Subepidermal calcified nodule presenting as a cutaneous horn: two cases and a review of the literature.

Subepidermal calcified nodules are an uncommon subtype of idiopathic calcinosis cutis. Morphologically, this entity typically present as a single, well-circumscribed, white-yellow nodule. Based on clinical appearance alone, subepidermal calcified nodules are frequently misdiagnosed and often requires histological confirmation. We describe two cases of subepidermal calcified nodules presenting atypically as cutaneous horns. Subepidermal calcified nodules presenting as a cutaneous horn has rarely been reported; on review, there are fewer than 10 such cases have been described within the past 30 years. The cases described here illustrate the clinical variety and should increase awareness of subepidermal calcified nodules presented.

Improvement of Unilateral Breast Hypoplasia With Oral Spironolactone in a Patient With Becker Nevus Syndrome.

Becker nevus (BN) is a benign cutaneous smooth muscle hamartoma that presents with a hyperpigmented patch or plaque with or without hypertrichosis.1 BN may be associated with ipsilateral breast hypoplasia or other musculoskeletal abnormalities, an association which has been termed Becker nevus syndrome (BNS).

Neutrophils in Fixed Drug Eruptions: Correction of a Mistaken Hypothesis.

ABSTRACT: Classical histopathological findings of fixed drug eruption (FDE) include a lichenoid/interface dermatitis and perivascular infiltrate in the upper and deep dermis composed of lymphocytes and eosinophils accompanied by pigment incontinence. The presence of neutrophils is also an established finding but is less investigated. Sporadic cases of "neutrophilic FDE" have been reported and suggested as a separate entity, a rare variant, or an early stage of the condition. In this article, we report 16 cases of FDE with quantitative analysis showing that neutrophils are relatively common in FDE (68.8%) and that cases with abundant neutrophils had a significantly shorter onset-to-biopsy interval (3.7 vs. 16.9 days, P < 0.023). Our findings support that neutrophilic FDE more likely represents the early phase of FDE rather than a different entity. The presence of neutrophils expands the histopathological differential diagnosis of FDE to include neutrophilic dermatosis, signifying the value of clinical correlation.

Cutaneous metastasis of primary diffuse large B-cell lymphoma of the central nervous system developing 4 years after complete remission: Diagnosis confirmed by comparison of clones.

B-cell lymphoma of the central nervous system (CNS) is a rare malignancy with diffuse large B-cell lymphoma (DLBCL) variant being most common. Although DLBCL has a high propensity to relapse locally within the CNS, only a few cases of cutaneous metastasis have been described in the literature. We present a unique case of cutaneous metastasis of a primary DLBCL of the CNS in a 79-year-old man who was in clinical remission for 4 years until presenting with a lesion in the left adrenal gland and cutaneous nodules on the left flank. Skin biopsy specimen revealed a diffuse dermal infiltrate of atypical B-cell lymphocytes with expression of CD20, BCL-2, BCL-6, and MUM-1, suggestive of DLBCL. For differentiation between another primary or a recurrent process, immunoglobulin kappa (IgK) light chain gene rearrangement was performed and demonstrated that the DLBCL of the skin and CNS were of the same clonal origin. Restaging computerized tomography after initiating chemotherapy and daily ibrutinib showed complete resolution of the left adrenal mass and resolving cutaneous lesions. Our case demonstrates the rare, late cutaneous metastasis of DLBCL of the CNS and highlights the importance of genetic testing for the distinction between the primary and secondary lesions.

Basaloid Follicular Hamartoma: An Additional Criterion of Nevoid Basal Cell Carcinoma Syndrome.

ABSTRACT: Basaloid follicular hamartoma (BFH) is a rare, benign follicular neoplasm which typically presents as brown to skin-colored papules on the face, scalp, and trunk. Histologically, BFH consists of cords and strands of basaloid cells forming cystic structures with scant stroma and should be distinguished from infundibulocystic basal cell carcinoma to avoid overly aggressive treatment. Although BFH has been found to be associated with distinct syndromes, including alopecia, myasthenia gravis, and cystic fibrosis, there is often clinical, histopathologic, and genetic overlap with nevoid basal cell carcinoma syndrome (NBCCS). In this article, we describe a case of a 13-year-old patient with NBCCS who presented with multiple BFHs and propose that it its inclusion into the diagnostic criteria for NBCCS be considered.

Fluorescence microscopy for the evaluation of elastic tissue patterns within fibrous proliferations of the skin on hematoxylin-eosin-stained slides.

