T. Rufli

Mu-Opiate Receptor and Beta-Endorphin Expression in Nerve Endings and Keratinocytes in Human Skin

We have previously shown that human epidermal keratinocytes express a functionally active µ-opiate receptor, which adds a new dimension to the recently developed research in neuroimmunodermatology and neurogenic inflammation in skin diseases. Human keratinocytes specifically bind and also produce β-endorphin, the endogenous µ-opiate receptor ligand. Using confocal imaging microscopy, we could now demonstrate that µ-opiate receptors are not only expressed in keratinocytes, but also on unmyelinated peripheral nerve fibers in the dermis and epidermis. Some of the peripheral nerve fibers also express the ligand β-endorphin. The keratinocytes positive for β-endorphin staining are clustered around the terminal ends of the unmyelinated nerve fibers. Therefore the opiate receptor system seems to be crucial in the direct communication between nerves and skin. The keratinocytes can influence the unmyelinated nerve fibers in the epidermis directly via secreting β-endorphin. On the other hand, nerve fibers can also secrete β-endorphin and influence the migration, differentiation and probably also the cytokine production pattern of keratinocytes.

Natural history of the Naegeli-Franceschetti-Jadassohn syndrome and further delineation of its clinical manifestations

BACKGROUND The Naegeli-Franceschetti-Jadassohn (NFJ) syndrome is rare; only three families have been reported. OBJECTIVE Our purpose was to determine the natural history of this ectodermal dysplasia and to delineate further its clinical manifestations. METHODS We reexamined the original family with the NFJ syndrome 65 years after the first description. RESULTS The pedigree includes 62 members with 14 affected patients. We examined the 10 living patients. Longitudinal analysis of the pedigree revealed that the reticulate pigmentation fades after puberty and may disappear completely in old age. Hypohidrosis, the main problem for the patients, remains constant. Teeth are always severely affected, leading to early total loss. All patients lack dermatoglyphics. Diffuse palmoplantar keratoderma may coexist with punctate keratoses that are sometimes accentuated in the creases or exhibit a linear pattern. Four patients had congenital malalignment of the great toenails, not described previously in association with the NFJ syndrome. CONCLUSION The NFJ syndrome is an ectodermal dysplasia with numerous specific abnormalities.

Acrodermatitis chronica atrophicans: A chronic T-cell-mediated immune reaction against Borrelia burgdorferi?: Clinical, histologic, and immunohistochemical study of five cases

Background: Acrodermatitis chronica atrophicans (ACA) is a late manifestation of infection caused by Borrelia burgdorferi . Objective: Our purpose was to study the clinical, histopathologic, and immunohistochemical findings in patients with ACA to understand better the pathogenesis of the disease. Methods: Five patients were studied. Skin biopsy specimens were obtained from active lesions for histologic and immunohistochemical studies. Results: Clinical lesions included an initial erythematous discoloration in one patient and violaceous infiltrated plaques and nodules in four patients, three of whom also had late atrophic lesions. Biopsy specimens showed a dermal perivascular and interstitial lymphocytic infiltrate with plasma cells. There was a predominance of CD3 + , CD4 + T cells in the dermal infiltrate. B cells were present in three patients. The dermal infiltrate showed an intense expression of lymphocyte function-associated antigen. The intercellular adhesion molecule type 1 was expressed on endothelial cells, perivascular mononuclear cells, and focally on basal ke-ratinocytes. Conclusion: Our findings suggest that a chronic, T-cell-mediated immune reaction against B. burgdorferi is involved in the pathogenesis of ACA.

