Erythema nodosum-like eruption in sarcoidosis

Two cases of sarcoidosis with tender, erythematous nodules on the legs are reported. The cutaneous lesions were clinically similar to those of erythema nodosum, but histologically showed non-caseating epithelioid granulomas. A review of 14 cases of this particular sarcoid eruption reported in Japan showed that 13 had ocular involvement as in our cases. In the light of the high frequency of ocular involvement, a skin biopsy should be considered in patients presenting with erythema nodosum-like eruptions situated on the legs.     

Löfgren's syndrome presenting with erythema nodosum-like eruption

L?fgren's syndrome is an acute form of sarcoidosis that mostly presents with erythema nodosum, periarticular ankle inflammation, arthritis, conjunctivitis, and unilateral or bilateral hilar or right paratracheal lymphadenopathy. Erythema nodosum is the most common cutaneous lesion in acute sarcoidosis, but it is histologically nonspecific, as it does not reveal sarcoidal granulomas upon microscopic examination. We present a case of a 57-year-old woman with acute onset of tender erythematous nodules on the calves, which were clinically erythema nodosum-like lesions. Histologic analysis revealed sarcoidal granulomas located in the dermis and subcutaneous tissue. Upon further work-up, the patient was found to have both pulmonary and ocular sarcoidosis. Based on this case, we recommend that all patients with erythema nodosum-like lesions undergo biopsy and further work-up for sarcoidosis is recommended in case of biopsy proven sarcoidal granulomas.     

Clinical and histopathological spectrum of cutaneous vasculitis in rheumatoid arthritis

BACKGROUND: Cutaneous manifestations are the most frequent, and often the initial feature of extra-articular involvement in patients with rheumatoid vasculitis. OBJECTIVES: The purpose of the study was to evaluate the clinical and histological spectrum of cutaneous vasculitis and the associated systemic involvement in patients with rheumatoid vasculitis. METHODS: Among 525 patients with rheumatoid arthritis, 20 tissue specimens with histologically proven cutaneous necrotizing vasculitis from 11 patients were investigated by studying the types and levels of affected vessels and related clinical features. RESULTS: Small-vessel vasculitis identified as dermal necrotizing venulitis was found in 10 patients, clinically characterized by palpable purpura, maculopapular erythema, erythema elevatum diutinum and haemorrhagic blisters. Arteritis histologically resembling cutaneous polyarteritis nodosa, clinically characterized by subcutaneous nodules, livedo reticularis, atrophie blanche and deep ulcers was identified in four patients all with systemic complications. Coexistence of venulitis and arteritis was identified in three patients. Different cutaneous vasculitic manifestations often coexisted and recurred in the same patient. Three patients with systemic complications of mononeuritis multiplex (two of three), interstitial pulmonary fibrosis (two of three) and abdominal microaneurysms (one of three) died within 1 year of onset of the cutaneous vasculitis. Immunofluorescence demonstrated vessel wall deposition of IgM and/or complement in six of the seven patients examined. CONCLUSIONS: Features of cutaneous rheumatoid vasculitis overlapping both the characteristics of cutaneous necrotizing venulitis and cutaneous polyarteritis nodosa together with coexistence of these different type of vasculitis in the same or different lesional skin account for the associated diverse cutaneous vasculitic manifestations. Although dermal venulitis (leucocytoclastic vasculitis) was the most common presentation, the presence of leucocytoclastic vasculitis in rheumatoid patients did not necessarily indicate a favourable prognosis. Associations with mononeuritis multiplex and bowel involvement had a fatal prognosis, while patients with superficial dermal venulitis without other extra-articular involvement may follow a favourable prognosis.     

Acute Crohn’s colitis with lobular panniculitis – metastatic Crohn’s?

One of the common lesions in Crohn's colitis is erythema nodosum, a septal panniculitis that may appear before diagnosis or in conjunction with flare up. We report a case of Crohn's colitis where the presenting sign was neutrophilic lobular panniculitis with few granulomas. The possibility that this case represents a forme fruste of metastatic Crohn's is suggested. As skin biopsies are not usually performed by gastroenterologists in erythema nodosum-like skin lesions in Crohn's patients it may well be that the incidence of lobular panniculitis is higher than reported in the literature.     

Sweet's syndrome associated with cellulitis

A 38-year-old woman developed an acute, painful cutaneous eruption on her lower legs, neck, chest and fingers while receiving a number of oral and intravenous antibiotics for left leg cellulitis caused by trauma. Clinically and histopathologically, she was diagnosed with Sweet's syndrome with typical pseudovesicular skin changes in conjunction with erythema nodosum-like lesions on her lower legs. She responded quickly to oral prednisolone. We propose Staphylococcal cellulitis as a cause of Sweet's syndrome.     

