Trigeminal trophic syndrome: a rare entity

Trigeminal trophic syndrome is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a classic triad of anaesthesia, paraesthesia, and a secondary persistent or recurrent facial ulceration. We describe a 60 year-old woman who developed this syndrome as a sequel to the gasserian ganglion block for trigeminal neuralgia. She had also developed melasma within 1 year. A remarkable benefit was achieved by proper patient education and topical antibiotics which led to the healing of all ulcerations within 4 weeks. In the case reported here, the diagnosis of the trigeminal trophic syndrome was made primarily as a result of the physician's experience with the syndrome previously.     

Case of livedoid vasculopathy with peripheral neuropathy successfully treated with low-dose warfarin

We report herein a case of a 28-year-old woman with persistent livedo racemosa and recurrent ulcerations on the lower extremities. The clinical presentation, together with histopathological findings of vascular occlusion without overt vasculitis in the dermis, led to the diagnosis of livedoid vasculopathy. The patient experienced recurrence of ulcerations and developed peripheral neuropathy affecting the distal extremities during the course of treatment with sarpogrelate hydrochloride. The skin lesions and neurological symptoms improved dramatically after adding low-dose warfarin potassium to the treatment regimen. This case suggests that administration of low-dose warfarin is an effective therapy of choice for patients with livedoid vasculopathy.     

Double skin tumors with an atypical clinical picture

The authors present a rare case of double skin tumors: acral lentiginous melanoma and metatypical carcinoma. The tumors were manifested as gradually enlarging ulcerations on the leg and sole of the foot that did not respond to standard treatment. The skin biopsies showed advanced acral lentiginous melanoma on the sole and metatypical carcinoma of the lower leg. Soon after the diagnosis was made, the melanoma generalized. The article discusses the differential diagnosis of both leg ulcerations, correct diagnostic procedures, and characteristic features of both tumors that are important questions for general practitioners, dermatologists, and surgeons.     

Healing of Ulcers due to Cryofibrinogenemia with Colchicine and High-Dose Pentoxifylline

Cryofibrinogenemia is due to the presence of reversibly cold-precipitating plasma proteins and material, consisting mostly of fibrinogen, fibronectin, and fibrin. This condition can be idiopathic or secondary to infection, thromboembolic states, neoplasm, or connective tissue disease. The characteristic lesions of cryofibrinogenemia include purpura and ulcerations. Histologically, the lesions of cryofibrinogenemia demonstrate fibrin thrombi within vessels, with no evidence of vasculitis. Treatment of cryofibrinogenemia should be directed at the underlying disease process, if one can be found. Other treatments have included the anabolic steroid stanozolol, which is presently unavailable, anticoagulants, immunosuppressive agents, plasmapheresis, and the combination of streptokinase and streptodornase. We report a case of a 61-year-old male smoker with a 10-year history of intermittent ulcerations of both legs and feet. Two separate biopsies showed epidermal ulceration and thrombi within superficial dermal vessels without evidence of vasculitis. These findings, together with the presence of elevated plasma cryofibrinogen, led to the diagnosis of cryofibrinogenemia. The patient continued to have ulcerations despite efforts to control his high blood pressure, cold avoidance, local wound care, and treatment with pentoxifylline 800 mg three times daily. However, when colchicine 0.6 mg twice daily was added to the patient’s care, this led to rapid healing of his ulcerations. He has remained ulcer free for 2 years taking the combination of colchicine and high-dose pentoxifylline. Efforts to reduce the dose of these agents have repeatedly led to recurrences, and remission has promptly followed re-establishment of the combination. To our knowledge, this is the first report documenting use of the combination of colchicine and high-dose pentoxifylline to successfully treat ulcers due to cryofibrinogenemia.     

Ulcus vulvae acutum

A 29-year-old female presented with a history of sudden onset of genital ulcers with fever and malaise. The clinical picture and course of the disease corresponded to the diagnosis of acute vulvar ulcer. No relevant etiologic factors could be ascertained. Treatment with doxycycline and systemic corticosteroids led to the resolution of the ulcerations after three weeks. Although the disease is rare, it is important to include it in the differential diagnosis of genital ulcers.     

