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Musculoskeletal features of acne, hidradenitis suppurativa, and dissecting cellulitis of the scalp
Abstract
This article describes the various forms of acne and the clinical and radiographic features of the associated musculoskeletal manifestations. Occasionally, acne may occur together with hidradenitis suppurativa and dissecting cellulitis of the scalp, the so called "follicular occlusion triad." The current understanding of the etiology of these conditions and their treatment are also reviewed.
... Par exemple, des dépôts de complexes immuns circulants pourraient être responsables de l'atteinte articulaire, comme ce mécanisme bien connu est évoqué dans un certain nombre d'affections rhumatologiques réactionnelles. Quelques publications confirment cette hypo- thèse en constatant la présence de complexes immuns circulants ou de cryoglobulinémie (7,11,12,58,59,60,61). Cependant cette recherche n'a été ni systématique ni identique dans les cas rapportés. ...
Un certain nombre d’affections ont été décrites en coexistence avec l’hidradénite suppurée.
Le niveau de preuve d’une relation causale entre l’hidradénite suppurée et ces affections est faible.
Le cancer est une complication rare de l’hidradénite suppurée évoluée.
... Diseases of follicular occulsion in the skin are common fellow travelers with HS. The triad or tetrad of acne conglobata, dissecting cellulitis, hidradentitis, and pilonidal cysts is well reported [17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45] and it is likely that reports of the triad or tetrad without complicating factors are no longer being published. Sometimes each of the components of the tetrad can occur in isolation, but such isolated cases will not be discussed herein. ...
Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity. Pyoderma fistulans sinifica (fox den disease) appears to be the same entity as Hurley Stage 2 of 3 HS. The rate of acne vulgaris in HS patients mirrors unaffected controls. The most common, albeit still uncommon, association is with seronegative, haplotype unlinked arthritis (most importantly B27), in particular spondolyarthritis. Crohn disease and HS occur together at a rate that varies from 0.6% to 38% in retrospective cases series. Ulcerative colitis occurred with HS in 14% of patients in one series. The next most common association is with pyoderma gangrenosum, but this association is likely under-reported. Synovitis-Acne-Pustulosis Hyperostosis-Osteitis (SAPHO) syndrome, which is rare, has more than 10 reports linking it to HS. Nine case reports have linked Dowling-Degos disease (DDD) to HS and two reports related HS to Fox-Fordyce disease (FF), but because both occur in the axilla this might be a mere coincidence. HS is rarely associated with ophthalmic pathology. Specifically, more than 5 reports link it to Keratitis-Ichthyosis-Deafness syndrome (KID); greater than10 cases link it to interstitial keratitis and 2 cases are linked to Behçet's disease. The presence of proteinuria and acute nephritis link HS to the kidney, especially since and reports have documented resolution of HS after renal transplant. Florid steatocystoma multiplex, Sjogren Syndrome, and HS have been linked and their reports likely underestimate their coincidence because all these entities involve occlusion (albeit by different mechanisms). Three reports link HS and amyloid, but both share some common genetic underpinnings and thus the coincidence of these diseases is likely underreported. Pyoderma vegetans has been noted in 2 cases of HS and 4 cases of Inflammatory Bowel Disease (IBD) and is likely a clue to the linkage of the pathology of IBD and HS. Pityriasis rubra pilaris, in particular Type VI related to HIV, has a relationship more commonly with acne conglobata, but with HS also. Single case reports of diseases associated with HS include systemic lupus erythematosus, acromegaly, Down syndrome, Bazex-Dupre´-Christol, and prurtis ani, but these might be coincidences. Pyogenic Arthritis, Pyoderma gangrenosum, and Acne (PAPA Syndrome) and Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis (PASH Syndrome) are pyodermic-arthritic syndromes that are associated with HS. Erythema nodosum and granulomatous lobular mastitis have been reported with HS but the significance of these reports is uncertain. Because of scarring, HS can result in lymphedema including scrotal elephantiasis and verrucous lymphedema. HS is sometimes accompanied by obesity, hypertension, and anemia and can be considered a disease in the spectrum of metabolic syndrome, a skin disease with systemic consequences. HS, like other types of chronic inflammation when long standing in the perianal and perineal areas, can result in squamous cell cancer. A variety of drugs can induce HS. These include lithium, sirolimus, cyclosporine, vemurafenib, and oral contraceptives. Inverse psoriasis or psoriasis vulgaris as a side effect of infliximab therapy may be associated with HS. These associations aside, most cases of HS occur in isolation without coincident morbidity.
... Au cours de l'évolution, des complications ont été décrites : des complications osseuses destructrices sous-jacentes, des atteintes ostéo-articulaires proches du SAPHO [55][56][57][58][59] et la survenue de carcinome épidermoïde [60,61]. ...
