Subcutaneous thrombotic vasculopathy syndrome: an ominous condition reminiscent of calciphylaxis: calciphylaxis sine calcifications?

Ischemic skin necrosis can be a cause of severe morbidity and mortality. It can be due to a number of systemic conditions such as (1) thrombotic vasculopathy syndromes, (2) calciphylaxis, (3) septic or cholesterol emboli, and (4) cutaneous vasculitis. We present 3 patients with a clinicopathological syndrome consisting of ischemic skin necrosis associated with histological pattern of subcutaneous thrombotic vasculopathy-extensive microvascular thrombosis confined to small vessels and capillaries of the subcutaneous tissue. All 3 patients were obese and had severe pre-existing medical conditions. Skin biopsies showed intravascular thrombosis involving small arterioles and capillaries of the subcutaneous tissue. Distribution of vascular involvement by thrombotic process was similar to that observed in calciphylaxis, but calcifications were not observed. Two patients died within 3 months of diagnosis. One patient died 2 years after the presentation. Review of 15 biopsies of calciphylaxis revealed areas of subcutaneous thrombotic vasculopathy in 11 cases (73%). Our study shows that subcutaneous thrombotic vasculopathy syndrome is a potentially lethal condition showing overlapping features between thrombotic vasculopathy syndromes and calciphylaxis. Clinicopathological analysis suggests that it may be a rare variant of calciphylaxis sine calcifications or an early prodromal stage of calciphylaxis. This conclusion is in keeping with increasing appreciation of importance of thrombosis and vascular injury in calciphylaxis.

Collagenous vasculopathy: a report of three cases.

Cutaneous collagenous vasculopathy (CCV) is an idiopathic microangiopathy involving the superficial blood vessels that was initially reported in a 54-year-old male. We recently have identified this rarely reported entity in three Caucasian males. The first patient was a 59-year-old male with diabetes, hypertension and hypercholesterolemia who presented with multiple, red, blanchable, asymptomatic telangiectasias covering the extensor surface of the forearms, the lower abdomen and parts of the chest. The second patient was a 62-year-old male with psoriasis and extensive arthritis who presented with prominent telangiectasias on the left lateral distal thigh with mild overlying epidermal atrophy. The third patient was an 80-year-old male with atrial fibrillation who presented with blanching, telangiectatic areas on the abdomen, thighs and back. Histologically, the skin lesions showed ectatic superficial small blood vessels with laminated, hyalinized concretions around vessels that were highlighted with periodic acid-Schiff staining following diastase digestion and reactive by immunohistochemical staining with an antibody to collagen type IV. CCV is a rare and poorly understood entity with distinct histopathological features that may clinically resemble generalized essential telangiectasia (GET), yet which may affect a different demographic population than GET. Awareness of this uncommon entity may further help to elucidate its etiology.

Clinical and pathologic findings of paraneoplastic dermatoses.

UNLABELLED: Paraneoplastic dermatoses comprise a heterogeneous group of noninherited skin conditions that manifest internal malignancy. Familiarity with paraneoplastic dermatoses is important to both clinician and pathologist alike, as recognition of such a condition offers opportunity for early diagnosis and treatment of internal malignancy; monitoring for tumor recurrence; and insight into pathophysiology which may yield possible clues to treatment. Herein are reviewed 16 of the best established paraneoplastic dermatoses that display distinctive clinical and pathologic findings. LEARNING OBJECTIVE: At the conclusion of this leaning activity, participants should be able to recognize, diagnose, and describe the clinical and pathologic findings of paraneoplastic dermatoses.

Neutrophilic dermatoses.

Neutrophils may infiltrate all layers of the skin and consequently may cause different disorders, each with its own characteristic clinical and laboratory findings. We discuss how these disorders present and how they are diagnosed and treated. In addition, important associations with internal diseases are discussed to assist clinicians in evaluating for a concurrent illness. Because treatment of these disorders may often require systemic therapy, the potential short-term and long-term effects of commonly used medications are discussed. Finally, treatment of recalcitrant diseases, mostly by use of therapies published in the form of small case series or reports, is also included to guide clinicians in dealing with the more challenging cases.

A Symmetric Eczematous Eruption Harboring Thousands of Melanocytic Lesions.

