 1 endemic areas, we have identified that a subset of adult T-cell leukemia/lymphoma, in the absence of human T-cell lymphotropic virus type 1 identification, are indistinguishable from other more common T-cell neoplasms. We retrospectively gathered serology results for anti-human T-cell lymphotropic virus type 1/2 antibody in patients diagnosed with T-cell neoplasms at our institution. A total of 220 human T-cell lymphotropic virus type 1/2 positive patients with T-cell neoplasms were identified; 199 (91%) were correctly classified as adult T-cell leukemia/lymphoma or provisionally as peripheral T-cell lymphoma (serology testing pending). Twenty-one cases (9%) were initially misclassified, including the following: 13 presenting with skin +/− peripheral blood involvement and misclassified as mycosis fungoides/Sezary syndrome; 7 with lymphomatous disease, absence of leukemic involvement, and diffuse CD30 expression, misclassified as ALK- negative anaplastic large-cell lymphoma; 1 thought to represent T-prolymphocytic leukemia with TCL-1 gene rearrangement and diffuse marrow involvement. We also present an example of adult T-cell leukemia/lymphoma, which mimicked lymphoepithelioid variant of peripheral T-cell lymphoma also with diffuse marrow involvement. A subset of adult T-cell leukemia/lymphoma can closely mimic a variety of other more common T-cell neoplasms. Due to its extreme clinicopathologic heterogeneity, identification of adult T-cell leukemia/lymphoma requires a high level of suspicion based on patient demographic alone, which should prompt anti-human T-cell lymphotropic virus type 1/2 serology testing in all T-cell neoplasms developing in patients of appropriate demographic. Absence of high level of suspicion, adult T-cell leukemia/lymphoma is easily misclassified.

CD30-negative lymphomatoid papulosis type D in an elderly man.

at the referring institution, revealed lymph node reactive hyperplasia without evidence of malignancy. Fine needle aspiration and flow cytometry of the submental mass and adjacent lymph node, reviewed at our institution, demonstrated a clonal diffuse infiltrate of small B cells, negative for CD23, CD5, cyclin D1, and CD10, and thus consistent with a low-grade B-cell lymphoma. Several months later, she noticed “golf ball– sized” enlargement of the right axillary mass. Excisional biopsy, reviewed at our institution, revealed a malignant epithelioid process in one lymph node (Fig. 1A). Because of lack of an obvious primary malignancy, we used a panel of immunohistochemical studies. Immunohistochemistry demonstrated tumor cell positivity with a melanocytic cocktail and anti-PLAP antibody with a cytoplasmic pattern while being negative for keratin (Figs. 1B, C). Based on the labeling with the melanocytic cocktail and the history of subungual melanoma, we established a diagnosis of metastatic melanoma. The patient underwent completion lymphadenectomy with a total of 2 of 15 axillary lymph nodes positive for melanoma, with a largest tumor deposit of 63 mm. During a follow-up of 10 months, the patient has developed multiple metastases to the lungs, liver, and spleen. She was started with high-dose interferon with minimal response. She recently started with a protocol of ipilimumab and temozolomide. It has been previously proposed that cytoplasmic reactivity of PLAP may represent nonspecific binding of the antibody, particularly because of its reported subcellular localization in the cytoplasmic membrane. However, because both cytoplasmic and membranous labeling is observed in placenta and seminoma, the cytoplasmic expression is more likely to be a result of abnormal subcellular localization of the protein. The only previous mention of alkaline phosphatase in metastatic melanoma was in a histochemical study by Kabat and Furth. They investigated the presence of alkaline phosphatase in both normal and malignant tissues, of which 2 samples were malignant melanoma. Both of these samples failed to demonstrate alkaline phosphatase histochemical reaction. In conclusion, to our knowledge, we report the first case of PLAP positivity in metastatic melanoma. Histopathologists should be aware of this possibility, as PLAP may be used in immunohistochemical panels in diagnostic surgical pathology and dermatopathology and thus may be a possible source of misdiagnosis.

Successful Treatment of Primary Cutaneous Mucormycosis Complicating Anti-TNF Therapy with a Combination of Surgical Debridement and Oral Posaconazole.

Lipid formulations of amphotericin B remain the first-line antifungal therapy for invasive mucormycosis. Posaconazole is an alternative for salvage therapy, but its use as primary therapy is not recommended due to the paucity of clinical data. Here we describe the case of a 57-year-old diabetic woman receiving etanercept and prednisone for the treatment of psoriatic arthritis who developed primary cutaneous mucormycosis after a minor gardening injury. Infection was successfully treated with aggressive surgical debridement followed by a 6-week course of the new delayed-release tablet formulation of posaconazole and temporary withholding of anti-TNF treatment. Primary antifungal therapy with posaconazole can be considered in selected cases of cutaneous mucormycosis.

Multifocal congenital pyogenic granuloma successfully treated with oral propranolol

Disseminated congenital pyogenic granuloma (DCPG) is an uncommon condition. Individual lesions of DCPG share clinical and histologic similarities with infantile hemangioma (IH); endothelial glucose transporter 1 (GLUT‐1), which is highly expressed in IH but generally not in pyogenic granulomas (PG), is an important diagnostic tool. Treatment for DCPG remains difficult. We describe a case of DCPG effectively treated with propranolol.