... It is more frequent in black people, but the incidence in China is unknown. To date, a few cases of it have been reported in different countries (Olafsson and Khan, 1992;Meyers et al., 2003;Scheinfeld, 2003;Loo et al., 2004;Montgomery et al., 2004). The histopathological studies have identified acne inversa as a disorder of follicular rather than apocrine occlusion (Yu and Cook, 1990;Attanoos et al., 1995). ...
Acne inversa (hidradenitis suppurativa) is a chronic relapsing inflammatory skin disease characterized by recurrent draining sinuses and abscesses, predominantly in skin folds that carry terminal hairs and apocrine glands. The genetic basis for this disease is unknown. In this study, we performed a genome-wide scan in a four-generation Chinese family to map the chromosome location of the responsible gene. We first identified a locus at chromosome 1p21.1-1q25.3 with the maximum logarithm of odds (LOD) score of 3.26 at the marker D1S2624 (at recombination fraction=0.00). The other two-point LOD scores >/=3 were observed at markers D1S2695, D1S2726, D1S252, and D1S2777. Haplotype analysis localized this locus to a 76 Mb region flanked by D1S248 and D1S2711. This is the first locus for the inversa acne and will be a starting point towards understanding the molecular mechanisms of this disease.
Background:
Hidradenitis suppurativa (HS) is an inflammatory skin disease causing painful inflammation and suppuration. It may occur in rare syndromes: follicular occlusion, Bazex-Dupré-Christol, Down's, KID, PAPASH, PASS, PASH, and SAPHO syndromes, as well as Dowling-Degos disease. An overview of syndromic HS may inform the search for etiological factors in HS.
Methods:
Pubmed, Ovid, and Web of Science were systematically searched using "(hidradenitis OR acne invers*) AND (syndrome OR KID OR PASS OR PAPA OR PASH OR SAPHO OR bazex-dupre OR "dowling degos" OR triad OR tetrad)" and Cochrane Library using "hidradenitis OR acne invers*". A total of 82 articles were included in the final review.
Results:
We summarize 134 cases collected from the 82 included articles. The syndromes are discussed, focusing on etiopathogenesis, clinical presentation, and treatment. This article is protected by copyright. All rights reserved.
Funktionseinschränkung, Behinderung, Gelenkszerstörung und vorzeitige Sterblichkeit charakterisieren die chronische Polyarthritis (Wolfe et al. 2003). Da der Zerstörungsprozess an Knochen und Knorpeln nur selten reversibel ist (Sharp et al. 2003), muss frühzeitig mit einer Therapie begonnen werden, um Destruktionen zu verzögern oder zu verhindern. Glukokortikoide, „konventionelle“ DMARDs, Biologika oder Kombinationen dieser Substanzen können sowohl in der frühen als auch in späteren Phasen der Erkrankung die Progression der destruktiven Prozesse verzögern oder sogar verhindern. Die Kumulation struktureller Schäden könnte verhindert werden, wenn diese Medikamente möglichst früh verabreicht werden. Auch die zugrunde liegenden pathogenetischen Prozesse könnten umso wirksamer beeinflusst werden, je früher eine effektive Therapie begonnen wird.
Primary cicatricial alopecias can be frustrating for both patients and physicians. Proper diagnosis guides more successful management of these challenging conditions. Part II will cover the remaining lymphocytic primary cicatricial alopecias, which include pseudopelade of Brocq, central centrifugal cicatricial alopecia, alopecia mucinosa, and keratosis follicularis spinulosa decalvans. It will also discuss the neutrophilic and mixed primary cicatricial alopecias, namely folliculitis decalvans, dissecting cellulitis, folliculitis keloidalis, folliculitis (acne) necrotica, and erosive pustular dermatosis.
Chronische, entzündliche Systemerkrankung mit bevorzugtem Befall der Gelenke, gekennzeichnet durch eine polyartikuläre, symmetrische Synovitis mit destruierendem Charakter.
Background:
Dissecting cellulitis of the scalp (DCS) is a rare and long-standing disease and its aetiopathogenesis remains unclear. Various therapeutic alternatives exist. We attempted to assess the efficacy of oral isotretinoin in a series of patients.
Patients and methods:
Seven cases of DCS followed and treated by one of the authors at the Sabouraud Centre in the Saint-Louis Hospital between 2007 and 2013 were retrospectively studied and the efficacy of oral isotretinoin was evaluated.
Results:
The seven patients included were males aged between 22 and 39 years, with alopecic nodules on the occiput (two cases) or the top of the vertex (five cases). Most patients were given oral isotretinoin at a dosage of 0.75mg/kg/day for at least nine months. The outcome was satisfactory with complete healing of the lesions and good regrowth in all cases. Follow-up after recovery lasted between 16 and 42 months.
Conclusion:
Based on published studies and the results of the present study, oral isotretinoin at a dose of 0.75mg/kg/day may be proposed as first-line treatment for DCS.
