Pigmented purpuric dermatosis (PPD) is a group of chronic disorders characterized by the extravasation of erythrocytes and marked hemosiderin deposits in the dermis. Schamberg disease is a subtype of pigmented purpuric dermatosis in which nonpalpable purpura is prominent on the lower extremities. No cases of primary antiphospholipid syndrome and pigmented purpuric dermatosis have so far been reported in the literature. We herein report a case of pigmented purpuric dermatosis associated with primary antiphospholipid syndrome.

BACKGROUND:

Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.

OBJECTIVE

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CONCLUSIONS:

In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality.

Spontaneous clostridial myonecrosis (gas gangrene) is an uncommonly encountered presentation of skin and soft-tissue infections with high morbidity and mortality. The diagnosis has been associated with colorectal carcinoma, hematologic malignancies, and diabetes. We report the case of an 81-year-old man who experienced a sudden onset of left ankle and foot pain in association with nonpalpable purpura and hemorrhagic bulla. The diagnosis was initially suspected on clinical presentation, gas in tissue on plain-film radiography, and Gram staining of aspirated bulla contents. Cultures grew Clostridium septicum, and a large cecum mass was found by computed tomography of the abdomen. A subsequent right hemicolectomy confirmed a well to moderately differentiated mucinous adenocarcinoma. We emphasized that the early recognition of necrotizing skin and soft-tissue infections warrants further evaluation and appropriate management.

A middle-aged woman presented from an outside hospital with a diagnosis of Neisseria meningitidis and meningococcemia. A nonpalpable purpuric skin rash evolved into multiple wounds, with gradual necrosis of bilateral lower and upper extremities. Throughout the course of hospitalization, the patient developed ventricular tachycardia, normocytic anemia, thrombocytosis, Clostridium difficile infection, depression, and transient right eye blindness. The finding of decreased CH50 in the complement cascade was considered as the potential cause of the meningococcemia. The subsequent ischemia and necrosis of extremities were attributed to the systemic effect and trauma ensuing from N. meningitidis.

Strongyloidiasis is a potentially lethal parasitic infection. Coinfection of a patient with human T-lymphotropic virus type I (HTLV-I) can lead to a more severe disease course and treatment-refractoriness. Here we report a patient coinfected with HTLV-I and Strongyloides stercoralis who developed disseminated, treatment-resistant disease. The patient presented with serpiginous, nonpalpable, purpuric streaks on the abdomen and proximal lower extremities. A biopsy of this eruption demonstrating filariform larvae in the dermis was consistent with disseminated strongyloidiasis. The patient's immune dysregulation due to HTLV-I positivity likely contributed to her development of disseminated disease. Awareness of the interaction between HTLV-I and strongyloidiasis has important implications in terms of prognosis and treatment. Recognition of the cutaneous manifestations of disseminated disease can facilitate diagnosis and implementation of appropriate therapy.

The anticoagulant warfarin can produce a skin necrosis that is clinically indistinguishable from the skin necrosis caused by purpura fulminans associated with disseminated intravascular coagulation (DIC) and heparin-induced thrombocytopenia (HIT). The similar clinical and histologic findings observed in each of these skin necroses create a challenge for diagnosis and eventual treatment. The authors report a patient with significant risk factors for warfarin-induced skin necrosis, DIC, and HIT presenting with painful, purpuric patches beginning on her feet and extending proximally before becoming hemorrhagic bullae on her lower extremities.

Splenectomy is increasingly being performed by various minimal access surgical modalities for select hematologic disorders.A retrospective analysis was performed on the first 50 patients on whom laparoscopic splenectomy (LS) was attempted. The data studied included indications for surgery, patient demographics, intraoperative parameters, and patient outcomes. A total laparoscopic approach (TLS) was employed in 38 patients and a hand-assisted technique (HALS) was used in 12 patients with massive splenomegaly. Eight patients had concomitant surgical procedures: 7 patients underwent laparoscopic cholecystectomy and 1 patient received a kidney transplant. The most common indications for LS were idiopathic thrombocytopenic purpura (ITP) (50%) and hereditary spherocytosis (24%).LS was successfully completed in 48 patients (96%). Thirty-four patients (68%) required perioperative blood or platelet transfusions. The mean spleen diameter was 17.1 cm (range, 11.2-28.4 cm) on imaging study and mean intact splenic weight was 1019 gm. The mean operative time was 188 minutes (range, 90-340 minutes) in the TLS group and 171 minutes (range, 120-240 minutes) in the HALS group. The mean intraoperative blood loss was 306 mL (range, 40-640 mL) in the TLS group and 163 mL (range, 100-300 mL) in the HALS group. The mean postoperative hospital stay was 3.2 days (range, 2-5 days).TLS is safe and feasible in patients with nonpalpable spleens. A concomitant laparoscopic procedure for treating coexisting abdominal pathology may be performed without additional morbidity. The HALS technique may be preferable in patients with splenomegaly (palpable spleens), as it appears to offer intraoperative advantages for retraction, dissection, hemostasis, and organ retrieval.

Minocycline hydrochloride, a synthetic tetracycline, is a systemic antibiotic that has received much attention over the past several years. Currently, minocycline is considered the most widely prescribed oral antibiotic in the management of acne. Minocycline has been associated with autoimmune events, hepatitis, lupus-like syndromes, serum sickness, vasculitis, Sweet's syndrome, and hyperpigmentation. We report a case of a patient who developed drug-induced immune thrombocytopenic purpura (DITP) after taking minocycline. The initial clinical presentation of nonpalpable, discrete nonblanching petechiae and cayenne pepper-like macules on his lower legs was diagnosed as pigmented purpuric dermatosis (Schamberg's disease). We report the first case of DITP with the clinical picture of Schamberg's disease associated with minocycline therapy.

Strongyloidiasis stercoralis hyperinfection presenting as vasculitic-like skin lesions is rare. An autoinfection cycle allows intestinal strongyloidiasis, usually a benign infection, to persist for many decades. We report a woman with disseminated S stercoralis infection presenting as nonpalpable purpuric skin rash and syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Upon admission, she was treated with corticosteroids for her vasculitic skin lesions, which then worsened her status. When the diagnosis was recognized, steroids were stopped, thiabendazole treatment was instituted, and she gradually recovered. Serious or fatal infection can occur in patients with strongyloidiasis who were treated with immunosuppressive drugs. Stool specimen screening and/or serological tests for S stercoralis infection in patients who require immunosuppressive therapy helps to prevent complications before embarking on such treatment. Unexplained hyponatremia, severe hypoalbuminemia without proteinuria, and unusual skin rashes, especially over the lower aspect of the abdomen and upper aspects of the thighs, in persons living in areas endemic to S stercoralis should raise suspicion of S stercoralis infection.

Erythematous macules, nonpalpable and palpable purpura, and flaccid pustules developed in a 59-year-old man with acute lymphocytic leukemia 8 days after reinduction chemotherapy with cytosine arabinoside and daunorubicin. Tissue and blood cultures grew Fusarium proliferatum, and a skin biopsy specimen revealed fungal vasculitis. Anemia and muscle weakness accompanied the disseminated infection, for which the patient received granulocyte transfusions and amphotericin B, ketoconazole, rifampin, and griseofulvin. Skin lesions and fungemia resolved with recovery of the bone marrow, and 51 days after the completion of his chemotherapy he returned home. If promptly recognized and aggressively treated, disseminated fusariosis is responsive to therapy. Infection with Fusarium species should be suspected in profoundly neutropenic patients in whom disseminated palpable purpura and myositis develop concomitantly.