[Facial manifestations of granulomatous diseases]. / Faziale Manifestationen granulomatöser Erkrankungen.

Clinical manifestations of granulomatous diseases are a recurring challenge in everyday dermatological practice. Clinical presentation and histological patterns are variable, depending on the particular disease. Knowledge about the differential diagnosis of granulomatous changes in the face is of decisive importance for making a rational diagnosis and therapy considering the patient's well-being. Therefore, histological and clinical characteristics of important granulomatous diseases that typically manifest on the face are presented here.

[Granuloma annulare]. / Granuloma anulare.

Granuloma annulare is a benign, chronic inflammatory skin disease. Its pathogenesis is still unclear, but reports on infections as a trigger can be found. In addition, some authors reported an association with other systemic disease, e.g., cancer, trauma, and diabetes mellitus; however, these have not been verified. The clinical picture of granuloma annulare ranges from the localized form predominantly at the extremities to disseminated, subcutaneous, or perforating forms. Diagnosis is based on the typical clinical presentation which may be confirmed by a biopsy. Histologically, necrobiotic areas within granulomatous inflammation are typical. The prognosis of the disease is good with spontaneous resolution being frequently observed, especially in localized forms. Disseminated manifestations tend to persist longer, and recurrences are reported. When choosing between different therapeutic options, the benign disease character versus the individual degree of suffering and the potential therapy side effects must be considered. For local treatment, topical application of corticosteroids is most common. Disseminated forms can be treated systemically with corticosteroids for several weeks; alternatively, dapsone, hydroxychloroquine, retinoids, fumaric acid, cyclosporine, and anti-TNFα appear to be effective.

[Fingertip necroses of unknown origin]. / Unklare akrale Nekrosen an den Fingern.

A 63-year old woman presented with dry necrotic areas on the distal and medial phalanges of her right index and middle finger caused by burns while smoking. Differential diagnostic considerations included occlusive arterial disease, vasculitis, endangiitis obliterans, peripheral embolisms and exposure to chemicals. The patient had the habit of smoking her cigarettes until the last possible drag, putting her skin in direct contact with the burning tobacco.

[Vohwinkel syndrome. Hearing loss and keratoderma on the hands and feet]. / Vohwinkel-Syndrom. Schwerhörigkeit und Keratosen an Hand und Fuß

The combination of sensorineural hearing loss and keratoderma on the hands and feet is rare. We report the case of a child that failed newborn hearing screening and also showed keratoderma on both hands and feet. The child's father exhibited the same constellation of symptoms, which is typical for mutilating keratoderma with deafness (Vohwinkel syndrome). This hereditary autosomal dominant disease is caused by mutation of the GJB2 gene that encodes the protein connexin 26. In our case it was highly likely that the GJB2 gene in the father carried a spontaneous mutation that was inherited by the daughter.