The occurrence of musculoskeletal manifestations (including synovitis, chest wall arthro-osteitis and multifocal aseptic osteomyelitis) in association with severe acne, palmoplantar pustulosis and perhaps with some presentations of psoriasis, have been described by many authors in the past 30 years. These different multifaceted descriptions have been designated by a variety of terms. More recently, a possible link between these conditions and spondarthritides has also been underlined by a slightly increased prevalence of HLA-B27 and occasional occurrences of sacroiliitis, chronic inflammatory bowel disease and possibly psoriasis. An acronym, the SAPHO syndrome (which stands for Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) is proposed for this group of diseases because of the similarity of musculoskeletal manifestations in patients with severe acne and pustulosis. The clinical, epidemiological, pathophysiological, immunogenetic and diagnostic aspects, as well as the management of this syndrome, are reviewed.
To review and highlight the association of acne conglobata, hidradenitis suppurativa, and dissecting cellulitis of the scalp with inflammatory arthritic conditions, we report five illustrative patients with this association, and a review of the literature. All our patients were African-American males, and their skin disease present before the onset of arthritis. Both asymmetric peripheral arthritis and axial disease can occur. The arthritis is usually insidious and lacks association with rheumatoid factor and HLA-B27. Imaging of peripheral joints can reveal erosions, periosteal bone reaction and new bone formation. When the axial skeleton is involved, imaging can reveal sacroiliitis, syndesmophyte formation. NSAIDs, oral and intra-articular steroids, DMARDs and TNF alpha antagonists have all been used with success. Controlled trials with larger numbers of patients are needed to assess which treatment options are the most effective for this group of patients.
SAPHO syndrome is a disorder characterized by Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis. As the osteoarticular and skin manifestations often do not occur simultaneously and there are no validated diagnostic criteria, the diagnosis can be difficult. Clinical and imaging investigation is necessary to establish the many differential diagnoses of SAPHO syndrome. The etiopathogenesis involves infectious (probably Propionibacterium acnes), immunologic, and genetic factors. Treatment is based on information gathered from case reports and small series, and is related to specific skin or articular symptoms.
A number of diseases have been described as co-occurring with HS. The association may often occur by chance taking into account the prevalence of HS in the general population. The serendipitous observation of co-occurrence may however also reflect a causal relationship between the diseases, and thereby lead to a better aetiological or pathogenic understanding of both diseases. Taking all the suggested associations into account a very heterogeneous picture emerges, which does not allow hard conclusions to be made. This review of the literature strongly underlines the continued need for systematic observations in larger numbers of patients. The establishment of patient cohorts of sufficient size to allow longitudinal studies is therefore encouraged.
Comorbidities of hidradenitis suppurativa (acne inversa) were reviewed by extracting original and review publications included in MEDLINE, EMBASE and COCHRANE libraries using the terms "hidradenitis," "Verneuil" and "acne inversa." Follicular occlusion disorders, inflammatory bowel diseases, especially Crohn disease, spondylarthropathy, other hyperergic diseases, genetic keratin disorders associated with follicular occlusion and squamous cell carcinoma were the most common hidradenitis suppurativa comorbid diseases. A first classification of these major comorbidities and their possible genetic background reveals a list of chromosome loci and genes, which could be hidradenitis suppurativa candidates. Most of these diseases belong to the group of autoinflammatory disorders, where th17 cell cytokines seem to play a central role.
Primary cicatricial alopecias (PCAs) are a rare, but important, group of disorders that cause irreversible damage to hair follicles resulting in scarring and permanent hair loss. They may also signify an underlying systemic disease. Thus, it is of paramount importance that clinicians who manage patients with hair loss are able to diagnose these disorders accurately. Unfortunately, PCAs are notoriously difficult conditions to diagnose and treat. The aim of this review is to present a rational and pragmatic guide to help clinicians in the professional assessment, investigation and diagnosis of patients with PCA. Illustrating typical clinical and histopathological presentations of key PCA entities we show how dermatoscopy can be profitably used for clinical diagnosis. Further, we advocate the search for loss of follicular ostia as a clinical hallmark of PCA, and suggest pragmatic strategies that allow rapid formulation of a working diagnosis.
Dissecting cellulitis (also called perifolliculitis capitis abscedens et suffodiens) manifests with perifollicular pustules, nodules, abscesses and sinuses that evolve into scarring alopecia. It predominantly occurs in African American men between 20-40 years of age, but can occasionally affect other races and women too. Associated musculoskeletal findings are sometimes reported. When it occurs with acne conglobata, hidradenitis suppurativa, and pilonidal cysts, the syndrome is referred to as the follicular occlusion triad or tetrad. Its course is chronic and relapsing, and treatment is often difficult. Medical therapies include isotretinoin, antibiotics, and prednisone. Destructive therapies include X-ray therapy, surgical excision, and skin grafting. Laser epilation of hair follicles is a promising new therapy for dissecting cellulitis.
