J. H. Sillevis Smitt

The millennium criteria for the diagnosis of atopic dermatitis.

Abstract: Atopic dermatitis forms an active area of basic and clinical research, where important new knowledge about genetics and immunopathogenesis has surfaced over the past years, and where simultaneous development of new and innovative therapies is under way. However, the inclusion of any patient in an atopic dermatitis study, whether it is on its genetics, pathogenesis or therapy, requires a diagnosis which is irrefutable. Since there is no simple and also no complicated laboratory procedure to reach a diagnosis of atopic dermatitis, different sets of clinical criteria have been developed for the purpose of making the diagnosis uniformly in different studies as well as in different study centers. The most commonly used are Hanifin and Rajkas set of diagnostic features, which have major and minor clinical criteria to be fulfilled in order to establish a diagnosis of atopic dermatitis. Recent developments in the immunology of atopy have clearly established the major abnormality in this syndrome, the preferential production of allergen‐specific IgE. In this contribution, it is suggested that the presence of such antibodies in a given patient should be a mandatory criterium for the diagnosis of atopic dermatitis. Such a diagnostic test however establishes a diagnosis of atopic syndrome, not atopic dermatitis. Thus, for atopic dermatitis we have to rely, for the time being, on additional clinical criteria. The clinical features described in the literature are critically evaluated, and it is suggested that in addition to the mandatory presence of allergen‐specific IgE, 2 of 3 principal criteria (pruritus, typical morphology and distribution, chronic or chronically relapsing) should be present for such a diagnosis. Finally, the minor features originally described by Hanifin and Rajka and later evaluated by others are revised and divided over 4 subcategories; a) related to subclinical eczema; b) related to dry skin; c) extra skin folds; and d) ophthalmological pathology. They are suggested to be used as additional criteria only, needed when clinical suspicion is high but the new mandatory and principal diagnositic criteria described here are inconclusive. For study purposes, we suggest that the mandatory and principal criteria are sufficient. They are now evaluated and validated in ongoing atopic dermatitis treatment studies.

Pemphigus vegetans in a child

Summary Pemphigus vegetans was diagnosed in a 12‐year‐old boy based on clinical, histopathological and immunohistological findings. To our knowledge, this is the first case of juvenile pemphigus vegetans to be published in the literature. Suggested treatment with prednisone and azathioprine was refused, and the patient was treated with a decoction of herbs prescribed by a practitioner of traditional Chinese herbal medicine. This treatment gave excellent results. Possible active components of the treatment are discussed.

An Evaluation of PUVA-Therapy for Alopecia areata

30 patients with alopecia areata were treated with 8-methoxypsoralen orally combined with whole body UVA exposure. 9 patients showed more than 60% regrowth of hair, 8 of them had recurrences of hair l

Features of epidermolysis bullosa simplex due to mutations in the ectodomain of type XVII collagen

Background  Mutations in COL17A1, coding for type XVII collagen, cause junctional epidermolysis bullosa with an ultrastructural plane of cleavage through the lamina lucida of the epidermal basement membrane.

Sensory loss following cryosurgery of skin lesions

Sensory testing by means of a graded bristle technique was performed following cryosurgical treatment of 183 skin lesions in 169 patients.

The Poikiloderma of Rothmund-Thomson Syndrome: Changes in Langerhans Cell Morphology and Distribution

A black girl with the Rothmund-Thomson syndrome is presented. Immunophenotyping of subpopulations of immunocompetent cells in a biopsy of an atrophic hyperpigmented skin lesion revealed sparsity and unusual distribution of epidermal Langerhans cells. These cells were mainly located in the basal layer of the epidermis and did not show the usual dendritic pattern. Impressive immunoreactivity of the dermal infiltrate was observed by anti-HLA-DR staining. The changes in Langerhans cell morphology and distribution may indicate functional impairment of the up-regulating arm of skin immunity.

