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Calciphylaxis is a rare and life-threatening disease that classically manifests with painful skin lesions. It occurs mainly in patients with end-stage renal disease (ESRD) treated with dialysis, has poor outcomes, and has no FDA-approved treatment. Our cohort study aims to examine the clinical and pathological features of calciphylaxis and investigates the correlation between cutaneous clinical manifestations and histopathological findings. Data from 70 calciphylaxis patients who were evaluated at the Massachusetts General Hospital between January 2014 and April 2018 were collected from the institutional electronic database. The median age was 58 years (interquartile range [IQR]: 49-69 years), 60% were women, and 73% were of white race. Most (74%) patients reported severe pain at the time of calciphylaxis diagnosis with a median pain intensity score of 8/10 (IQR: 6-10) on a 0-10 pain scale. The median time from symptom onset to clinical diagnosis was 9 weeks (IQR: 6-16 weeks). The majority (87%) of patients presented with open necrotic wounds (advanced stage lesion) at the time of diagnosis. Common cutaneous clinical features included ulceration (79%), induration (57%), and erythema (41%), while common pathological features included cutaneous microvascular calcification (86%) and necrosis (73%). The presence of fibrin thrombi in skin biopsies was associated with pain severity (p = 0.04). The stage of a skin lesion positively correlated with the presence of necrosis on histological analyses (p = 0.02). These findings have implications for improving understanding of calciphylaxis origins and for developing novel treatments.

We describe a case of mpox characterized by a circularly distributed facial rash but no identified risk factors. Fomite transmission of monkeypox virus from contaminated linen at a massage spa was suspected. Clinicians should consider mpox in patients with consistent clinical syndromes, even in the absence of epidemiologic risk factors.

Within the Department of Dermatology at the Brigham and Women’s Hospital and Dana-Farber and Brigham and Women’s Cancer Center, where he remained the Director of the Mihm Cutaneous Pathology Consultative Service, a second opinion dermatopathology service and the Director of the Melanoma Program, Dr. Mihm consulted on more than 350,000 cases, nationally and worldwide, and offered accurate diagnosis, prognosis and treatment recommendations for routine and challenging cases in dermatology and dermatopathology. Since the beginning, he was interested in melanoma pathology, and with his mentor and friend, Wallace Clark, he published a paper in 1969 entitled “The histogenesis and biological behavior of primary human malignant melanoma of the skin” [1]. Among the very long list of his substantial contributions to dermatopathology, we can cite the following: the recognition of melanoma tumor progression: radial and vertical growth; the identification of different clinicopathological types of melanoma; the recognition of immunologic variants of cutaneous necrotizing vasculitis; the identification of syngeneic human graft-versus-host disease; the definition of the specificity and clinical utility of the lupus band test; the discovery of CD1a expression on human Langerhans cells and the demonstration of the utility of CD1a immunophenotyping for Langerhans cell histiocytosis; the recognition of borderline/minimal deviation melanoma variants; the introduction of classification schema for conjunctival melanocytic lesions; the development of a grading system for cytologic atypia in dysplastic nevi; the characterization of nevoid melanoma; the significance of tumor infiltrating lymphocytes in melanoma nodal metastases; the description of osteogenic melanoma; the establishment of guidelines for histological reporting of melanoma excisions; the clinicopathological insights into animal-type melanoma; the description of pigmented epithelioid melanocytoma; the description of use of MITF in melanoma diagnosis; the key contributions to AJCC melanoma staging; the discovery of a new marker (GLUT1) for juvenile hemangiomas; the contribution to the development of engineered melanoma vaccines based on dendritic cells; the setting of an EORTC melanoma policy; the identification of tumor lymphangiogenesis as a novel indicator of melanoma prognosis; the identification of the biology of desmoplastic melanoma; the elucidation of lentiginous subtype melanoma; the discovery that Prox-1 promotes the invasion of kaposiform hemangioendotheliomas [3].

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis that most commonly affects the postcapillary venules in the skin. It classically presents with purpuric macules that progress to palpable purpura on the bilateral shins 7–10 days after an inciting medication or infection, or in the setting of connective tissue disease, malignancy, or inflammatory bowel disease. Up to 50% of cases have no identifiable cause. Lesions on the buttocks, abdomen, upper extremities, and face are uncommon, as are bullae and ulcers. We present a rare case of bullous LCV manifesting as grouped vesicles on the face and body mimicking varicella-zoster infection.