Perifolliculitis capitis abscedens et suffodiens (or dissecting folliculitis of the scalp or dissecting cellulitis of the scalp or dissecting perifolliculitis of the scalp) is a rare entity and constitutes the equivalent over the scalp, of hidradenitis suppurativa and acne conglobata. Etiologic factors are unknown. Diagnosis is proven histologically. Management is very difficult and consists in systemic administration or intralesional injection of several drugs or in surgical manipulations. An 18 year-old white patient with cystic infiltrations, alopecia plaques, pustules and other inflammatory elements (clinicohistological features consistent with dissecting folliculitis of the scalp), is presented. Isotretinoin topical application assured successful control of the disease and averted the evolution of the clinical aspect to scarring alopecia and nodule formation. Topical isotretinoin exercises a curative, inhibitory and antiproliferative action, in perifolliculitis capitis abscedens et suffodiens.
Arthritis is a well-recognized but uncommon accompaniment to several chronic cutaneous inflammatory conditions in which severe acne is one component. We report the case of a man with dissecting cellulitis of the scalp who developed severe peripheral and axial arthritis.
Unlabelled:
The cicatricial alopecias encompass a diverse group of disorders characterized by permanent destruction of the hair follicle and irreversible hair loss. Destruction of the hair follicle can result from primary, folliculocentric disease or as a secondary result. This article focuses on the former, or primary cicatricial alopecias. The cause and pathogenesis of many of these disorders are largely unknown. Although unique clinicopathologic features allow for accurate diagnosis in some cases, diagnostic certainty is often elusive and reflects the limits of present understanding. Classification of the primary cicatricial alopecias on the basis of pathology provides a diagnostic and investigational framework and, it is hoped, will facilitate future enlightenment. Details of classification, etiopathogenesis, clinicopathologic features, differential diagnosis, and practical management of the primary cicatricial alopecias will be discussed.
Learning objectives:
Upon completion of this learning activity, participants should be familiar with the following aspects of the primary cicatricial alopecias: (1) the new, consensus-issued classification scheme, (2) current understanding about etiopathogenesis, (3) salient clinicopathologic features, (4) differential diagnosis, and (5) therapeutic management.
Many inflammatory, metabolic and infectious diseases affect the skin and joints. Most of these, such as rheumatoid arthritis and systemic lupus erythaematosus, are considered to be rheumatic conditions with secondary skin involvement. However, several primary cutaneous diseases are associated with arthritis and may even present with joint symptoms prior to cutaneous lesions. Common skin disorders, such as acne and psoriasis, have well-known musculoskeletal manifestations. Other less common conditions, such as dermatomyositis, multicentric reticulohistiocytosis, pyoderma gangrenosum, Sweet's syndrome and various cutaneous vasculitides, also have frequent joint involvement. This review will discuss the clinical presentation, both cutaneous and musculoskeletal, diagnosis and management of these disorders.
Perifolliculitis capitis abscedens et suffodiens (PCAS) is a suppurative process that involves the scalp, eventually resulting in extensive scarring and irreversible alopecia. This condition often presents in males of African American origin.
This article describes the clinical presentation, diagnosis, and treatment of an Aboriginal Canadian male suffering from PCAS. A literature review on the etiology, pathology, differential diagnosis, and management is also discussed.
Careful analysis of the pathology and clinical presentation can aid in the timely diagnosis and management of this challenging condition. The clinician dealing with patients suffering from PCAS has several treatment options available to help successfully manage patients with straightforward or recalcitrant disease.
We evaluated 10 patients with hidradenitis suppurativa or acne conglobata who developed arthritis. In contrast to patients with acne fulminans and arthritis, all our subjects were adults over 22 years of age; nine were black; and four were women. Nine patients had episodic inflammatory oligoarthritis affecting mainly larger joints of the upper and lower extremities. Eight patients had roentgenographic evidence of peripheral arthritis, four with erosions. Nine had clinical axial arthropathy, but roentgenograms showed abnormalities of the axial skeleton in all 10 patients. Pyoderma gangrenosum, erythema nodosum, conjunctivitis, urethritis, and oral and penile ulcers occurred in some patients. Rheumatoid factor was negative in all patients; the erythrocyte sedimentation rate was elevated in nine; five had chronic anemia; four had circulating immune complexes; and complement components were elevated in four. There was no increased incidence of HLA-B27 or other HLA-B7 cross-reacting antigens. A temporal relation of skin and joint disease activity was suggested. We report a spondyloarthropathy associated with hidradenitis suppurativa and acne conglobata. Clinical and laboratory manifestations suggest the arthropathy may be reactive to chronic cutaneous infection.
