The 97th SGDV Congress, held this year at the University of Irchel, followed the motto “Without vessels no skin – without skin no vessels” and was dedicated to the multifaceted connection between dermatology and vascular medicine. We have picked out some exciting presentations for you.
PD Katrin Kerl, MD, Dermatology Clinic, UniversitySpital Zurich, presented the case of a 58-year-old man who presented with fever and an upper respiratory tract infection. In addition, he had angiomatous papules with pale halos on the extremities. Possible differential diagnoses included: vasculitis, reaction to a drug, infection (viral, syphilis, meningococcemia, rickettsiosis), vitamin D deficiency, mastocytosis (telangiectasia eruptiva macularis perstans). Histology showed enlarged dilated vessels in the upper dermis and thickened walls with plump endothelial cells.
Based on the findings, the diagnosis of “eruptive pseudoangiomatosis” was finally made. This condition has similarities to erythema punctatum Higuchi. It was first described in 1969 by Cherry et al. described. It is characterized by eruptive hemangioma-like lesions on the face and extremities and usually occurs in childhood. In adults, it is often associated with immunosuppression. Under vitropression, the lesions disappear.
The question of whether the disease has a viral component is debated, as it has an association with cytomegalovirus, Epstein-Barr virus, echovirus 32, and coxsackie virus B. In this case, the virus, which is transmitted by insects, would attack the pericytes of small vessels. Initial contact results in viremia and systemic symptoms (mainly in children). In reinfection in adulthood, the reaction is more localized, often without prodromes, and is clustered on exposed skin sites. There are reports of epidemic-like outbreaks within families and communities.
Red or white fingers – what’s behind them?
Prof. Beatrice Amann-Vesti, MD, Clinic for Angiology, University Hospital Zurich, first spoke about the clinical picture of white or reddish-purple fingers.
A very useful tool in the early diagnosis of Raynaud’s phenomenon is the so-called nail fold capillaroscopy. According to studies, the best predictor for a transition of Raynaud’s phenomenon into a secondary disease is a pathological finding in capillary microscopy (e.g. giant capillaries, irregular architecture, avascular fields): Positive predictive value is 47%. In contrast, the detection of antinuclear antibodies only reaches a value of 30%. On average, according to the meta-analysis, it took about ten years for the first signs of Raynaud’s phenomenon to develop into secondary disease. 12.6% of all patients were affected [1]. A follow-up over an average of 6.5 years of 133 patients with Raynaud’s phenomenon but negative serology also demonstrated the good predictive value of nailfold capillaroscopy: patients who developed connective tissue disease (e.g., systemic sclerosis, CREST syndrome, or mixed connective tissue disease) during this time had significantly more frequently been found to have giant capillaries, irregular architecture, or avascular fields [2].
According to Prof. Amann-Vesti, the sudden appearance of reddish-purple, swollen and painful fingers should be considered a paroxysmal finger hematoma, the so-called Achenbach syndrome. It is benign and self-limiting (regression within a few days).
Fluorescence microlymphography
“For various forms of edema, minimally invasive fluorescence microlymphography can give us valuable information,” Prof. Amann-Vesti said. This method takes advantage of the fact that certain large-molecule substances require lymphatic transport, i.e. molecules above a certain size can only be removed via the lymphatic system. The removal of the high molecular weight dextran (along with the attached dye: FITC) from the tissue depot via the lymphatic capillaries can be observed in the fluorescence reflected light microscope in vivo. The dye is normally promptly removed via deeper lymphatic collectors. Therefore, staining of only a few meshes of the superficial mesh occurs.
The larger the area of the superficial network shown, the poorer the drainage to depth and consequently the more pronounced the congestion. This picture is seen, for example, in lymphedema (in the case of hereditary lymphedema of the Nonne-Milroy type, however, no initial lymphatic vessels appear on the affected limb). In severe lymphatic microangiopathies in the course of chronic venous insufficiency, fluorescence microlymphography reveals, among other things, insufficiency of the collectors, a destroyed or interrupted network of initial lymphatic vessels, or increased permeability of the lymphatic vessels.
Lymphatic vessels in grafts and in psoriatic lesions.
It has been shown that split skin grafts, some of which are used for venous ulcers, are characterized by abnormal regeneration and function of lymphatic vessels even more than 70 months after transplantation [3], whereas lymphatic vessels in a graft used because of a severe burn (mesh graft) regenerate rapidly and form a normal network [4].
Recently, lymphatic microangiopathies in psoriatic skin lesions have also been reported in depth: In vivo, lymphatic permeability and blood flow were shown to be increased in such lesions, which may reflect the local inflammatory process [5].
Cat scratch disease
Dr. med. Lukas Flatz, Clinic for Dermatology/Allergology, Cantonal Hospital St. Gallen, went into more detail about the so-called cat scratch disease. The classic form is manifested primarily by regional lymphadenopathy (in the axillary or cervical region) and, as the name implies, by the presence of a cat scratch or bite wound with suspected inoculation. Diagnosis proceeds by histopathology, culture, serology and PCR. The most likely differential diagnoses are infection with atypical mycobacteria (specifically M. marinum) or sporotrichosis. Syphilis, tularemia, or primary tuberculosis inoculation should also be considered. Lymphoma must be ruled out in any case.
There are currently no clear guidelines for treatment. In the mild-to-moderate range, there is little data to suggest a clear benefit from antimicrobial therapy. Therefore, these patients should be treated conservatively with symptomatic therapy. The lymphadenopathy associated with the condition is self-limiting and resolves within two to four months, so most patients can only be observed.
In immunocompromised individuals, however, the infection causes a wide spectrum of clinical pictures, from the classic form to bacillary angiomatosis, to peliosis or septicemia.
The clinical picture of bacillary angiomatosis includes numerous, blood-red, superficial, smooth papules distributed over the entire body and skin-colored or dark subcutaneous nodules. Diagnosis is by histology, serology, and PCR, and treatment is with antibiotics against B. henselae. Kaposi’s sarcoma is an important differential diagnosis.
“So-called trench fever is another infectious disease caused by bacteria of the Bartonella genus, namely B. quintana,” the speaker said. “Transmission is via human body lice (which explains the name of the disease). Patients complain of severe headache, neck pain, back pain, and shin splints, and recurrent fevers appear every four to eight days. There are no specific cutaneous manifestations with this condition.”
Source: 97th Annual Meeting of the SGDV, August 26-28, 2015, Zurich.
Literature:
- Spencer-Green G: Outcomes in primary Raynaud phenomenon: a meta-analysis of the frequency, rates, and predictors of transition to secondary diseases. Arch Intern Med 1998 Mar 23; 158(6): 595-600.
- Meli M, et al: Predictive value of nailfold capillaroscopy in patients with Raynaud’s phenomenon. Clin Rheumatol 2006 Mar; 25(2): 153-158.
- Amann-Vesti BR, et al: Microangiopathy of split-skin grafts in venous ulcers. Dermatol Surg 2004 Mar; 30(3): 399-402.
- Meier TO, et al: Microvascular regeneration in meshed skin grafts after severe burns. Burns 2011 Sep; 37(6): 1010-1014.
- Meier TO, et al: Increased permeability of cutaneous lymphatic capillaries and enhanced blood flow in psoriatic plaques. Dermatology 2013; 227(2): 118-125.
DERMATOLOGY PRACTICE 2015; 25(5): 38-39
