Should hidradenitis suppurativa/acne inversa best be renamed as "dissecting terminal hair folliculitis"?

Hidradenitis suppurativa/acne inversa is a diverse, enigmatic and distressful disease that has aroused growing interest in specialists from different disciplines. Both names describe its classical manifestations in the intertriginous regions and reflect the historical view of the disease definition, but cause confusions in the understanding of its pathogenesis and classification. In the light of the advance in clinical, histopathological and pathophysiological findings, we propose the term "dissecting terminal hair folliculitis" (DTHF) to characterize its disease nature as folliculitis instead of acneiform disease or apocrine gland disorder. DTHF attacks exclusively the terminal hair follicles in an overwhelming majority of adults, initiating from the fragile acroinfundibulum leading to a non-infectious overreaction of innate immunity system with inflammation that may fiercely dissect and engulf all the surrounding tissues accompanied by secondary bacterial infections. Evidence indicates that perifolliculitis capitis abscedens et suffodiens and pilonidal disease are very likely regional variants of DTHF with the same pathogenesis. Treatment of DTHF remains frustrating. The benefit of biologics in targeting inflammation is so far non-specific, palliative and inconsistent. Hair epilation and photodynamic therapy in treatment of the disease is questionable in consideration of the pathogenesis. Genetic and translational research, especially on the Notch signalling pathways, will yield breakthrough in the development of novel treatment modalities.

Who described Civatte bodies?

Eosinophilic apoptotic (necrotic) keratinocytes in the lower epidermis and at the dermoepidermal junction are a feature of many interface dermatoses but are most reliably found in lichen planus. These structures are universally known as Civatte bodies. Nonetheless, they were first described by Raymond Sabouraud in 1912. Even after Achille Civatte discussed and beautifully illustrated them a decade later, it took until the late 1960s for the term Civatte body to win acceptance.

Impaired Notch-MKP-1 signalling in hidradenitis suppurativa: an approach to pathogenesis by evidence from translational biology.

Recent findings in familial hidradenitis suppurativa (HS) demonstrated loss-of-function mutations of components of the γ-secretase (GS) complex leading to decreased protease cleaving activity, which may compromise canonical Notch signalling. Appropriate Notch signalling is of pivotal importance for maintaining the inner and outer root sheath of the hair follicle and skin appendages. This viewpoint on the pathogenesis of HS is primarily supported by circumstantial evidence derived from translational biology. Impaired Notch signalling is proposed to be the major pathogenic mechanism of HS. Deficient Notch signalling switches the fate of outer root sheath cells, resulting in conversion of hair follicles to keratin-enriched epidermal cysts. Impaired Notch signalling may compromise apocrine gland homoeostasis as well. Damage-associated molecular pattern molecules released by either ruptured epidermal cysts exposing keratin fibres or altered structural components of less maintained apocrine glands may both stimulate TLR-mediated innate immunity. All aggravating factors of HS, that is, smoking, obesity, skin occlusion, androgens and progesterone, may further promote inflammation by release of proinflammatory cytokines derived from activated monocyte/macrophages. Inappropriate Notch signalling may not only initiate inflammation in HS but may lead to insufficient feedback inhibition of overstimulated innate immunity. Regular Notch signalling via induction of MAPK phosphatase-1 (MKP-1) terminates TLR-MAPK-signalling in macrophages and IL-23 secreting DCs, the key players for Th17 cell polarization. Thus, impaired Notch signalling links HS to other Th17-driven comorbidities. All major therapeutic interventions in HS appear to attenuate increased MAPK activation of innate immune cells due to impaired Notch-mediated feedback regulation of innate immunity.

Acne: risk indicator for increased body mass index and insulin resistance.

Acne appears to represent a visible indicator disease of over-activated mTORC1 signalling, an unfavour-able metabolic deviation on the road to serious common Western diseases of civilisation associated with increased body mass index and insulin resistance. Exaggerated mTORC1 signalling by Western diet explains the association of acne with increased body mass index, insulin resistance, and early onset of menarche. Both, a high glycaemic load and increased consumption of milk and milk products, staples of Western diet, aggravate mammalian target of rapamycin complex 1 signalling. This review of the literature summarises present evidence for an association between acne, increased body mass index, insulin resistance and Western diet. By dietary intervention with a Palaeolithic-type diet, the dermatologist has the chance to attenuate patients' increased mTORC1 signalling by reducing glycaemic load and milk consumption, which may not only improve acne but may delay the march to more serious mTORC1-driven diseases of civilisation.

Interleukin-4 therapy of psoriasis induces Th2 responses and improves human autoimmune disease.

Selective skewing of autoreactive interferon-gamma (IFN-gamma)-producing T helper cells (Th1) toward an interleukin-4 (IL-4)-producing (Th2) phenotype can in experimental animals alleviate autoimmune disease without inducing general immunosuppression. In a prospective dose escalation study, we assessed treatment with human IL-4 (rhuIL-4) in 20 patients with severe psoriasis. The therapy was well tolerated, and within six weeks all patients showed decreased clinical scores and 15 improved more than 68%. Stable reduction of clinical scores was significantly better at 0.2-0.5 microg rhuIL-4 than at < or =0.1 microg rhuIL-4 (P = 0.009). In psoriatic lesions, treatment with 0.2-0.5 microg/kg rhuIL-4 reduced the concentrations of IL-8 and IL-19, two cytokines directly involved in psoriasis; the number of chemokine receptor CCR5+ Th1 cells; and the IFN-gamma/IL-4 ratio. In the circulation, 0.2-0.5 microg/kg rhuIL-4 increased the number of IL-4+CD4+ T cells two- to three-fold. Thus, IL-4 therapy can induce Th2 differentiation in human CD4+ T cells and has promise as a potential treatment for psoriasis, a prototypic Th1-associated autoimmune disease.