Unlike acne conglobata, cystic acne, or tropical acne the syndrome of acute febrile ulcerative acne is sudden in onset, and associated with severe ulcerations, fever, and polyarthralgias. Comedo formation is not pronounced. Ulcers are filled with gelatinous granulation tissue. Response to curettage and corticosteroid hormone therapy is generally prompt, but malaise and arthralgias may persist for a long time.
• Eight cases of acne fulminans (acute febrile ulcerative acne) are compared with 13 previously reported cases. This rare disorder of male teenage patients is characterized by the sudden appearance of highly inflammatory, tender, ulcerative and crusted lesions on the back, chest, and face: it is one of the most scarring acute dermatologic disorders of young patients. At the onset of the disease, all patients showed febrile temperatures and marked leukocytosis; other systemic symptoms occurred with varying frequency. Polyarthralgia was noted in half of our series.Investigations of potential trigger mechanisms (eg, septisemia due to Corynebacterium acnes, immunologic defects, altered polymorphonuclear leukocyte function, Shwartzman phenomenon) did not yield any clues regarding the pathogenesis of this disease. Clinical and laboratory findings suggest that acne fulminans is not directly related to acne conglobata.(Arch Dermatol 113:444-449, 1977)
• Multifocal lytic bone lesions were found in a patient with a severe form of acne. The clinical presentation was suggestive of "acne fulminans." Propionibacterium acnes was isolated from a subculture of bone tissue. The association of acne fulminans and osteolytic lesions is rare and the pathophysiology is unknown. However, the use of corticosteroids for systemic effect combined with local measures seems to give excellent treatment results.
(Arch Dermatol 1985;121:662-664)
Acne conglobata has the same features as acne vulgaris plus double comedones and dissecting interconnecting subcutaneous abscesses which heal to leave scar bridges. This report describes the association of acne conglobata with an asymmetric self-limited, non-deforming peripheral arthritis in a young male. It is suggested that the association of acne conglobata and arthritis is the consequence of the same pathogenetic mechanism as that involved in the association of arthritis with ulcerative colitis.
Acne conglobate ha le mesme characteristicas como acne vulgar, sed—in plus—duple comedones e dissecante inter-connectite abscessos subcutanee que lassa—post lor curation—cicaticesponte. Le presente reporto describe le association de acne conglobate con asymmetric, auto-limitante arthritis non-deformante peripheric in un juvene masculo. Es formulate le suggestion que le association de acne conglobate con arthritis es le consequentia del mesme mechanismo pathogenetic que es responsabile pro le association de arthritis con colitis ulcerative.
In 25 of 35 cases of acne conglobata, a reduction in or lack of delayed reactivity was found by intracutaneous tests with a battery of antigens. On the basis of these findings as well as of normal T-cell function in lymphocyte culture and of negative Kveim-tests (in three cases), the possible immunological mechanisms are discussed.
Delayed hypersensitivity reactions were investigated in 5 patients with febrile acne conglobata. Each of them had febrile periods, large abscesses and leukocytosis. They reacted negatively to tuberculin 10 TU/ml, to Schick toxin, to oidiomycin, to trichophytin as well as to 25 common contact allergens. DNCB did not induce sensitization in those 4 patients in whom it was carried out. However, the transforamtion of lymphocytes by phytohaemagglutinin (PHA) was normal. Five of 7 control patients with cystic or ordinary acne had positive tuberculin test reactions and 3 of them were sensitized to DNCB.
A syndrome of arthralgia associated with one form of acne is described. Orthopaedic surgeons should be alert to the association of profound arthralgia and myalgia with this particular variant of acne vulgaris. Thirteen patients are described, all of whom are adolescent boys with a chronic moderately active acne which suddenly became extremely aggressive and toxic. The symptoms and signs included proximal arthralgia and myalgia, fever, elevated sedimentation rate, and altered immunoglobulins. Control of acne and conservative physical therapy resulted in complete resolution of the musculoskeletal complaints.
Patients with severe nodulo-cystic acne are known to have elevated serum antibody levels and increased immediate hypersensitivity reactions to Propionibacterium acnes. This organism is the predominant bacterium in normal pilosebaceous follicles of human skin, and can be consistently isolated from pustular lesions in acne. Previously it had been observed that delayed cutaneous hypersensitivity reactions to P. acnes were negative in patients with acne. The present study investigated the proliferative response of lymphocytes from patients with nodulo-cystic acne to phytohaemagglutinin (PHA) and P. acnes antigen stimulation. The response to PHA stimulation was within normal limits. The response to P. acnes antigen showed a significant increase over control values obtained by testing lymphocytes from acne-free subjects. Thus cell mediated immunity to P. acnes may be present in subjects with severe inflammatory acne. These findings raise the possibility that reactions to P. acnes may contribute to intensifying the inflammatory response in acne lesions.