Erythema chronicum migrans: Evidence for Cellular Immune Reaction in the Skin Lesion

Skin biopsy specimens from 9 patients with erythema chronicum migrans (ECM) were studied immunohistochemically using a series of monoclonal antibodies. In biopsy specimens taken from the erythematous peripheral portion of ECM the perivascular infiltrates were composed predominantly of LEU-4+ T cells. LEU-3a + helper/inducer T cells were more numerous than LEU-2a + cytotoxic/suppressor T cells. Of particular interest was the high number of LEU-6+ Langerhans cells in the epidermis and dermis of specimens taken from the erythematous portion of ECM as well as from the noninflammatory skin outside the erythema. The presence of LEU-6+ Langerhans cells and T cells in the ECM lesions suggests that, apart from humoral factors, a cell-mediated immune response directed against Borrelia burgdorferi antigen is important as well in the pathogenesis of this disease.

Follicular mucinosis associated with mycosis fungoides.

We report a patient who developed erythematous indurated plaques with alopecia on the face and multiple well-demarcated infiltrated scaling lesions on the trunk and extremities. Skin biopsy showed a mucinous degeneration of the follicular outer root sheaths and sebaceous glands consistent with the diagnosis of follicular mucinosis. A histologic examination of a specimen from the left forearm showed microscopic changes of mycosis fungoides. The present case confirms the need of multiple skin biopsies in the evaluation of patients with follicular mucinosis.

Is there a relation between risk groups or initial CD4 T cell counts and prevalence of seborrheic dermatitis in HIV-infected patients?

Objectives: (i) To investigate whether there is a difference in the prevalence of seborrheic dermatitis (SD) between homo- or bisexual HIV-infected patients and HIV-infected intravenous drug users, (ii) to study whether the initial CD4 T cell count at the first positive HIV test is of any significance for the prevalence of SD and furthermore to analyze whether (iii) antiretroviral treatment influences the prevalence and time course of SD. Patients and Methods: Since 1992 we have been following, within the scope of the Swiss HIV Cohort Study, a group of individuals with proven HIV infection. In this study all HIV-infected patients belonging either to the risk group of homo- or bisexuals or that of intravenous drug users were included for further analysis. Results: We included 226 men and 51 women. The ages ranged from 17 to 68 years (mean 30.1). One hundred and forty-four were homo- or bisexual men and 133 (82 men and 51 women) were intravenous drug users. Out of these 277 HIV-infected patients, 66 (23.8%) had SD at baseline and 7 (2.5%) developed SD during the observation period (male:female = 68:5). Conclusion: In our study we found that (i) the risk group influences the prevalence and time course of SD, yet that (ii) neither the initial CD4 T cell count nor (iii) antiretroviral treatment is of any significance.

Lichen planus of the eyelids.

Ocular involvement of lichen planus (Lp) is a rare event. Only 10 cases with Lp of the eyelids have been reported in the literature. Ocular Lp may also affect mucosal surfaces of the eye and lead to cicatrizing conjunctivitis. Lp of the eyelids may appear as isolated lesions and in those cases diagnosis is very difficult. Lp should be considered in the differential diagnosis of all erythematous and papular eruptions of the eyelids together with lupus erythematosus, psoriasis vulgaris or contact dermatitis.

The Petrified Ear

A case of an otherwise healthy 66-year-old male with bilateral symmetric ossification of his ears is described. The rare occurrence of petrified ears is emphasized, and possible etiologic factors are discussed. No other explanation than anamnestic prolonged exposure to hypothermia was found.

Acrodermatitis chronica atrophicans involving the face. Evidence for Borrelia burgdorferi infection confirmed by DNA amplification.

In a female patient with increasing redness of the hands and face, proteins of Borrelia burgdorferi were detected in a biopsy of the ear by DNA amplification. Although acrodermatitis chronica atrophicans has been documented to be caused by B. burgdorferi, this is the first case with proven spirochetal presence in the skin of the head. After 2 weeks of intravenous therapy with ceftriaxone marked improvement of discoloration of the skin was noted.

Localized Unilateral Hyperhidrosis

We describe a case of localized unilateral hyperhidrosis on the forehead in a 52-year-old woman. The pathophysiology of this unique clinical picture and its treatment are described.