Skin nodules and macules resembling vasculitis in the antiphospholipid syndrome—a report of two cases

Two patients who demonstrated skin lesions in the presence of antiphospholipid antibodies are documented. The first had a long history of recurrent painful nodules. She had also suffered two deep vein thromboses. The second developed a rash on the lower limbs resembling vasculitis which did not respond to prednisolone, but to low dose salicylate therapy. Histology in both patients revealed microthrombosis of cutaneous vessels.     

Infiltrating basal cell carcinoma in the setting of a venous ulcer

A 77-year-old man was referred with a 5-year history of an intermittently painful, nonhealing right medial ankle ulcer. The ulcer had not responded to multiple treatment modalities, including Unna boots, compression therapy, sclerotherapy, and split-thickness skin grafting. The past medical history was significant for a deep venous thrombosis in the right leg 30 years earlier (treated with warfarin for 3 months) and a history of greater saphenous vein harvesting for coronary bypass grafting 28 years previously. After the vein stripping, the patient had suffered from increasing right leg edema and stasis changes in the right leg. His history was also remarkable for coronary artery disease, dyslipidemia, and lymphoma treated with chemotherapy 8 years before presentation, with no evidence of recurrence. He had stopped smoking approximately 20 years earlier. Medications included atenolol, simvastatin, nicardipine, nitroglycerin, and aspirin. Skin examination revealed a 3.0 x 3.5-cm ulcer adjacent to the medial malleolus. The edges of the ulcer appeared raised and rolled (Fig. 1). Centrally, there was granulation tissue, which appeared healthy. There were surrounding dermatitic changes. Dorsalis pedis and the posterior tibial pulses were normal. Noninvasive vascular studies revealed severe venous incompetence of the right popliteal and superficial veins. Arterial studies and transcutaneous oximetry were normal. Computed tomographic scan of the pelvis did not reveal any adenopathy, and radiographic imaging did not reveal any bony changes suggestive of osteomyelitis. Biopsy of the ulcer edge and base showed infiltrating basal cell carcinoma (Fig. 2). Mohs' micrographic surgery required three layers; the final extent of the ulcer was 7.8 x 6.9 cm. A split-thickness skin graft was placed.     

Behçet's disease with relapsing cutaneous polyarteritis-nodosa-like lesions, responsive to oral cyclosporine therapy

Cutaneous polyarteritis-nodosa-like lesions are rarely described in Behcet's disease. We report a case of recurrent cutaneous polyarteritis-nodosa-like (C-PAN-like) lesions in Behcet's disease with multiple deep vein thromboses as part of systemic vasculitis. The mucocutaneous manifestations responded to prednisolone; however, C-PAN-like lesions were refractory and responded to oral cyclosporine. We conclude that C-PAN-like lesions may be marker of severe disease and require intense immunosuppressive therapy.     

Fatal group A streptococcal necrotizing fasciitis and toxic shock syndrome in a patient with psoriasis and chronic renal impairment

A 78-year-old woman presented with rapid onset of skin pain which evolved into oedema, discoloration and infarction. She was diagnosed with group A beta-haemolytic streptococcus (Streptococcus pyogenes) necrotizing fasciitis and streptococcal toxic shock syndrome. The patient had a past history of psoriasis and end-stage renal impairment. Despite treatment with multiple antibiotics in an intensive care unit, the skin infarction involving the upper trunk continued to expand and the patient died within 24 hours of hospital admission. Group A streptococcus and Staphylococcus aureus were cultured from a tissue biopsy. Renal failure and compromised skin barrier function are known to predispose to invasive streptococcal infections, but necrotizing fasciitis has only rarely been reported in association with psoriasis. This case illustrates the fulminant nature of the infection.     

Reactions to Penicillamine: A Case of Cutis Laxa,Elastosis Perforans Serpiginosa and “Pseudo” Pseudoxanthoma

This patient was a 61‐year‐old white female who received several years of penicillamine therapy for the treatment of cystinuria. She subsequently developed penicillamine induced cutis laxa, elastosis perforans serpiginosa, and pseudoxanthoma elasticum like skin lesions. In addition, she suffered from numerous chronic bilateral lower extremity skin ulcerations. Her past medical history was also significant for end stage renal disease requiring hemodialysis and pulmonary fibrosis. She presented to the University of Miami Wound Care Center in 1/04 for treatment of her chronic ulcerations. On physical examination, the patient had multiple large hyperpigmented plaques with central ulcerations on her lower extremities. Some of the ulcers had overlying crust and others were covered with yellow fibrinous tissue. She also had generalized thickened, lax skin with multiple folds. On her neck, thighs, back and arms were violaceous, atrophic, serpiginous plaques with peripheral crusted erosions. A biopsy taken from the patients left thigh revealed dermal elastosis and the features of pseudo‐pseudoxanthoma. Two additional biopsies taken from the left thigh demonstrated elastosis perforans serpiginosa. This case highlights multiple skin manifestations of penicillamine therapy.     