Patients with varying degrees of acne (70), acne-free adult controls (28), and samples of cord blood (15) were investigated for cell mediated immunity to Propionibacterium acnes using a leukocyte migration inhibition test. Despite the fact that the mean migration index tended to decrease with acne severity, only the patients with severe acne showed cell-mediated immunity. It is suggested that when cellular immunity arises it is a late event which may contribute to inflammation but is probably not a factor in its initiation.
To better define the role of bacteria in inflammatory acne vulgaris, we have investigated the ability of four strains of Propionibacterium acnes and three strains of Propionibacterium granulosum to activate complement. Complement activation was assayed by incubating normal human serum with varying concentrations of each strain and measuring residual total hemolytic complement activity. When serum was tested unaltered, P. acnes strains were approximately threefold more potent than an equal weight of P. granulosum in consuming complement, which could reflect classical and/or alternative pathway activation. All strains also consumed complement in serum chelated with ethyleneglycol-bis (beta-aminoethyl ether)-N,N'-tetraacetic acid, which selectively assays alternative pathway activation. Incubation of unaltered serum with both P. acnes and P. granulosum resulted in immunoelectrophoretic conversion of C4, C3, and factor B of the alternative pathway. Incubation of chelated serum resulted in conversion of C3 and factor B. These data taken together suggest that both species can activate complement through either pathway. Serum incubated with P. acnes was chemotactic for polymorphonuclear leukocytes, and this chemotactic activity was largely C5 dependent as shown by antibody inhibition. It is suggested that complement activation may occur in vivo in acne, and the inflammatory response may be contributed to by the generation of C5-dependent chemotactic factors.
An 18-year-old West Indian male presented with severe sternal pain and an exacerbation of facial acne. Radiographs of the sternum revealed several lytic lesions which appeared as hot areas on successive technetium bone scans. Painful areas over the right iliac crest and left greater trochanter likewise appeared as transient hot areas on successive scans. Histology of affected bone revealed reactive changes only. High dose prednisolone provided rapid alleviation of pain, which recurred on reducing the dose to less than 10 mg daily. Auto-immune complex disease has been considered the most likely aetiological mechanism of systemic acne (acne fulminans), but lytic lesions of bone have never previously been reported in auto-immune disorders.
The following is a review of the literature concerning hidradenitis suppurativa with emphasis on aspects of this disease which suggest that it may be a result of altered host-defense mechanisms.Deep fistula formation, anemia, and the development of carcinoma are complications seen only in disease affecting the perianal area. The term perianal is used loosely to describe the buttock, perineum, pubic, and genital areas.A variety of treatment regimens has been used with limited success. Surgery has evolved as the treatment of choice for advanced and chronic disease.
Host-defense mechanisms were studied in seven patients with active hidradenitis suppurativa (HS). Granulocyte phagocytic function was measured by ingestion of Staphylococcus aureus labeled with radioactive carbon 14 and intracellular killing was determined by bactericidal pour plate method. Chemotaxis was measured by radioactive counting of sodium chromate Cr 51 granulocytes migrating in modified Boyden chambers. Granulocyte adherence was estimated in vitro by filtering blood samples through nylon fiber columns. Cell-mediated immunity was measured by intradermal delayed hypersensitivity responses to Candida, mumps. streptokinase/streptodornase, and purified protein derivative antigens. No abnormality was demonstrated in any granulocyte or cell-mediated immune function tests. Moreover, all patients had normal immunoglobulin levels and elevated total hemolytic complement. Therefore, we conclude that HS is a localized chronic infection of apocrine glands without a generalized defect in host defense.
The acne conglobata (AC)-, acne fulminans (AF)-, and isotretinoin-associated musculoskeletal syndromes are three distinct clinical entities. The AC-associated musculoskeletal syndrome occurs primarily in black men over the age of 22, who develop sacroilitis with or without a peripheral arthropathy. In contrast, the AF-associated musculoskeletal syndrome is found almost exclusively in white male teenagers. Fever, weight loss, and arthralgias are prominent components of this syndrome. A unique feature of the AF-associated musculoskeletal syndrome is osteolytic lesions that occur most frequently in the clavicle, sternum, long bones, and ilium. The isotretinoin-associated musculoskeletal syndrome occurs with equal frequency in male and female acne patients. Mild, transient myalgias and arthralgias are very common and do not require discontinuation of isotretinoin therapy. Asymptomatic, small, hyperostotic lesions of the spine occur in approximately 10% of acne patients with the isotretinoin-associated musculoskeletal syndrome.
