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Acrokeratosis verruciformis: (Hopf)--A clinical entity?

Contrary to the unitarian concept of acrokeratosis verruciformis and Darier's disease, a comparative familial, clinical and histopathological analysis of six cases each of these two diseases has suggested that they are separate entities. Though clinically similar, acrokeratosis remained non-dyskeratotic throughout life, whereas the acral lesions of Darier's disease showed, on careful scrutiny and follow-up, various gradations of benign acantholytic dyskeratosis. Malignant transformation indicated that dyskeratosis in Hopf's disease would be very rare and of a different nature. The probability of a genetic linkage between the two genodermatoses is reviewed and discussed.

Acrodermatitis↗

A new apparatus for the delivery of high intensity UVA and UVA+UVB irradiation, and some dermatological applications.

A new apparatus consisting of a 200 W super pressure mercury lamp and a flexible light guide with a liquid light-conducting core is presented. Its transmission for UVA irradiation is more than threefold as compared to conventional quartz fibre optic bundles. The light guide is capable of transmitting density levels exceeding I W/cm2. UVA and UVB are transmitted. By inserting a filter platelet (WG 345) into the light guide the UVB is cut off. The output of the unfiltered guide is 700 mW (3570 mW/cm2), of the filtered guide 400 mW (2040 mW/cm2), centered mainly at 366 nm. The light apparatus proved useful for several dermatological applications. It replaces in many respects conventional Kromayer lamps, especially because the flexible light guide, which is 160 cm long and weighs only 210 g, can be brought into direct contact with the skin. Results of determining the minimal erythema dose (MED), of phototesting (8-MOP photoallergy, persistent light reactor, erythropoietic protoporphyria) and of photochemotherapy are given.

Acrodermatitis↗

Dermatitis repens, Lapière and generalized pustular psoriasis.

The presence of subcorneal pustules in a solitary, indolent, crusted plaque, or in erythema annulare-like lesions with a trailing scale, is evidence of atypical psoriasis. When these lesions appear late in life there is a significant risk of generalized pustular psoriasis with a poor prognosis. A case is presented in whom both of these atypical forms occurred and who developed severe generalized disease. Clinical and histological features are described.

Acrodermatitis↗

Kaposi-like acroangiodermatitis in an above-knee amputation stump.

The clinical, histological and immunohistological features of a Kaposi-like acroangiodermatitis in an above-knee amputation stump are reported. There was no evidence for an underlying arteriovenous malformation and the irregular purple-blue skin lesions resulted from chronic circulatory disturbance of the distal stump caused by a poorly fitting suction-type prosthesis.

Acrodermatitis↗

Genetic heterogeneity in acrokeratosis verruciformis of Hopf.

BACKGROUND: Acrokeratosis verruciformis of Hopf (AKV) is a rare genodermatosis characterized by multiple flat-topped, flesh-coloured papules on the dorsa of hands and feet, and punctuate keratoses on the palms and soles. A mutation in the ATP2A2 gene has been shown to be associated with AKV and with Darier's disease (DD). OBJECTIVES: To explore the molecular aetiology of AKV and DD. METHODS: We investigated the clinical and histological information in two families and a sporadic case with AKV and one family and a sporadic case with DD in China. Mutation analysis of ATP2A2 was performed by PCR and direct sequencing, and genotyping and linkage analysis performed using six polymorphic microsatellite markers spanning the locus at 12q23-12q24 containing ATP2A2. RESULTS: Mutational analysis showed no mutation in ATP2A2 among the AKV patients, but we found two novel mutations (p.C318F and p.M719fs) in the DD patients. The genotyping and linkage analysis results revealed no linkage evidence of the locus at 12q23-12q24 in a large AKV family. CONCLUSIONS: Our findings provide evidence for the genetic heterogeneity of AKV and demonstrate that mutations in genes other than ATP2A2 are responsible for AKV in a proportion of the Chinese population.

Acrodermatitis↗

Expression of HLA-DQ antigens on keratinocytes in Borrelia spirochete-induced skin lesions.

Skin biopsies were investigated with two different immunohistochemical techniques, thus revealing HLA-DQ antigens on HLA-DR-expressing keratinocytes in the late skin manifestations of a Borrelia spirochete infection. In the early skin lesions only HLA-DR antigens were present on the keratinocytes. The invariant gamma chain of class II transplantation antigens was observed on keratinocytes in 1:5 of the late cases. Upon penicillin treatment detectable HLA-DR and HLA-DQ antigens disappeared completely from the keratinocytes. Furthermore, the mononuclear cell infiltrates dominated by anti-Leu 1 and anti-Leu 3a-reactive cells and containing many cells with markers for activation (HLA-DR, HLA-DQ, transferrin, and interleukin 2 receptors) diminished markedly. The possibility that the expression of different class II transplantation antigens on keratinocytes might reflect separate functional demands of these cells or an altered immunological reactivity in the host, is discussed. The precise functional role of the temporary expression of the class II antigens on non-lymphoid cells, however, remains an enigma.

Acrodermatitis↗

Acroangiodermatitis: a study of ten cases.

BACKGROUND: Acroangiodermatitis is a benign vesicular process encountered on the lower extremities that histologically resembles the superficial form of stasis dermatitis, but is clinically characterized by circumscribed violaceous, brown or dusky macules, papules, or plaques. Furthermore, unlike stasis dermatitis, acroangiodermatitis is usually associated with minimal epidermal changes and eosinophils in the inflammatory infiltrate in the dermis. Kaposi's sarcoma, pigmented purpura, vasculitis, and lichen planus are other conditions that should be considered when making a diagnosis of acroangiodermatitis. METHODS: Ten patients with acroangiodermatitis were referred to us by area physicians. Hematoxylin and eosin, periodic acid-Schiff, Pearl's (an iron stain), and an immunoperoxidase stain for Factor VIII were performed on routinely embedded paraffin sections. RESULTS: All cases showed new vessel proliferation, perivascular inflammation of superficial and mid-dermis, consisting of lymphocytes, histiocytes, eosinophils, occasional plasma cells, extravasation of red blood cells, and hemosiderin pigment deposition. Dermal fibrosis was observed in all cases. None of the patients showed changes of vasculitis or Kaposi's sarcoma. Only one patient displayed significant epidermal changes of spongiosis and mild acanthosis. CONCLUSIONS: Acroangiodermatitis is an uncommon entity with peculiar clinical and histologic features and should be confirmed by histologic tests.

Acrodermatitis↗