Scurvy presenting with ecchymotic purpura and hemorrhagic ulcers of the lower limbs

INTRODUCTION: The risk of vitamin C deficiency is underestimated in industrialized countries and is only disclosed in rare cases of severe scurvy. CASE REPORT: We report three cases of scurvy presenting with ecchymotic purpura and hemorrhagic ulcerations of the lower limbs. Vitamin C supplementation led to rapid improvement of the skin lesions. DISCUSSION: Clinical diagnosis of low-grade deficiency can be difficult. Biological diagnosis requires special care in sample taking and transport.     

Peristomal pyoderma gangrenosum in a patient with ulcero-hemorrhagic rectocolitis

Cutaneous manifestations of chronic ulcerative colitis are numerous and present in 20 p. 100 of the patients. The case of a 49-year old man with peristomal pyoderma gangrenosum (PG) is presented. In November, 1983, one year after the onset of chronic ulcerative colitis, the patient underwent total colectomy. The formation of an ileal reservoir resulted in a pelvic abscess which necessitated right lower ileostomy. Because of severe ulcerations around the stoma, the ileostomy was transferred to the left lower abdomen. Similar inflammatory ulcerations developed, and the diagnosis of pyoderma gangrenosum was made. The patient responded very well to dapsone combined with prednisone for only 6 months. The possibility of PG being an autoimmune condition is discussed, as well as the differential diagnosis. The several therapeutic approaches are summarized. The peristomal localization of PG in this patient suffering from chronic ulcerative colitis seems to be rather unique. No other case has been reported so far, to our knowledge.     

Autoimmune ulceration of the leg

Autoimmune diseases are those disorders characterized by circulating autoantibodies or specific immune effector cells targeted towards a variety of self antigens. These disorders have traditionally included the so-called collagen vascular diseases, such as lupus erythematosus or scleroderma, certain vascultides, and a variety of immunologically mediated bullous diseases. The collagen vascular diseases and vasculitides, in particular, are occasionally associated with chronic, relapsing lower extremity ulcerations that may be refractory to traditional management schemes. Table 1 offers a classification of such disorders and provides a basis for the discussion which follows.

The frequency of these diseases among leg ulcer patients in general is difficult to gauge and depends in large part on the demographics of one's patient population as well as on efforts made to solicit such patients. For the last 18 months at the University of Pittsburgh, we have seen 303 new patients with leg ulcerations as part of a multidisciplinary wound healing and limb preservation clinic. Of this number, 20 patients (6.6%) have had leg ulcerations that were felt to be directly attributable to a variety of collagen vascular diseases or vasculitides. An understanding of these disorders and their potential to form ulcerations of different types is important in the differential diagnosis of patients with leg ulcerations in general, and management of these patients in particular.

The following is a discussion of the most important autoimmune disorders associated with leg ulcerations, with emphasis on practical aspects of diagnosis and management.     

Kutane Kalziphylaxie

Calciphylaxis represents a dermatological emergency with a mortality of up to 80%. The disease is characterized by a triad of arteriolar medial calcification, thrombotic cutaneous ischemia and necrotic ulcerations. Recently several mechanisms of vascular calcification have been identified. This may led to preventive measures in the future. Early diagnosis is important to avoid complications such as sepsis. The dermatologist plays an important role in early diagnosis based on the recognition of clinical presentation and histopathology. Patients with end-stage renal disease are most commonly affected by calciphylaxis. The most frequent non-uremic predisposing conditions are primary hyperparathyroidism, malignancies, alcohol-induced liver disease, and autoimmune connective tissue diseases. Medical treatment aims to normalize mineral metabolism to reduce the serum concentration of sodium phosphate and thus to prevent precipitation and calcification. Newer compounds are bisphosphonates, non-sodium/non-aluminium phosphate binders, cinacalcet, paricalcitrol, and sodium thiosulfate. Among the surgical procedures parathyroidectomy did not result in a significant survival benefit. An aggressive surgical debridement of necrotic ulcerations, on the other hand, improved survival. Early diagnosis and a multidisciplinary treatment approach including re-vascularization by the vascular surgeon, repeated surgical debridement and split skin transplantation support wound healing and insure limb conservation.     