Subklaviathrombose nach Portanlage bei metastasiertem Melanom

The use of implanted central venous catheters with ports has become almost irreplaceable in clinical treatment of cancer patients with advanced disease. It improves the patient's quality of life and simplifies medical treatment. For safe use during outpatient follow-up treatment, possible complications such as wound infections, thrombosis or even pulmonary embolism have to be considered. The incidence of catheter-associated thrombosis in cancer patients has been estimated as 25-66% in screening evaluations. Clinical symptoms were present in only in 6-28% of all patients with diagnosed thromboses. We report on a patient with metastatic malignant melanoma, who developed a deep arm vein thrombosis two weeks after port implantation. The prognosis of deep arm vein thrombosis is determined by the development of pulmonary embolism (8-36%). In such cases, early diagnosis and treatment are essential for the patient's survival.     

ACQUIRED PERFORATING DISEASE ASSOCIATED WITH CHRONIC RENAL FAILURE

A 48-year-old man, in a chronic hemodialysis program, had numerous skin lesions develop that consisted of follicular papules and small hyperkeratotic nodules, located on the trunk, hips, and upper and lower extremities (where the lesions were more numerous). The nodules measured 3 to 5 mm and had a dark discoloration (Figs 1 and 2). Four months previously, the patient noticed the appearance of disseminated papular and pruritic lesions. The lesions did not improve with the prescribed local nonspecific treatment. The patient had chronic renal failure during the previous 10 years, secondary to renal vein thrombosis. As a result, he has had periodic hemodialysis for 18 months. The clinical diagnosis of the skin lesion was Kyrle disease, and a skin biopsy was performed on a nodular lesion from the thigh. The histologic sections showed skin with hyperkeratosis, an invagination of the epithelium filled with keratin and amorphous material. In some sections, the invagination appeared to be a cyst, which in serial sections, showed partial rupture of the lateral wall. No evidence of hair was found inside the invagination.     

The impact of skin disease following renal transplantation on quality of life

BACKGROUND: The immunosuppressive therapy a patient requires to sustain a functioning renal allograft in the long term is associated with various skin complications. While quality of life (QoL) after renal transplantation has been studied, no publications document the effect of post-transplant dermatological complications on QoL. OBJECTIVES: The objective of the study was to document the prevalence of the skin diseases that commonly occur in association with post-transplant immunosuppression. A general dermatological quality of life questionnaire, the Dermatology Life Quality Index (DLQI), was used to assess the QoL effect of these cutaneous complications. The study was designed to examine further the impact of age, sex, duration since transplant and immunosuppressive regimen on the DLQI score of renal transplant recipients (RTR). METHODS: One hundred and seventy-three RTR completed the DLQI, were interviewed and examined for evidence of common post-transplant skin diseases. RESULTS: Sixteen per cent of RTR had DLQI scores >6, reflecting a significant impact on their QoL. Dry skin, itch, hypertrichosis, sebaceous gland hyperplasia, acne, genital warts and a history of >4 herpes simplex virus type 1 infections in the past year were all found to have a significant impact on the quality of life (P < 0.05). Multivariate analysis revealed that the greatest impact on QoL was in RTR who were younger, female and with multiple skin problems (P < 0.05). CONCLUSIONS: The dermatological complications of immunosuppressive therapy are common in RTR and can significantly impair QoL in certain individuals. Visible, infectious and cosmetic skin problems had most impact on QoL while a history of skin cancer had a lesser impact. Early dermatological referral and careful choice of immunosuppression may enhance the QoL, particularly in young and female RTR.     

Sclerotic bodies beyond nephrogenic systemic fibrosis

Sclerotic bodies are round to oval structures made up of collagen with entrapped elastic fibers, which may be sometimes ossified. These bodies may be found in skin biopsies from patients with nephrogenic systemic fibrosis (NSF), a disease linked to the use of gadolinium in radiologic procedures and chronic renal failure. Sclerotic bodies have not been reported in other diseases. Herein, we report sclerotic bodies as an incidental finding in a re‐excision specimen of a squamous cell carcinoma (SCC) from the forearm of an 85‐year‐old man with chronic renal failure. The patient had had multiple SCC removed over time. Additional clinical history revealed patient having received gadolinium in 2003 and 2004, preceding his dialysis that began in 2009. All of his excision specimens revealed sclerotic bodies in 20 of 30 specimens from 2008. None of the 26 re‐excision specimens prior to gadolinium exposure had these bodies. Our findings suggest that sclerotic bodies are the result of gadolinium exposure in patients with chronic renal insufficiency. Because the bodies were found near the re‐excision scar, it may be that gadolinium or its metabolites activate fibroblasts in the setting of wound healing. The reasons why this patient did not develop NSF are unclear.     