Natural killer cells involved in lymphomatoid granulomatosis

weeks was without effect. Over the next 2 months the rash slowly extended centrifugally becoming confluent with pigmentation centrally and blistering occurring along the advancing edge (Fig. i). After 3 months the rash was controlled and i year later prednisolone was reduced to 15 mg daily without relapse. Reports of this appearance in bullous pemphigoid are rare (Vakilzadeh & Suter, 1981) but there are some similarities with the bullous variant of erythema annulare centrifugum described by Kind (1975). Other patients with bullous pemphigoid have had transient erythema gyratum repens-like rashes (Breathnach, Wilkinson & Black, 1982; Saikia, Mackie & McQueen, 1973). IgG and C3 immunofluorescence have been seen along the basement membrane zone in erythema annulare centrifugum (Holt & Davies, 1976). Perhaps there is some similar mechanism involved in the pathogenesis of annular erythemas and these cases of bullous pemphigoid, particularly with regard to the spread of the rash from involved to uninvolved skin, which might depend on coupled immune interactions linked by diffusion or migratory immunoregulatory factors as postulated by Moore (1982).

Discrepancy between the clinical and histopathologic diagnosis of soft tissue vascular malformations

Background: Soft tissue vascular malformations are generally diagnosed clinically, according to the International Society for the Study of Vascular Anomalies (ISSVA) classification. Diagnostic histopathologic examination is rarely performed. Objective: We sought to evaluate the validity of the current diagnostic workup without routinely performed diagnostic histopathology. Methods: We retrospectively determined whether there were discrepancies between clinical and histopathologic diagnoses of patients with clinically diagnosed vascular malformations undergoing therapeutic surgical resections in our center (2000–2015). Beforehand, a pathologist revised the histopathologic diagnoses according to the ISSVA classification. Results: Clinical and histopathologic diagnoses were discrepant in 57% of 142 cases. In these cases, the pathologist indicated the diagnosis was not at all a vascular malformation (n = 24; 17%), a completely different type of vascular malformation (n = 26; 18%), or a partially different type with regard to the combination of vessel‐types involved (n = 31; 22%). Possible factors associated with the discrepancies were both clinician‐related (eg, diagnostic uncertainty) and pathology‐related (eg, lack of immunostaining). Limitations: Retrospective analysis of a subgroup of patients undergoing surgery. Conclusion: The large discrepancy between clinical and histopathologic diagnoses raises doubt about the validity of the current diagnostic workup for vascular malformations. Clear clinical and histopathologic diagnostic criteria might be essential for a uniform diagnosis.

Acquired arteriovenous malformation induced by pressure: a case report.

Arteriovenous malformations may be congenital or acquired. In the latter case, usually a traumatic injury to the arteries precedes the arteriovenous anastomoses. Two elderly patients presented with large, purple-colored verrucous tumors on the buttocks. Both patients were obese and immobile, and reported repeated bleeding from the lesions after minor trauma. The tumors were soft and could be emptied by applying pressure. Doppler examination revealed arterial pulsations over the lesions. Both cases were diagnosed as pressure-induced arteriovenous malformations. The lesions are assumed to have been caused by tissue damage in the deep subcutis induced by decubitus.

The skin in severe combined immunodeficiency: a case with transient cutaneous presence of γ/δ(TRC1+) T cells

Summary We report the case of a boy with severe combined immunodeficiency (SCID) and serious skin problems. The level of purine 5′‐nucleotidase was greatly reduced in the lymphocytes of this patient. To our knowledge, no patients with SCID and this enzyme deficiency have been described previously. The relationship between reduced levels of this enzyme and the immunodeficiency is unclear. This case is also unusual because of the presence of large numbers of T lymphocytes expressing TCR1 (γ/δ) in the skin. Moreover, the presence of so many TCR1‐positive cells was not consistent with the low numbers of these cells in the peripheral blood. These cells were not present in skin biopsies taken at a later stage during the course of the disease. An oligoclonal lymphocytosis developed during follow‐up, and a monoclonal antibody reactive with these clones was found, indicating that these lymphocytes were present in the skin.