Cutaneous calciphylaxis

Calciphylaxis represents a dermatological emergency with a mortality of up to 80%. The disease is characterized by a triad of arteriolar medial calcification, thrombotic cutaneous ischemia and necrotic ulcerations. Recently several mechanisms of vascular calcification have been identified. This may led to preventive measures in the future. Early diagnosis is important to avoid complications such as sepsis. The dermatologist plays an important role in early diagnosis based on the recognition of clinical presentation and histopathology. Patients with end-stage renal disease are most commonly affected by calciphylaxis. The most frequent non-uremic predisposing conditions are primary hyperparathyroidism, malignancies, alcohol-induced liver disease, and autoimmune connective tissue diseases. Medical treatment aims to normalize mineral metabolism to reduce the serum concentration of sodium phosphate and thus to prevent precipitation and calcification. Newer compounds are bisphosphonates, non-sodium/non-aluminium phosphate binders, cinacalcet, paricalcitrol, and sodium thiosulfate. Among the surgical procedures parathyroidectomy did not result in a significant survival benefit. An aggressive surgical debridement of necrotic ulcerations, on the other hand, improved survival. Early diagnosis and a multidisciplinary treatment approach including re-vascularization by the vascular surgeon, repeated surgical debridement and split skin transplantation support wound healing and insure limb conservation.     

Subtotal C4 deficiency and SLE-like disease

Extremely low C4 values were found in a 65-year-old man with relapsing arthritis and skin lesions of many years duration of the scalp, face, hands and feet together with painful ulcerations of the toes and fingers. The discovery was made during an exacerbation, but the deficiency of C4 persisted in repeated controls after remission. The clinical findings in connection with these low C4 values are in congruence with the diagnosis of inherited deficiency of C4.     

Temporal arteritis presenting with scalp ulceration

We report the case of a 75-year-old-woman who presented with bilateral scalp ulcerations and blindness, accompanied by severe headache and scalp tenderness, due to bilateral temporal arteritis without systemic involvement. A biopsy taken from the border of an ulceration showed evidence of giant cell arteritis. She was treated with oral prednisone, 60 mg per day. The ulcerations healed in a few weeks but the vision loss was irreversible. This case highlights for temporal arteritis the importance of accurate and timely diagnosis as well as the need for prompt therapy with systemic steroids in order to avoid major complications, namely loss of vision. It also demonstrates that scalp necrosis and ulcerations are skin signs associated with a poor prognosis.     

Pyoderma gangrenosum of the breasts precipitated by breast surgery

Pyoderma gangrenosum may occur in unusual sites and not be readily recognized. Delays in diagnosis and appropriate treatment may result in extensive ulcerations and scarring. We present two patients with pyoderma gangrenosum involving the breasts after breast operation. Delays in diagnosis resulted in extensive ulcerations and scarring of the breasts.     

Protracted calciphylaxis,Part II

An unusual protracted course of calciphylaxis without cutaneous ulcerations has been encountered in 14 cases. Calciphylaxis may include several clinical presentations ranging from an acute, rapidly fatal course to an indolent, more benign variant. Calciphylaxis should be included in the differential diagnosis for patients with kidney disease presenting with a clinical picture of panniculitis. Patients with renal disease with a characteristic clinical presentation with or without cutaneous ulcerations should undergo an urgent biopsy to confirm the diagnosis.     

Fatal cytomegalic inclusion disease. Associated skin manifestations in a renal transplant patient.

A case of cytomegalovirus-induced vasculitis led to skin ulcerations in a renal transplant patient. Light and electron microscopy revealed inclusion bodies and viral particles, respectively, characteristic of cytomegalovirus. To our knowledge, this is the first report of cytomegalovirus-induced vasculitis producing skin ulcerations, and it illustrates another of the protean manifestations of cytomegalovirus inclusion disease that may have important clinical implications.     