IMPORTANCE: The abrupt appearance of melanocytic lesions is a unique phenomenon that can occur in the setting of eruptive nevi or epidermotropic melanoma metastases. OBJECTIVE: To examine the immunohistochemical and genetic mutative features of a novel case of an eczematous reaction followed by the abrupt appearance of melanocytic lesions. DESIGN, SETTING, AND PARTICIPANT: Case report of a 48-year-old woman with no significant medical history who first presented with an eczematous dermatitis on her torso, extremities, and buttocks and who subsequently developed thousands of pinpoint, histologically atypical melanocytic tumors and invasive melanoma within the areas of inflammation. MAIN OUTCOMES AND MEASURES: Immunohistochemical and mutational analyses of the patient's melanocytic tumors were conducted. RESULTS: Mutational analysis of the pigmented lesions did not identify any activating mutations in BRAF, PTEN, NRAS, KRAS, and HRAS. Immunohistochemical analyses of 9 biopsied pigmented lesions all showed normal expression of the tumor suppressors p16 and PTEN and no expression of mutated BRAF V600E protein. CONCLUSIONS AND RELEVANCE: To our knowledge, this is a previously unreported eruption comprising 2 distinct components: an eczematous reaction and a wave of melanocytic proliferations within the inflammatory regions. Possible explanations for this patient's condition, include immune stimulation leading to nevogenesis, benign "nevic" metastases, eruptive nevi, and epidermotropic metastatic melanoma.

Is there a role for tumor necrosis factor-alpha inhibitors and especially thalidomide in dermatology?

Because of the relative shortcomings and their side effects of the available anti-inflammatory drugs such as systemic nonsteroids, corticosteroids, and immunosuppressive drugs, and since tumor necrosis factor-a plays a major role in noninfectious inflammatory and autoimmune disorders, tumor necrosis factor-a inhibitory drugs and the available tumor necrosis factor-a inhibitory biologic modifying reagents are described. Among the drugs reviewed are pentoxifylline, thalidomide, etanercept, infliximab, and adalimumab. Their relative effectiveness and side effects are reported and recommendations are made.

Cutaneous mucormycosis secondary to acquired reactive perforating collagenosis.

Acquired reactive perforating collagenosis (ARPC) is a rare perforating disease of the skin. It is characterized by hyperkeratotic papules with transepidermal elimination of degenerated material including collagen and elastic fibers. The disease presents clinically as umbilicated papules with a central adherent keratotic plug. Mucormycosis infection, caused by the molds of the class Zygomycetes and order Mucorales, generally occurs as an opportunistic infection. It presents most frequently in patients with diabetes mellitus, in patients with leukemia receiving chemotherapy, and in those on sustained immunosuppressive therapy. We describe a patient with type 2 diabetes mellitus and end-stage renal disease requiring hemodialysis in whom extensive cutaneous mucormycosis with secondary spread to the brain, lumbar spine, and breast developed in the setting of ARPC. To our knowledge, this is the first case report of a patient with ARPC who developed extensive cutaneous mucormycosis.

Is there a role for infliximab in the current therapy of hidradenitis suppurativa? A report of three treated cases.

BACKGROUND: Hidradenitis suppurativa is one of the follicular occlusion diseases favoring the flexural areas of the body. Because of the past failure of medical therapy, surgery is today the definitive therapeutic approach. There is a need for effective medical anti-inflammatory therapy to control the disease and minimize the pathologic and socioeconomic consequences of the disease process. Generally the patients fail to respond to topical or systemic antibiotics and retinoids. Temporary responses are achieved with intralesional steroids and cyclosporin. METHODS: The association of hidradenitis suppurativa with inflammatory bowel disease permitted the opportunity to utilize infliximab and to observe not only its impact on the bowel, but also skin. RESULTS: Infliximab dramatically and favorably impacted on three cases of hidradenitis, but also in one of the patients who also had pyoderma gangrenosum. CONCLUSIONS: Infliximab appears to be an effective medical approach to the management of hidradenitis suppurativa and also to prepare the patient for "curative" surgery. However, one must be cognizant of the potential of acquired drug resistance or adverse drug reactions, which can be minimized by "no vacations" from this systematic therapy and the use of methotrexate to minimize the above potential problems.

Sarcoidosis: un updated review

Sarcoidosis, a multisystem disease of unknown cause characterized by the formation of noncaseating granulomas, may involve any organ of the body, but the commonest sites of predilection are the lungs, lymph nodes, skin, and eyes. Sarcoidosis can be associated with polyclonal hyperglobulinemia, hypercalcemia, hypercalciuria, circulating immune complexes, cutaneous anergy, and in vitro depressed cell-mediated immunity. Recent studies of the cellular composition and cellular interactions of sarcoid-involved tissue have resulted in some insight into the pathophysiology of the disease. In early stages the predominance of activated T-helper cells in affected sites may account for many of the immunologic aberrations. The production of macrophage chemotactic substances by these cells may be the initial stimulus for the formation of the granuloma. The natural history of the disease is highly variable; the acute forms tend to resolve spontaneously, whereas the chronic forms rarely involute. Although multiple therapeutic modalities have been used and some have reportedly been effective, systemic corticosteroids remain the most effective available therapeutic agents.