Tumorlike eosinophilic granuloma of the skin.

In 1952, Kuske reported on a patient with a peculiar tumor on the dorsum of the right hand; histological analysis revealed a dense dermal infiltrate with numerous eosinophils. Not aware of any similar case report in the literature, he coined the descriptive term "tumor-like eosinophilic granuloma of the skin." In 1995, a 55-year-old white man with cancer of the prostate presented with a 4-month history of two reddish-brown, solid skin tumors on his left forearm and on the right side of his abdomen, respectively. Histologic examination revealed a dense, superficial and deep, tumorlike dermal inflammatory infiltrate consisting mainly of eosinophils as well as neutrophils and in part epithelioid, in part foamy histiocytes. Flame figures were absent. Immunohistochemical analysis was negative for S-100 protein, whereas sporadic cells in the infiltrate were CD1a positive and many mononuclear-histiocytic cells reacted with MAC 387. Stains as well as cultures for bacteria, mycobacteria, and fungi were negative. The descriptive diagnosis of tumorlike eosinophilic granuloma of the skin was made. Seven weeks after prostatectomy, both tumors resolved spontaneously and so far has not recurred. In our opinion, this is the second report of Kuske's tumorlike eosinophilic granuloma of the skin. Perhaps tumorlike eosinophilic granuloma of the skin, eosinophilic ulcer of the mucosa, and transient eosinophilic nodulomatosis should be considered a mucocutaneous reaction pattern as is seen in cats. In humans, hypersensitivity reactions or atopy could emerge as an etiological link.

Circadian elevation of IL-6 levels in Muckle-Wells syndrome: a disorder of the neuro-immune axis?

Muckle-Wells syndrome (MWS) is a rare autosomal dominant hereditary disorder characterized by chronic recurrent urticaria, arthralgia, sensorineural deafness, and in some cases nephropathy due to amyloidosis (AA type). We report a 21-year-old woman and her father, both suffering from this syndrome, in whom elevated serum levels of IL-6 could be documented during the flares of urticaria, and discuss the relevance of this finding for MWS.

[Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of associated illnesses]. / Erythema elevatum diutinum. Eine seltene Dermatose mit einem breiten Spektrum assoziierter Erkrankungen.

Erythema elevatum diutinum (EED) is a rare disease presenting with persistent red to yellow-brown papules or plaques which are mainly localized symmetrically on the extensor aspects of the hands and fingers, the elbows and the knees. The histology shows a leucocytoclastic vasculitis in early lesions and fibrosis of the dermis later on. Dapsone is the treatment of choice. Today, EED is usually assigned to the neutrophilic dermatoses in which an association with hematological disturbances is well documented. We report on a patient with EED and glioma WHO grade IV, a coincidence unreported hitherto. Additionally, we review the literature on diseases associated with EED.

Toxocariasis and Wells' syndrome: a causal relationship?

BACKGROUND: The etiology of Wells' syndrome or eosinophilic cellulitis is unknown. Various triggering factors, such as myeloproliferative disorders, lymphoma, infections/infestations, insect bites and drugs have been reported. In 1979, Wells was the first who pointed out some common features of eosinophilic cellulitis and skin lesions in toxocariasis. OBJECTIVE: We report 2 patients who exhibited the characteristic clinical and histological features of Wells's syndrome together with elevated antibody titers to the excretory-secretory antigen of Toxocara canis. RESULTS: In both patients, the skin lesions disappeared after oral albendazole treatment and no recurrences were observed. The clinical response was followed by a normalization of the Toxocara antibody titer. In contrast, a patient with eczematoid skin lesions, eosinophilia and an elevated Toxocara antibody titer did not benefit from albendazole treatment despite serological normalization. CONCLUSION: Taken together, these cases lend support to a causal relationship of Toxocara in selected patients with Wells' syndrome.

[Angioma serpiginosum, a skin change along Blaschko lines?]. / Angioma serpiginosum, eine Hautveränderung entlang den Blaschko-Linien?

We present a 34-year-old woman with angioma serpiginosum on the medial aspect of the left thigh, extending to the groin and the lower abdomen, and on the volar aspect of the left forearm. In addition to the late onset, the distribution along the lines of Blaschko was a remarkable feature of this case. We therefore reexamined our recent cases of angioma serpiginosum. In 6 of 7 cases, the distribution indeed corresponded to the lines of Blaschko. Thus, we suggest adding angioma serpiginosum to the list of skin lesions that may follow the lines of Blaschko.

[Angiokeratoma corporis diffusum without associated metabolic disorder]. / Angiokeratoma corporis diffusum ohne assoziierte Stoffwechselstörung.

In a 22-year-old woman with mental impairment and some signs of dysmorphism, numerous angiokeratomas developed, starting when she was 3 years old and resulting in the clinical picture of angiokeratoma corporis diffusum. Extensive laboratory analyses did not disclose any associated metabolic disorder,and electron microscopy failed to demonstrate lysosomal inclusions. Therefore, this patient can be classified as one of the rare cases of angiokeratoma corporis diffusum without associated metabolic disease (idiopathic angiokeratoma corporis diffusum).