Fallbericht zur Therapie mit Granulozyten stimulierendem Faktor beim diabetischen Fuß

Several pathogenetic factors such as peripheral neuropathy, vasculopathy and infection are responsible for the development of diabetic foot ulcerations. An important factor contributing to the high infection risk in diabetic patients is a defect in neutrophil granulocytes. Deficiencies in neutrophil chemotaxis, phagocytosis and respiratory burst activity with the decrease of the super- and peroxids are known to be associated with diabetes. Granulocyte-colony stimulating factor (G-CSF) increases the release of neutrophils from the bone marrow and improves neutrophil function. A 78-year old patient with non-insulin-dependent diabetes presented with ulcerations of both big toes and a malum perforans on the right sole. He also had generalized arteriosclerosis as well as a polyneuropathy with a dry foot and typical foot deformation as well as decreased in sensitivity. Intensive local care for 35 days led to no improvement of the ulcerations. Then G-CSF (Neupogen) was administered in a total dose of 165 million IU over 11 days; the daily dose varied between 15-30 million IU depending on the absolute leucocyte count. In addition 500 mg of oral ciprofloxacin (Ciproxin) was given b.i.d. This treatment led to a significant improvement of the lesions. Within 11 days cost analysis suggests G-CSF may be a cost-effective addition to antimicrobial therapy in diabetic foot infection.     

Clinical trial of lysozyme treatment of crural ulcers in humans

Twenty patients with chronic crural ulcerations refractory to previous treatment were subjected to local treatment with a solution of ovalbumin lysozyme in normal saline (1 mg/ml). Before this treatment cultures were made from the bottom of the ulcerations determining the type of the infecting micro-organism and its sensitivity to various concentrations of lysozyme. Good (5/20) and very good (15/20) results were obtained. No local or systemic unfavourable effect of lysozyme was noted. The ulcerations were cleared quickly of pus, granulation tissue developed, the inflammatory reaction around the ulcers decreased and pains were no longer felt. The cultured bacteria were insensitive in vitro to lysozyme concentrations applied in vivo. During the treatment with lysozyme the bacterial flora remained unchanged. The beneficial effect of lysozyme on wound healing may be due to its cationic influence on the cell membranes in the epithelium and to pH change in the ulcerations.     

Localized mucosal involvement and severe pulmonary involvement in a young patient with paraneoplastic pemphigus associated with Castleman's tumour

We describe a 19-year-old female patient who developed recurrent ulcerations limited to the orogenital mucosa for the last 3 years. She also developed dyspnoea 5 months after the onset of the orogenital lesions. Castleman's tumour of the retroperitoneum was found incidentally during routine physical examination. The diagnosis of paraneoplastic pemphigus (PNP) was made by pathological and immunological studies. The orogenital ulceration responded well to corticosteroid therapy, but severe bronchiolitis obliterans progressed despite intensive care. The patient eventually died from respiratory failure. This case demonstrates the diversity of clinical features of paraneoplastic pemphigus.     

Atypical presentation of herpes simplex virus in a patient with chronic lymphocytic leukemia.

Perianal infections caused by herpes simplex virus are common in immunocompromised patients. The cutaneous presentation in these patients is often atypical, overlaps with the clinical features of other diseases, poses a difficulty in diagnosis, and responds poorly to treatment. An immunocompromised patient with chronic lymphocytic leukemia, treated with oral corticosteroids, presented with chronic perianal ulcerations. This patient was referred for evaluation and treatment of "recalcitrant" pyoderma gangrenosum. Prompt diagnosis was possible when the clinical features were recognized and appropriate biopsy and cultures were obtained. We describe an atypical presentation of herpes simplex virus associated with both an endogenous and exogenous induced immunodeficiency, and stress the importance of routinely performing cultures on all perianal ulcerations and anal fissures to avoid the misdiagnosis, inappropriate treatment, and prolonged discomfort of these afflicted patients.     

Chronic ulcerative stomatitis

Chronic ulcerative stomatitis (CUS) has recently been described as a new disease entity characterized by chronic ulceration of oral mucosa which responds to treatment with hydroxychloroquine. It has a particular type of stratified epithelium-specific, antinuclear autoantibody as an immunological marker. Twelve cases have been reported in the literature. We present a 40-year-old woman with an 11-year history of chronic oral ulcerations. Other dermatological diseases, including oral lichen planus, pemphigus vulgaris and cicatricial pemphigoid, as well as bullous lupus erythematosus, were excluded. The clinical diagnosis of CUS was confirmed on the ground of the immunological and ultrastructural findings. The lesions initially responded to high doses of systemic corticosteroids but relapsed promptly after dose reduction. Dapsone was ineffective. Hydroxychloroquine, given at a dosage of 200–400 mg/day, led to a complete and longlasting remission.