Granulomatous vasculitides and the skin

Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss granulomatosis may all have cutaneous involvement. The morphology of cutaneous lesions in these disorders varies from macular erythema to frank gangrenous ulceration. Most often lesions are located on the extremities; however, truncal or facial involvement has been reported, the latter especially in Wegener's granulomatosis. A common histologic finding in these cutaneous lesions is necrotizing vasculitis. However, it is also possible to see Churg-Strauss extravascular granulomas and even periarteritis. Cutaneous involvement with these three forms of systemic granulomatosis generally parallels the systemic course. The treatment for the cutaneous lesions is dictated by the treatment for the systemic vasculitis. It is important to recognize that the cutaneous extravascular granuloma and cutaneous granulomatous vasculitis can be associated with other disorders in addition to systemic granulomatosis. These disorders include most importantly lymphoproliferative diseases, inflammatory disorders such as arthritis, autoimmune diseases, and other inflammatory disorders such as sarcoidosis. Cutaneous involvement with giant cell or temporal arteritis is not common, but ulcerative temporal-parietal scalp lesions are distinctive. Although not common in the United States, Takayasu's arteritis may have several cutaneous manifestations, including erythema nodosum-like lesions. Granulomatous vasculitides have a myriad of cutaneous manifestations. Knowledge of these manifestations may allow for prompt diagnosis in many cases and increased surveillance in other cases for associated systemic illnesses.     

An Unusual Case of Cutaneous Pancreatic Fat Necrosis

BACKGROUND: Cutaneous pancreatic fat necrosis is a pathognomonic sign for pancreatic disease and usually presents as subcutaneous nodules in the pretibial region. OBJECTIVE: A case of cutaneous pancreatic fat necrosis is presented in which the clinical presentation of diffuse erythema was unusual. This disease is discussed and its possible etiologies are reviewed. METHODS: A MEDLINE search for cases of cutaneous pancreatic fat necrosis presenting as diffuse erythema without nodules was conducted. RESULTS: Diffuse erythema is an unusual presentation of cutaneous pancreatic fat necrosis. CONCLUSION: This may be the first case of cutaneous pancreatic fat necrosis presenting as diffuse erythema.     

Disseminated Mycobacterium marinum infection with extensive cutaneous eruption and bacteremia in an immunocompromised patient

Mycobacterium marinum can cause fish tank granuloma (or swimming pool or aquarium granuloma) in immunocompetent patients. Dissemination of Mycobacterium marinum-infection is a rare condition which occurs mainly in immunocompromised patients and can be life-threatening. We report the case of an 87-year-old woman who was treated with oral corticosteroids for polymyalgia rheumatica for many years and developed erythema nodosum-like lesions on the right forearm and arthritis of the right wrist. By increasing the steroid dosage and adding methotrexate only short-term remission was achieved. Seven months later painful erythematous nodules occurred on all extremities which became necrotic, ulcerative and suppurative. Ziehl-Neelsen staining revealed acid-fast bacilli and Mycobacterium marinum was cultured from skin biopsies, blood, and urine. The critically ill patient was treated with clarithromycin and ethambutol resulting in a dramatic improvement of the general condition. After four months, doxycycline had to be added because of new skin lesions. This case illustrates the impact of Mycobacterium marinum infection in immunocompromised patients.     

Disseminated cutaneous nocardiosis mimicking cellulitis and erythema nodosum

Infection with Nocardia asteroides is a rare, life-threatening infection, which is most commonly encountered in immunocompromised patients. Cutaneous involvement is usually seen with disseminated infection but may also occur as primary cutaneous nocardiosis. We present a case of an immunocompromised patient who presented with cellulitis of the right hand and disseminated subcutaneous nodules of the lower extremities resembling erythema nodosum. Cultures from both a skin biopsy of a subcutaneous nodule on the leg as well as a surgical specimen from the debridement of her hand grew Nocardia asteroides. The patient was treated successfully with trimethoprim-sulfamethoxazole. This case likely represents primary cutaneous nocardiosis with secondary dissemination, which has been rarely reported. It also emphasizes that nocardial infection should be considered in the differential diagnosis of lesions suggestive of cellulitis or erythema nodosum in the severely immunocompromised patient.     

Sweet syndrome (acute febrile neutrophilic dermatosis) and erythema nodosum in Crohn disease

We report on 2 patients who developed an acute febrile neutrophilic dermatosis (Sweet's syndrome) and erythema nodosum in association with Crohn's disease. The first patient showed symmetrical painful erythemas on her cheeks after hemicolectomy. Additionally, red painful nodules appeared on her lower legs. The second patient disclosed typical Sweet's syndrome-like lesions with pustules and plaques on her face, scalp and extremities after activation of Crohn's disease. Simultaneously, erythema nodosum-like lesions appeared on her lower legs.     

Ultrastructure of vascular changes in cutaneous manifestations of Beh?et's disease

We observed small dermal blood vessels embolized by thrombus at the sites of needle-prick reaction as well as erythema nodosum-like lesions in 4 patients with Beh?et's disease, by means of electron microscopy. The ultrastructural finding associated with overproductive proliferation of the endothelial cells leading to obliteration of the vascular lumen was the sporadical appearance of degenerated cells among proliferating endothelial cells in both cutaneous manifestations. These degenerated cells showed contraction changes and may be involved in thrombogenesis.     

Crohn's disease--non-caseating granulomas on the legs.