Bullous CD4+ CD8+ adult T-cell leukemia/lymphoma, a rare diagnostically challenging cutaneous variant

Human T-cell lymphotropic virus-1 (HTLV-1) is a retrovirus that causes adult T-cell leukemia/lymphoma (ATLL), an aggressive malignancy of CD4+ T-lymphocytes. This virus is endemic to the Caribbean, Japan, and regions of South America and Africa.[1] In Miami/ South Florida, whose population is enriched with immigrants from HTLV-1 endemic areas, we have identified multiple cases of ATLL. [2] This article is protected by copyright. All rights reserved.

Giant proliferating trichilemmal cyst arising from a nevus sebaceus growing for 30 years

Nevus sebaceus of Jadassohn, a congenital cutaneous hamartoma, has the potential to develop into various epidermal adnexal‐origin neoplasms. While the most common neoplasms are trichoblastoma or syringocystadenoma, proliferating trichilemmal cysts are exceptionally rare. We report a case of a 63‐year‐old Cuban male with a giant proliferating trichilemmal cyst arising from a nevus sebaceus on the right shoulder which had been growing for 30 years. Proliferating trichilemmal cysts arising from nevus sebaceus cases are difficult to diagnose clinically and histologically as they are very rare and have not been defined by exact diagnostic criteria. Our case creates awareness of this particular tumor in nevus sebaceus and shares clinical and histological diagnostic information that can be used to make a proper diagnosis.

Morbihan Disease Complicated by Dermatosis Neglecta: A Unique Presentation.

Morbihan disease, also referred to as solid facial edema, or rosacea lymphedema, is a rare disorder that involves chronic erythema and solid edema of the cheeks, eyelids, forehead and glabella and may arise as a complication of acne vulgaris or rosacea. Of note, it may be the only initial presenting symptom of these associated diseases. Few cases have been described in the literature, as its first description by Robert Degos in 1957. The condition is characterized by its chronicity, a typical clinical appearance and the lack of specific histopathologic or laboratory findings. The condition may wax and wane but typically does not resolve without treatment. Many cases of this condition tend to be recalcitrant to therapy, with topical and oral antibiotics regimens commonly used for rosacea generally being ineffective. The disease may easily go undiagnosed, as it mimics other more common skin conditions. We present a case of originally undiagnosed Morbihan disease mistaken for an atypical allergic rash, resistant to treatment, and complicated by dermatosis neglecta.

Carcinoma erysipeloides due to primary cutaneous squamous cell carcinoma

Sir, Carcinoma erysipeloides is a rare variant of cutaneous metastasis. Although most commonly seen in patients with breast adenocarcinoma, it has also been associated with adenocarcinoma of the lung, tonsil, parotid, stomach, pancreas, rectum, colon, ovary, prostate and uterus.1 There has been only one case report of carcinoma erysipeloides arising from cutaneous squamous cell carcinoma (SCC) and another case from SCC of unknown origin.1,2 Clinically, carcinoma erysipeloides exhibits sharply‐defined, erythematous, inflammatory papules and plaques with significant induration due to the blockage of dermal lymphatics. Its appearance often mimics erysipelas or cellulitis and poses a diagnostic challenge. In this article, we report a case of carcinoma erysipeloides arising from cutaneous SCC and discuss the clinical, histological and immunohistochemical (IHC) features used to diagnose this condition.

Nail Changes of Systemic Amyloidosis After Bone-Marrow Transplantation in a Patient With Multiple Myeloma.

Report of a Case | A 60-year-old man presented with a 2-year history of nail dystrophy, alopecia, and blisters and milia on his hands. Review of systems revealed weight loss, hoarseness, and dyspnea on exertion. Medical history included cardiac dysfunction, gastroesophageal reflux disease, and bilateral carpaltunnel syndrome. Physical examination demonstrated atrophic scars with erosions and milia on the dorsal hands and diffuse alopecia of the scalp and eyebrows. Onychodystrophy of all 20 nails with longitudinal ridging and onychorrhexis, mimicking lichen planus, was also observed (Figure 1A and B). Skin biopsies from lesions on the hand and scalp demonstrated amyloid deposition. A longitudinal nail biopsy demonstrated amorphous pink deposits in the nail bed and nail matrix dermis and vessels; polarizing light examination revealed apple-green birefringence with Congo red staining, confirming amyloid deposition (Figure 2). The most prominent amyloid deposition was observed in the proximal nail matrix, which was associated with nail plate atrophy. Hematology was consulted, and a bone-marrow biopsy revealed multiple myeloma. The patient underwent 4 rounds of chemotherapy with cyclophosphamide-bortezomibdexamethasone before undergoing an autologous stem-cell

Perieccrine and pericapillary calcification in calciphylaxis.

Implication for health policy/practice/research/medical education Calciphylaxis, also known as calcific uremic arteriolopathy, is characterized by ischemic tissue necrosis secondary to an obliterative vasculopathy. On histological examination, small and medium-sized arteries demonstrate medial calcification, intimal hyperplasia and thrombosis. However, vascular pathology may not be evident when small skin biopsies are examined. Subtle stippled perieccrine calcification revealed by calcium staining (von Kossa) is highly specific for calciphylaxis and can aid in the histological diagnosis of calciphylaxis when vascular calcification is not evident on small skin biopsies.