[Adjunctive acne therapies--2010]. / Ergänzende Verfahren in der Aknetherapie--Aktualisierung 2010.

Modern acne therapy is based on the application of anticomedogenic, antimicrobial, or antiinflammatory substances, and antiandrogens. Additionally, traditional or newly developed therapeutic approaches may be considered, including pharmacologic (dapsone, zinc) and physical measures (phototherapy, photodynamic therapy, laser, comedone extraction, abrasives). This article reviews such adjunctive therapies with regard to efficacy and their roles in the therapy of acne.

Blemished noses in the art of three masters: Ghirlandaio, Rembrandt, and Warhol.

Blemished noses are portrayed in the paintings of such noted artists as Ghirlandaio, Rembrandt, and Andy Warhol. Sometimes, the deformity results from a skin disorder such as rhinophyma as in Ghirlandaio's An Old Man and his Grandson or a saddle nose deformity from congenital syphilis as in Rembrandt's Portrait of Gerard de Lairesse. Andy Warhol's Before and After portrays a large nose before and after cosmetic surgery. This contribution explores some of the lessons that can be learned, both artistically and medically, from these famous works of art.

PUVA-bath photochemotherapy and isotretinoin in sclerodermatous graft-versus-host disease.

Chronic graft-versus-host disease (GVHD) is almost always associated with skin diseases appearing either as lichenoid GVHD, sclerodermatous GVHD (sGVHD) or as eosinophilic fasciitis-like disease. The two latter frequently result in severe and deep sclerosis. Immunosuppressive therapy is of little help in sclerodermatous or eosinophilic fasciitis-like types of GVHD. Based on data showing that PUVA-bath photochemotherapy is effective in the treatment of severe localized sclerosis of the skin, we investigated the efficacy of PUVA-bath photochemotherapy and isotretinoin in sGVHD. In a retrospective study we analyzed fourteen consecutive patients with sGVHD who received PUVA-bath photochemotherapy, five in combination with oral isotretinoin. Seven patients improved and four showed complete remission. Surprisingly, the therapy was complicated by the development of ulcers within the sclerotic plaques during the early periods of treatment. These ulcers cleared in most patients when PUVA-bath photochemotherapy was continued. Thus, PUVA-bath photochemotherapy alone or in combination with isotretinoin may resolve or improve GVHD associated sclerosis in selected patients.

Late onset of a facial variant of steatocystoma multiplex - calretinin as a specific marker of the follicular companion cell layer.

Steatocystoma multiplex is a rare,almost exclusively autosomal dominant disease of the sebaceous glands. The sites of predilection are the sternal region, upper back, axillae and proximal parts of the extremities. A 78-year-old woman developed multiple yellowish nodules only on the face over a few months. Histology revealed large cysts with a thin wall and connection to sebaceous glands. Immunohistochemical analysis showed the inner epithelial layer of the cysts was positive with calretinin.With curettage and CO2 laser therapy, a good cosmetic result was achieved.This is the first case report in the German literature of a rare sporadic form of steatocystoma multiplex with late onset and exclusive localization in the face.

[Expert opinion on occupational skin diseases: a Munich statement regarding the Bamberg medical bulletin (Bamberger Merkblatt)]. / Begutachtung von Berufskrankheiten der Haut: Eine Münchner Stellungnahme zum Bamberger Merkblatt.

The Bamberg medical bulletin (Bamberger Merkblatt) is intended to assure as an anticipated expert opinion an objective as possible assessment of the sequels of an occupational disease. However, the Bamberg medical bulletin is not suitable for this task in form and content. In case of an allergic contact dermatitis recognized under No. 51 01 in appendix 1 of the German ordinance on occupational diseases the disadvantage for the insured person consists mainly in the live-long existing change of the immune system and not as much in the intensity of the skin condition triggered by a new exposure to the allergen. The prerequisite for compensation imposed by the regulator, i.e. that the skin condition must be so severe as to have forced the affected individual to refrain from all activities which led or could lead to the development, aggravation or recurrence of the condition, is in its function as a typing sign widely unnecessary in the case of an allergic occupational dermatosis. The omission of all activities which led or could lead to the development, aggravation or recurrence of the condition imposed on the affected individual by the regulator has to be taken in account for the calculation of the reduction in earning capacity in the Bamberg medical bulletin. The Bamberg medical bulletin is by no means an anticipated expert opinion. Even as a qualified experience it urgently needs a revision which might be done with, but not governed by, the statutory occupational accident insurance. Our critical scientific comments do not absolve the medical evaluator from the responsibility to consider existing recommendations as the Bamberg medical bulletin. Our aim is to initiate a process leading to an appropriate judgement of the sequels of an occupational skin disease in each individual case.

[Betel quid chewing: traditional habit with side effects]. / Betelkauen ist in weiten Teilen Asiens fester Bestandteil des Alltags. Traditioneller Genuss mit Nebenwirkungen.

The chewing of a betel quid or nut is a widespread tradition, in particular in Southeast Asia, for example, as a means of welcoming guests and friends. The ceremony surrounding the betel quid has become a highly developed cult, for which artistically designed instruments, bowls and boxes are used. The major component of betel quid, the seed of the areca palm (Areca catechu), is a psychotropic drug, the side effects of which include the destruction of the teeth, impairment of oral hygiene and bronchial asthma. The betel quid is an oral carcinogenic. The addition of tobacco increases the risk of cancer of the pharynx and upper gastrointestinal tract.