A 27-year-old black man with Crohn's disease and nodules on the legs is described. Such nodules constitute one of three types of cutaneous manifestations which have a histological picture of a non-caseating granuloma. The two other varieties of skin lesions described are: that resulting from a direct spread from the gastrointestinal tract, and that resulting from spread over contiguous skin.     

Sarcoidosis presenting with erythema multiforme-like cutaneous lesions

Sarcoidosis is a systemic disease that involves the skin in approximately 35 percent of cases. Typical cutaneous lesions are violaceous or hyperpigmented papules, plaques, or nodules. Less commonly, cutaneous lesions of sarcoidosis simulate the lesions of other cutaneous diseases such as psoriasis, ichthyosis, and erythroderma. We report on a patient who presented with cutaneous sarcoidal lesions resembling the target lesions of erythema multiforme (EM).     

Colchicine in the treatment of the cutaneous manifestations of Behçet's disease

Five patients with Behçet's disease were satisfactorily treated with colchicine. Oral aphthosis, erythema nodosum-like lesions and genital erosions improved greatly within a month as did laboratory findings. We believe colchicine to be the first choice in the management of the cutaneous and ocular lesions of Behçet's disease.     

Nodular vasculitis in systemic lupus erythematosus

A 42-year-old man presented with fever, photosensitivity, headaches, myalgia, hyperhidrosis, muscle weakness, alopecia, nasal crustae, weight loss, painful nails, arthritis, oral ulcers, erythema, discoid cutaneous lesions, and painful subcutaneous nodes. We made a diagnosis of systemic lupus erythematosus (SLE), type II cryoglobulinemia, and nodular vasculitis. In the skin, different types of vasculitis may be observed. Typically, histology shows leukocytoclastic vasculitis of superficial vessels both in SLE and mixed cryoglobulinemia, which clinically results in palpable purpura. In our patient, however, histopathological examination of the subcutaneous nodes not only revealed leukocytoclastic vasculitis of the superficial vasculature but also showed even more extensive involvement of dermal and subdermal small and medium sized vessels, giving rise to a nodular vasculitis.     

Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever

Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. Although various skin lesions such as “erysipelas‐like erythema”, urticaria, nonspecific purpura, and subcutaneous nodules have been described, cutaneous vasculitis is rare. We report a Japanese case of sporadic FMF accompanied by cutaneous arteritis at the time of febrile attacks of FMF. Gene analysis revealed M694I mutation in a single allele of the MEFV gene, and oral colchicine successfully controlled both periodic fever and subcutaneous nodules of arteritis. Cutaneous necrotizing vasculitis repeatedly emerging with febrile attacks should be included among the skin manifestations of FMF.     

Cutaneous sarcoid with varied morphology associated with hypercalcaemia and renal impairment

Sarcoidosis is a multisystem disorder of unknown aetiology, which presents with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous involvement. Cutaneous lesions often present as erythema nodosum, maculopapular, plaque, scar, subcutaneous nodule or lupus pernio. Most patients with cutaneous involvement have a single type of skin lesion, but some cases may have ≥ 2 types. We report a case of sarcoidosis presenting with various types of skin lesions. The case was also complicated by hypercalcaemia and renal dysfunction, and was successfully treated with oral corticosteroids. Presentation of various skin lesions may indicate systemic organ involvement requiring treatment with systemic corticosteroid.     

Isomorphic phenomenon in erythema multiforme

The cutaneous lesions of erythema multiforme have a propensity to localize at previously inflamed or traumatized skin sites. Evidence of the isomorphic phenomenon may be demonstrated in most cases of erythema multiforme by a careful history and physical examination. We present three cases of erythema multiforme which illustrate localization of skin lesions around scratches, recent surgical scars, lacerations, traumatized nail folds and folliculitis. The isomorphic phenomenon may serve as an important clue in understanding the clinical features and possibly, the pathogenesis of erythema multiforme.     

Erythema nodosum induced by the synergism of acupuncture therapy and flu-like infection

A 32-year-old female patient developed erythema nodosum-like lesions at needle prick sites after acupuncture therapy. Over the next few days, she developed similar new lesions over the extremities, trunk and face along with flu-like symptoms. There were neither genital ulcerations nor eye lesions. A skin biopsy specimen from an extremity lesion showed the characteristic findings of erythema nodosum. Treatment with oral potassium iodide at a dose of 750 mg/day was effective, and there has not been any recurrence to date. We diagnosed this case as erythema nodosum induced by a synergism between acupuncture therapy and a flu-like infection.     

Bullous Mastocytosis: Diffuse Cutaneous Mastocytosis with Extensive Blisters Mimicking Scalded Skin Syndrome or Erythema Multiforme

Bullous mastocytosis (diffuse cutaneous mastocytosis) is a rare form of mast cell disease that begins during the first month of life and causes extensive blisters that mimic scalded skin syndrome or bullous erythema multiforme. Discrete pigmented macules, papules, and nodules are absent and the characteristic leathery induration of skin may not develop until 6 months of age. Skin biopsy shows a subepidermal blister with mast cells at the base. The most serious complications are gastrointestinal hemorrhage and shock. The symptoms of bullous mastocytosis may be modified by a number of new therapeutic agents.