As in other diseases of undetermined etiology, the diagnosis of ankylosing spondylitis (AS) and related spondyloarthropathies (SpA) is based on clinical and roentgenographic features. The current criteria for diagnosis of some of these diseases are too restricted, and do not recognize the existence of a much wider disease spectrum. For example, radiographically detected sacroiliitis is extremely frequent in AS, but may not be an obligate manifestation, especially in early or atypical forms of the disease. Arthritis involving the axial skeleton, including the sacroiliac joints, can be present in some patients without evidence of erosive disease roentgenographically. The disease spectrum of Reiter's syndrome has also been broadened considerably, and "incomplete" forms of Reiter's syndrome are observed much more commonly than the classical triad of arthritis, conjunctivitis, and urethritis. The term "B27-associated reactive arthritis" has been used in recent years to refer to SpA following enteric or urogenital infections, and the disease spectrum includes the clinical picture of typical Reiter's syndrome. The clinical spectrum of psoriatic SpA has been better clarified. Some of the less well defined B27-associated clinical syndromes include seronegative oligoarthritis, polyarthritis, or dactylitis ("sausagelike" toes) of the lower extremities, and heel pain caused by calcaneal (and tarsal) periostitis. These and other undifferentiated SpA have been ignored in previous epidemiological studies because of the inadequacy of the existing classification criteria. The European Spondylarthropathy Study Group (ESSG) has completed a study aimed at developing preliminary classification criteria for the whole group of SpA patients, with the specific intention of encompassing patients with undifferentiated SpA.
We report a case of a rare association between acne conglobata and ankylosing spondylarthritis B27 negative which occurred in a young man. The pathogenetic relationship of the association is not certain, although some researches suggest that the onset of ankylosing spondylarthritis can result from cutaneous disease. Despite the long evolution of ankylosing spondylitis, it is not severe. Thus, the presence of B35 CREG antigens confirm that locus B antigens different from B27 could be associated with a more favourable prognosis of the disease.
A family study of hidradenitis suppurativa was undertaken based on 26 subjects with the disease. The probands were obtained from Hospital Activity Analysis (HAA) records for a three year period (1980 to 1983) and by direct referral from hospital specialists over a six month period (1983 to 1984). Family pedigree information was collected by home visits or hospital interviews and confirmation of the disease in relatives was obtained by examination where possible or by telephone contact and hospital/GP records. A total of 62 affected persons was eventually ascertained, 40 females and 22 males. In 11 families there was evidence in favour of a genetic aetiology with single gene transmission. In another three families there was historical evidence of familial occurrence and in nine families no family history was obtained at the time of enquiry. Problems of ascertainment, including variability of age of onset and psychosocial factors, were noted and could be responsible for false negative family histories or underestimation of affected persons.
The association of acne conglobata and arthritis is rare and has been reported in only single case reports in the literature. We describe a patient whose associated arthropathy appeared 21 years after the onset of the skin disease. The relevant literature is reviewed.
Experience with six patients with severe acne and associated axial and peripheral arthritis is described. Four of the patients had a dermatologic triad of severe acne, hidradenitis suppurativa, and dissecting cellulitis of the scalp, the so-called follicular occlusion triad. All were black men with episodic peripheral arthropathy and low back pain. One had inflammatory bowel disease. Rheumatoid factor and HLA-B27 were absent in five patients who had these determinations. An erosive and proliferative arthritis of the axial and appendicular skeleton is described. The radiographic findings were indistinguishable from those of the seronegative spondylarthropathies. We found no previous reports in the radiologic literature describing this articulocutaneous entity.
Skeletal toxicity is known to occur with high doses of isotretinoin (greater than 2 mg/kg/day). We have attempted to evaluate the clinical significance and document the extent of musculoskeletal toxicity associated with a relatively low dose of isotretinoin (0.5 mg/kg/day) used in the treatment of severe acne. Radiographs of 120 patients were examined. Twelve per cent showed minor changes (four patients had spinal hyperostoses and 10 had calcaneal hyperostoses). None of the musculoskeletal changes we observed was clinically significant. Comparison with matched control X-rays showed 8% of the controls to have similar non-significant changes. Follow-up of 11 of the patients with abnormal X-rays showed minor deterioration in one patient, no change in four and improvement in six. Thus, doses of 0.5 mg/kg/day isotretinoin in such patients did not produce any significant long-term musculoskeletal changes. With increasing use of this beneficial drug in acne, radiologists and dermatologists should be aware of its skeletal toxicity.
A 24-year old man developed a bilateral sacroiliitis in association with acne conglobata. The acne conglobata was followed after the use of prednisone during 9 months. The prednisone was taken for a severe form of sarcoidosis. This may be a new instance of a relatively uncommon and poorly understood relationship between a particular form of acne and associated arthritis.
