Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome

BACKGROUND: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a marked peripheral blood eosinophilia, but the pathogenesis remains unknown. OBSERVATIONS: A retrospective review was performed in 9 cases of CSS in whom cutaneous findings were present as an initial manifestation. All 9 patients had purpura and petechiae as well as severe pain and paresthesias of the lower extremities. Four patients (44%) used leukotriene receptor antagonists to treat their asthma, and 3 (75%) of them developed CSS within 3 months. Five patients (56%) were positive for perinuclear antineutrophil cytoplasmic antibodies before therapy, but in all 5 the levels of perinuclear antineutrophil cytoplasmic antibody normalized. Serum IgE levels were elevated in all patients before treatment but decreased after treatment. Histologically, all patients demonstrated leukocytoclastic vasculitis and eosinophilic infiltration. Eight biopsy specimens (73%) revealed marked eosinophilia around the nerve fibers in the dermis. Palisading granulomas in association with vessel-based changes were present in 4 (36%) of 11 biopsy specimens. CONCLUSIONS: These characteristic cutaneous clinical patterns that are consistent with the presence of mononeuropathy multiplexes in the lower extremities may help physicians establish an earlier diagnosis. Both eosinophils and IgE, as well as perinuclear antineutrophil cytoplasmic antibodies to some degree, likely participate in skin lesion development in CSS. Furthermore, there appears to be a correlation between treatment with leukotriene receptor antagonists and the onset of CSS in some cases.     

Granulomatous arteritis in cutaneous lesions of Churg-Strauss syndrome

BACKGROUND: Although granulomatous arteritis is usually found in extracutaneous Churg-Strauss syndrome (CSS) lesions, the vasculitis in CSS cutaneous lesions typically shows small vessel vasculitis (leukocytoclastic vasculitis) without demonstrating the feature of granulomatous arteritis confirming the proper classification of CSS in the category of granulomatous vasculitis. METHODS: Four deep excisional biopsies were obtained from three untreated CSS patients who presented with livedo reticularis and subcutaneous nodules. Tissue blocks were recut and submitted for hematoxylin and eosin and elastic tissue staining to evaluate the histological features of the affected vessels. Immunostaining for histiocytes, lymphocytes, and neutrophils were performed on serial sections to confirm the cellular infiltration. RESULTS: In all specimens, subcutaneous granulomatous arteritis was observed. The unique histological feature distinct from other vasculitic disorders is characterized by marked infiltration of histiocytes and multinucleated giant cells in and around the disrupted subcutaneous arterial walls mixed with an eosinophilic infiltrate. In two specimens, granulomatous arteritis was found in the subsequent serial sections, not in the initial sections. The initial section may show extravascular granulomatous inflammation without evidence of vasculitis. CONCLUSIONS: Granulomatous arteritis as identified in the extracutaneous lesions can also be found in subcutaneous CSS lesions presenting with livedo reticularis and/or subcutaneous nodules.     

Myelodysplastic syndrome presenting as cutaneous vasculitis

A patient presenting with cutaneous vasculitis was noted to be pancytopenic. Subsequent investigation showed a myelodysplastic syndrome (MDS)–refractory anaemia with excess of blasts. Within 3 months her MDS progressed to acute myeloid leukaemia.     

Myelodysplastic syndrome presenting as cutaneous purpura

Myelodysplastic syndrome is a preleukemic, clonal disorder of the hematopoetic stem cell. Cutaneous manifestations include infections, vasculitis, neutrophilic dermatoses, and leukemia cutis. Senile purpura is a common condition occurring in elderly individuals who lack other systemic or hematologic diseases. We present a case of myelodysplastic syndrome in which cutaneous lesions resembling senile purpura were the initial clinical manifestation.     

The T-lymphocyte and cutaneous Churg-Strauss granuloma

Studies of biopsy specimens from 4 patients with Churg-Strauss granulomas of the skin were performed with monoclonal antibodies to T-lymphocyte subset antigens. The finding of a predominance of a LEU3 antibody-reactive helper T-cell population in the perivascular areas and in the palisading and central granuloma areas was noteworthy. Pronounced reduction of suppressor T-cells in the lesions was observed in 2 patients; the lesions were associated with vasculitis and rheumatoid arthritis in one patient and with vasculitis and lupus erythematosus in the other. The Churg-Strauss granuloma demonstrated features of a cutaneous helper T-cell granuloma.     

Churg-Strauss syndrome

Churg-Strauss syndrome is a systemic, granulomatous, necrotizing, and eosinophilic angiitis, associated with febrile bronchitis. The full-blown picture of the disease does not develop, however, but in the course of several years. We discuss the problems of diagnosis and treatment of this syndrome in the light of a case report concerning a 43-year-old woman suffering from Churg-Strauss syndrome.     

Childhood sarcoidosis presenting with extensive cutaneous lesions, bilateral hilar lymphadenopathy and severe hypercalcaemia

A 9-year-old boy with a history of atopic dermatitis presented with a 4-month history of an asymptomatic papular eruption. This was predominantly perioral in distribution with lesser involvement of the neck, arms and trunk. Investigations revealed severe hypercalcaemia of 3.77 mmol/L (normal range 2.10-2.60) and bilateral hilar lymphadenopathy. The diagnosis of sarcoidosis was supported by granulomata in skin and lymph node biopsies on histopathological examination. Prednisolone (2 mg/kg/day) rapidly normalized serum calcium.     

Churg-Strauss syndrome involving medium-sized arteries

A 69-year-old Japanese man suffered from bronchial asthma, atrial fibrillation, general fatigue, high fever, and weight loss of about 5 kg within a month. He also had intermittent claudication, a tingling feeling in his fingers and toes, and an ulcer on his toe. His laboratory data revealed leukocytosis with absolute eosinophilia. The patient was treated with predonisolone 30 mg daily. Although the ulcers healed once, the lesions recurred with tapering predonisolone. The patient visited us because of the ulcer on his toe. Physical examination showed a 2 cm ulcer surrounded by slight erythema on his right fourth toe. Magnetic resonance angiography detected tapering stenosis of the medium-sized arteries in both legs. A biopsy from his myocardium showed the infiltration of eosinophils into the myocardium. The neuron conduction rate of his lower leg was slower than that of the normal control, demonstrating mononeuritis. From these findings, we diagnosed this patient as Churg-Strauss syndrome.     

Malignant histiocytosis presenting with vasculitis and cutaneous erythrophagocytosis

We report a case of malignant histiocytosis that presented initially as a cutaneous vasculitis without malignant features. A subsequent biopsy specimen of an erythematous skin lesion showed erythrophagocytosis by normal-appearing histiocytes within the subcutis. The recognition of erythrophagocytosis was followed by systemic symptoms and signs of malignant histiocytosis and a rapidly deteriorating clinical course. Possible mechanisms for erythrophagocytosis are discussed, and other diseases associated with this histologic finding are reviewed briefly.