Keratotic vascular papules over the feet: a case of Waldenström’s macroglobulinaemia‐associated cutaneous macroglobulinosis

Waldenström’s macroglobulinaemia (WM) is a plasma‐cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48‐year‐old man presented with a 4‐year history of multiple painful, hyperkeratotic deep‐seated papules over the pressure areas of both soles. He had a 1‐year history of Raynaud’s phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid‐like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic‐acid–Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone‐marrow examination revealed a lymphoplasmacytoid malignancy. The patient’s systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.     

Coexistence of Basal Cell Carcinomas and Multiple Sebaceous Gland Hyperplasias in a Cyclosporine (Ciclosporin)-Treated Renal Transplant Recipient

A 55-year-old man presented with multiple, asymptomatic, yellowish papules on his face with a 4-year history, and two non-healing tumoral lesions on his nose with a 7-month history. He was a renal transplant recipient and had been treated with cyclosporine (ciclosporin) for 9 years. A biopsy from the asymptomatic, yellowish papule on the face showed sebaceous gland hyperplasia, and biopsies from the lesions on the nose revealed basal cell carcinomas. The lesions on the nose were excised. Sebaceous gland hyperplasia and skin cancers are among the cutaneous neoplasms observed in renal transplant recipients receiving cyclosporine. To our knowledge, this is the third reported case of the coexistence of basal cell carcinomas and multiple sebaceous gland hyperplasias in a cyclosporine-treated renal transplant recipient.     

Cutis verticis gyrata

An 82-year-old man had deep, linear skin folds on the scalp. The patient did not have a history of neuropsychiatric disorders and was generally in good health except for diabetes mellitus, hypertension, and benign positional vertigo. Cutis verticis gyrata is a term used to describe the appearance of deep, linear skin folds in the scalp. Primary and secondary types of this condition exist. No treatment is typically needed, although surgical modalities may be beneficial for severely disfiguring types.     

New aspects in the diagnosis of venous and lymphatic diseases of the legs

A survey on new methods for the detection of deep vein thromboses and for the investigation of lymphedemas of the lower extremities is given. Doppler-ultrasound, plethysmography and tele-thermography are valuable screening procedures for the detection of a deep vein thrombosis. Nuclear medical methods such as 131 J fibrinogen or 99-m-TC-plasmin-uptake test as well as isotopic phlebography are additional non-invasive procedures. For the routine diagnosis of lymphedema, isotopic lymphography can be recommended. Recently described methods such as quantitative isotopic lymphography and indirect lymphography with the new contrast medium Iotasul will probably enhance our present knowledge concerning pathophysiology and therapeutical effects in lymphedemas.     

A Case of Cutaneous Mycobacterium Chelonae Abscessus Infection in a Renal Transplant Patient

Abstract Background: Mycobacterium chelonae is an atypical "fast-growing Mycobacteria" that is a rare cause of human infection. There have been several reports of cutaneous infection among immunosuppressed patients, as well as in immunocompetent individuals following trauma. Most cases to date seem to have occurred among renal transplant recipients, raising the possibility that there is something inherent to the renal transplant patient that increases their susceptibility more than other immunocompromised patients. Objective: The differential diagnosis of subcutaneous nodules distributed in a sporotrichoid pattern is extensive, particularly in an immunocompromised host. Although several cases of cutaneous M. chelonae abscessus infection have been reported among both immunosuppressed and immunocompetent patients, the clinical presentation has varied, and few cases have reported the appearance of lesions in a sporotrichoid pattern. We present a case of a renal transplant patient with a reported history of trauma to the lower extremities, who presents with subcutaneous nodules distributed in a sporotrichoid pattern. The patient is found to have M. chelonae abscessus infection, fails several treatment regimens, and presents with a recurrence. The literature of M. chelonae infection is reviewed, and the various treatment options are discussed. Methods: An initial skin biopsy was stained with Hematoxylin and Eosin and revealed deep dermal abscesses with acid-fast bacilli in clusters. The culture became positive for Mycobacterium chelonae abscesses in four days and was found to be sensitive to multiple antibiotics. The patient underwent surgical excision of 14 nodules, which revealed findings consistent with the skin biopsy, and was subsequently treated with the appropriate antibiotics. Results: Despite treatment with a full course of an organism-sensitive antibiotic regimen, the patient returned with persistent and recurrent nodules six weeks later. The patient was then treated as an inpatient with a seven-week course of intravenous antibiotics and was discharged home on a combined intravenous and oral regimen. Conclusion: Although M. chelonae abscessus is an extremely rare cause of infection among humans, there seems to be a predominance of cases reported among renal transplant patients. The explanation for this is not entirely clear; however, the organism must be considered as a cause of infection in any renal transplant recipient who presents with subcutaneous nodules. Eradication of the organism presents a tremendous challenge to the clinician, and, as presented here, even with appropriate antibiotics, there is a high rate of recurrence.     