In order to determine which structures in Propionibacterium acnes are most antigenic to severe acne patients, we studied the specificity of anti-P. acnes antibodies in serum from 15 nodulocystic acne patients and 5 normals. Complement fixation titers to P. acnes cell wall fractions were determined using guinea pig serum as a complement source. The mean titers of patients and normals to whole cells were 39.6 and 3 (p less than 0.1); to crude cell wall, 138 and 8 (p less than 0.01); and to protein and nucleic acid-free cell wall, 225 and 9.33 (p less than 0.001), respectively. The mean precipitin titer to P. acnes cytosol was 12.7 for patients and 0 for normals. Immunoelectrophoresis of cytosol from 8 P. acnes strains were developed with each of the 15 patient sera. A single broadly migrating anionic antigen was detected. The antigen was also present in P. acnes culture supernatants. Sephadex G-100 chromatography of cytosol revealed a single peak of antigenic reactivity at Mr = 100,000. Three patients' sera revealed a second weakly reacting antigen in the cytosol preparation. Twentyfold concentration of immunoglobulin from patient sera failed to reveal any other antigenic reactivities. The antigen was found to be resistant to nuclease, pronase, and lysozyme treatment; was precipitable with 70% ethanol; and was destroyed by sodium m-periodate--findings that are consistent with a carbohydrate structure.
The association between hidradenitis suppurativa and joint involvement is well recognized. We describe a 63-year-old man with a severe HLA-B27 negative ankylosing spondylitis associated with hidradenitis suppurativa. We are not aware of any reports of such an association in the literature.
Treatment with isotretinoin (retinoic acid), which is frequently used in the control of acne, is associated with transient arthralgias in up to 16% of patients. We encountered two cases of acute, aseptic arthritis of the knee in male patients receiving isotretinoin, during the third week and third month of therapy. Synovial fluid obtained from one of the patients was noninflammatory. The drug concentration in the synovial fluid was 131 ng/mL--a level that was compatible with diffusion from the blood (simultaneous serum concentration, 229 ng/mL). Arthritis resolved in both patients without sequelae, despite continuation of drug treatment in one of them. This observation indicates that arthritis with joint effusion may complicate isotretinoin use; it also suggests that alternative measures should be considered before administering the drug to patients with rheumatologic disorders.
The etiology and pathogenesis of acne arthritis and the arthritis of hidradenitis suppurativa remain unknown. Some patients have an asymmetrical pauciarticular arthritis compatible with the 'reactive' peripheral and central forms of Reiter's syndrome, whereas others have a symmetrical polyarthritis/polyarthralgias syndrome. The cutaneous disease is clinically manifest as acne conglobata or hidradenitis of axilla or groin. We determined human leukocyte antigens (HLA), levels of immunoglobulins, C3, C4 and circulating immune complexes from six patients with acne arthritis. Four of six patients had HLA antigens of B7 Creg group (B7, BW22, B27, BW40, BW42) and all six possessed DRW4. Isolated elevations of immunoglobulins, C3 and C4 were observed. Immune complexes were elevated uniformly. These observations suggest that immunogenetic mechanisms may play a role in the systemic manifestations of these skin diseases.
An 18-year-old man with acne fulminans developed an acute inflammatory myositis, an association not previously reported. The condition resolved with treatment using systemic corticosteroids.
Arthralgias have previously been reported in association with acne fulminans, but the following case in the first report of osseous lesions associated with this disease. The destructive bony lesion was rapid in onset but self-limited.
We describe a relatively uncommon and poorly understood relationship between a particularly severe form of acne and an associated arthritis. Two young men are described, 1 of whom possesses the HLA-B27 antigen. Their clinical courses are described, responses to therapeutic intervention outlined, and some speculation regarding etiology is made. A review of the literature is included. The prognosis is uncertain, since 1 of the patients has sacroiliitis.
A 17-year-old girl, hospitalized because of severe cystic acne of six weeks' duration, developed symptoms of systemic disease, with fever, accelerated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, arthralgia and anemia. Extensive investigation ruled out other possible causes for the systemic involvement, and there was a prompt response to steroid therapy. This case had features similar to those associated with "acute, febrile, ulcerative conglobate acne with poly-arthralgia," except that there was an absence of ulcerative lesions: nonetheless, the dominant picture was that of cystic involvement. We believe that the use of the term "acne fulminans" for this and similar cases would be appropriate.
HIDRADENITIS suppurativa and the more severe types of acne, such as acne conglobata and dissecting cellulitis of the neck and scalp, not infrequently occur in the same persons. These disorders have been described as distinct entities; however, in clinical characteristics and in the course of the disease, they have many similarities which indicate a close relationship among them. They may be considered as regional counterparts in which the acne process is manifested in its extreme variants and in its more fulminating forms. Acne conglobata, dissecting cellulitis, hidradenitis, and other variants of acne have these features in common: functional glandular hyperplasia of the apocrine gland and pilosebaceous apparatus; double comedo formation and impaction of the follicular orifice; bacterial invasion with suppuration and undermining of the loose areolar tissue, and eventual healing with cicatrization and not infrequent keloid formation.
This presentation is concerned with a correlation of various aspects of the
Manifestations rhumatismales de l’acne conglobata
- Bastin
Bone lesions of acne fulminans
- Jemec