Background: Lues maligna (also known as malignant syphilis or ulceronodular syphilis) is a rare dermatologic manifestation of syphilis more commonly seen in patients with HIV infection. The classic lesion of lues maligna is an oval, papulopustular skin lesion with well demarcated borders sometimes covered with a lamellar crust, but myriad clinical presentations of this disease also exist. Goals: To report a presentation of lues maligna in a patient with probable early HIV infection, emphasizing the diagnostic criteria and clinical manifestations of lues maligna. Study Design: Case report of lues maligna in a patient with probable early HIV infection. Conclusions: As syphilis becomes more common in many developed regions, it is important to recognize even atypical presentations of this clinical entity, especially among individuals who have unrecognized or early HIV infection.

To the Editor: The case presented by Merola et al. (July 18 issue) 1 describes a woman with postpartum fever, endometritis, and ulcerative skin lesions who received a diagnosis of Sweet’s syndrome. However, this diagnosis does not fully explain several clinical features, such as the multiple intrahepatic and intraabdominal abscesses, one of which yielded sterile purulent fluid, and the arm lesion resembling pyoderma gangrenosum rather than cutaneous Sweet’s syndrome. Given the patient’s presentation, aseptic abscess syndrome should be considered the most likely diagnosis. Aseptic abscess syndrome is an autoinflammatory disease first identified in 1995; it is characterized by sterile neutrophilic abscesses . . .

Tumid lupus is a rare subtype of chronic cutaneous lupus that is characterized by urticaria-like photosensitive plaques. Unlike discoid lupus, it has minimal to no surface change and resolves without scarring. On pathological examination, it may be distinguished from other types of lupus by abundant interstitial mucin deposits. Herein, we describe a case of tumid lupus in a 23-year-old Kuwaiti male with hyper-IgM syndrome. To our best knowledge, this is the first report of tumid lupus in a patient with a primary immunodeficiency.

Key Clinical Message Diffuse cutaneous mastocytosis (DCM) is a rare but potentially fatal condition when diagnosis and targeted treatments are delayed. This case illustrates the life‐threatening complications in DCM and reviews the currently available treatments. To our knowledge, this is the first report of mastocytosis with somatic K509I mutation and concomitant tuberous sclerosis. Diffuse cutaneous mastocytosis (DCM) is a rare but potentially fatal condition when diagnosis and targeted treatments are delayed. This case illustrates the life‐threatening complications in DCM and reviews the currently available treatments. To our knowledge, this is the first report of mastocytosis with somatic K509I mutation and concomitant tuberous sclerosis.

Key PointsDrug Reaction with Eosinophilia and Systemic SymptomsDrug reaction with eosinophilia and systemic symptoms (DRESS) is a T-cell–mediated severe cutaneous adverse reaction characterized by rash, fever, internal organ involvement, and systemic manifestations after prolonged exposure to a medication.DRESS accounts for approximately one fifth of cutaneous reactions among hospitalized patients; antibiotics are one of the most commonly identified triggers.Approximately 5% of DRESS cases result in death; the patients who survive may have relapses in addition to medical and psychological sequelae.The differential diagnosis includes morbilliform drug eruption; other severe cutaneous adverse reactions, such as the Stevens–Johnson syndrome and acute generalized exanthematous pustulosis; and additional conditions, such as hemophagocytic lymphohistiocytosis, angioimmunoblastic T-cell lymphoma, and acute graft-versus-host disease.The validated European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system is a rubric used to diagnose DRESS; however, elements of the diagnostic criteria may manifest at different time points, some late in the course of the disease.After identification and removal of the causative drug, treatment of DRESS consists of supportive care and glucocorticoids; glucocorticoid-sparing drugs and targeted biologic treatments are emerging as alternatives and complements to such treatment.There is no validated test to establish the cause of DRESS; dermatologists or allergist–immunologists may help assess drug causality and determine alternative drug therapy.

A 69-year-old Vietnamese female presented with fever and new-onset tender subcutaneous nodules on her trunk and lower extremities initially thought to be clinically consistent with erythema nodosum. A biopsy showed an atypical, predominantly lobular lymphocytic panniculitis with admixed neutrophils, karyorrhectic debris, and histiocytes with subcutaneous fat necrosis. Immunohistochemistry was consistent with gamma-delta T-cell lymphoma. The patient was initiated on a chemotherapy regimen of cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone (CHOEP) with partial remission, and is currently undergoing evaluation for bone marrow transplant. This case highlights the ability of cutaneous gamma-delta T-cell lymphoma to mimic more common cutaneous conditions such as erythema nodosum, and stresses the importance of a broad differential for new presentation of tender subcutaneous nodules with concomitant systemic symptoms.