Hyperkeratotic eruption, hand-foot skin reaction, facial erythema, and stomatitis secondary to multi-targeted kinase inhibitor sorafenib

Background Sorafenib and sunitinib, novel anti‐tumor agents approved for the treatment of renal cell carcinoma, have been associated with multiple adverse cutaneous effects. Objective To further investigate the constellation of possible adverse effects of sorafenib and sunitinib on the skin. Subjects and Methods Case report of a 62 year old male with history of renal cell carcinoma treated with adjuvant sorafenib. Results Our patient experienced a diffuse hyperkeratotic rash, hand‐foot skin reaction, facial erythema, and stomatitis within three weeks of initiation of sorafenib. Conclusion Further investigation regarding the possible adverse cutaneous effects of sorafenib and sunitinib, pathogenesis and risk factors for development, associated time course, and preventative and therapeutic methods, will allow for better patient and physician education.     

Heparin-induced thrombocytopenia and warfarin-induced skin necrosis: case report

This paper describes a case of heparin-induced thrombocytopenia complicated by warfarin-induced skin necrosis in a 74-year old female patient hospitalized with diagnoses of a hip fracture, deep vein thrombosis and pulmonary thromboembolism. Warfarin-induced skin necrosis is a rare complication of anticoagulant therapy, with high morbidity and mortality that may be associated with heparin-induced thrombocytopenia.     

Cutaneous neonatal lupus erythematosus with unusual features

A three month-old boy was brought by his mother with complaints of multiple reddish lesions on his trunk and face since birth. The patient had erythematous annular plaques with scaling on his extremities, palms and soles with periorbital erythema and edema giving the characteristic "eye mask" or "owl's eye" appearance. His mother did not have history of any illness. Hemogram, liver and renal function tests were within normal limits. A skin biopsy was suggestive of subacute cutaneous lupus erythematosus. Immunological work-up was positive for antinuclear antibodies (ANA) (1:40) with anti-Ro titers of 3.4 and 3.47 (>1.1 = clinically significant titre) in the mother and child respectively, although negative for anti-La antibodies. The child's electrocardiogram and 2D echocardiography were normal. We are presenting a case of anti-Ro-positive cutaneous lupus erythematosus with an uncommon skin manifestation.     

Basal Cell Carcinoma Following Chronic Otitis Media

Abstract: A 36-year-old white woman presented with a gradually enlarging lesion in the right preauricular area of 15 years duration. The patient had a 25-year history of chronic otitis media of the right ear; in spite of multiple courses of systemic antibiotics, she frequently noted a slight discharge from the affected ear, draining across the right preauricular area, causing a mild pruritic eruption. She recalled having had approximately 30 roentgenographs of mastoids, but denied any history of fluoroscopic examination. The patient denied a history of excessive sun exposure or exposure to arsenic or other carcinogenic factors. There was no family history of skin cancer.     

Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis

We report a patient with nephrogenic fibrosing dermopathy. He had chronic renal failure with arthritis, uveitis and histologically severe tubulointerstitial nephritis for which he received a renal transplant from a family relative. After an episode of acute renal failure with the transplant he developed painful, erythematous, firm papules and plaques with geographic borders on the legs, anterior thorax and elbow. A skin biopsy revealed increased fibroblast and collagen fiber content of the dermis and subcutaneous septae. Mucin deposition, sparse smooth-muscle-actin-positive cells and an increased number of CD34-positive cells in the deep dermis were found. After several weeks of hemodialysis, the lesions changed from an inflammatory to a purely sclerotic phase. The fibrocyte, a recently described circulating cell type, that is deposited in scar tissue may be involved in the pathogenesis of this novel pseudosclerodermatous skin disorder.     

Cowden's disease

A 24-year old male patient developed multiple lesions of keratoacanthoma in the epidermal verrucous naevus. He also had multiple papillomatous lesions on the lips, buccal mucosa, gingiva and tongue, with positive family history of similar lesions. He also had multiple skin tags and patchy palmoplantar keratoderma and minimal kyphoscoliosis.