BACKGROUND: Diagnosis of fibrous tumors can be challenging and expensive due to the use of special stains. OBJECTIVE: Determine the usefulness of fluorescence microscopy in the evaluation of elastic tissue patterns on hematoxylin-eosin-stained slides. METHODS: In total, 228 slides representing different fibrous tumors were evaluated for their elastic tissue patterns by fluorescence microscopy, and sensitivity and specificity were determined for relevant comparisons. RESULTS: Fluorescence microscopy was found to be useful, especially for distinguishing dermatofibroma from dermatofibrosarcoma protuberans and dermatomyofibroma from other fibrous tumors. LIMITATIONS: In some cases, excessive background staining made patterns difficult to interpret. CONCLUSION: Evaluation of elastic tissue patterns by fluorescence microscopy in fibrous tumors is a cheap and efficient means to further delineate these often challenging tumors.

Spindle cell variant of epithelioid cell histiocytoma (spindle cell histiocytoma) with ALK gene fusions: Cases series and review of the literature.

BACKGROUND: Epithelioid fibrous histiocytoma (EFH) is an uncommon dermal neoplasm expressing anaplastic lymphoma kinase (ALK) protein. Rarely a histopathological variant of this entity exhibits exclusively spindle cells. We report three cases of EFH that do not completely fulfill phenotypic criteria featuring spindle cell morphology and expressing ALK protein. We also analyze the fusion partner genes rearranged with ALK in these cases. METHODS: ALK expression and rearrangement status were evaluated by immunohistochemistry (IHC), fluorescence in situ hybridization (FISH), and next generation sequencing based gene fusion analysis. RESULTS: Three cases, all from females between 25 and 55 years old, have been biopsied from back, left arm, and thumb. All three cases showed tumor with exclusively spindle cell morphology without any epithelioid cells. The tumor cells exhibited strong ALK expression by IHC and FISH study confirmed ALK gene rearrangement in all three cases. DCTN1-ALK fusion was identified in two cases. CONCLUSION: EFH is not always purely epithelioid and its spindled cell variant, spindle cell histiocytoma, should be included in the differential diagnosis of superficial dermal spindled cell neoplasms. ALK immunostain is a useful diagnostic marker for this entity and further studies may be useful to investigate whether DCTN1-ALK fusion mutations are specific to EFH with spindled cell features.

Sweet syndrome with pulmonary involvement in a patient with myelodysplastic syndrome.

We report a patient with Sweet syndrome involving the pulmonary system in the context of myelodysplastic syndrome. Although Sweet syndrome may involve a variety of organ systems, the pulmonary system is rarely affected and can result in poor clinical outcomes, including acute respiratory distress syndrome. Both cutaneous and pulmonary symptoms respond well to systemic corticosteroid therapy and early diagnosis and treatment can improve the prognosis. Our case highlights the importance of collaboration between hematologists, dermatologists, and pulmonologists to facilitate effective diagnosis, triage, and treatment of these patients.

Melanoma Ex Blue Nevus With GNA11 Mutation and BAP1 Loss: Case Report and Review of the Literature.

Cutaneous melanomas may demonstrate a variety of histopathological features and genetic abnormalities. Melanomas that arise in the setting of blue nevi, also known as "malignant blue nevus" or melanoma ex blue nevus (MBN), share a similar histopathological and mutational profile with uveal melanoma. Most uveal melanomas show characteristic GNA11 or GNAQ mutations; additional BAP1 mutation or loss is associated with the highest risk of metastasis and worst prognosis. However, the significance of BAP1 loss in melanomas ex blue nevus remains unclear. We present a case of MBN arising from the scalp of a 21-year-old woman. The diagnosis was established on histopathological findings demonstrating a markedly atypical melanocytic proliferation with increased mitotic activity, necrosis, and a focus of angiolymphatic invasion. Immunohistochemical analysis demonstrated the absence of BAP1 nuclear expression within tumor cells. Next generation sequencing detected GNA11 Q209L mutation and BAP1 loss (chromosome 3p region loss), supporting the diagnosis. We reviewed another 21 MBN cases with reported BAP1 status from the literature. MBN with BAP1 loss presented at a younger average age (41 vs. 61 years), demonstrated larger average lesion thickness (9.0 vs. 7.3 mm), and had a higher rate of metastasis (50% vs. 33%) compared with BAP1-retained MBN. BAP1 expression studies may assist in the diagnosis and management of MBN, but further research is needed.

Historical and Current State of Dermatologic Care for Sexual and Gender Minority Populations.

The lesbian, gay, bisexual, transgender, and queer or questioning/sexual and gender minority (LGBTQ/SGM) community is a growing population with unique lifestyles, sexual practices, beliefs, health issues, and concerns. Although significant advances have been achieved in recent years to establish better care for LGBTQ/SGM patients, they still face insurmountable stigmatization and health care inequality. Dermatologists play an important role in LGBTQ/SGM patients' well-being because they not only treat their skin conditions, but also help them achieve desirable physical characteristics. This article discusses historical perspectives and current state of LGBTQ/SGM dermatology and attempts to define directions for future research and improvement.