Pilonidal sinus disease: an intergluteal localization of hidradenitis suppurativa/acne inversa: a cross-sectional study among 2465 patients.

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.

[Acne flare on isotretinoin: A pointer to diagnosis of hidradenitis suppurativa]. / « Poussées inflammatoires d'acné ¼ sous isotrétinoïne révélant une hidradénite suppurée : 4 cas.

BACKGROUND: Isotretinoin is the most potent treatment for acne but paradoxical flares can occur. HS lesions of the face may be mistaken for acne. We report on 4 patients in whom an "acne" flare on isotretinoin led to the correct diagnosis of HS. PATIENTS AND METHODS: Four young male patients aged 15 to 28 years were referred to us because of an acne flare on isotretinoin. Three of them had clinical features considered unusual in acne : involvement of the nape of the neck (2), retroauricular cysts (2), a rope-like pre-sternal lesion (1), a large bag-like sinus on the face (1), large deep depressed U-type scars on the back (3). Questioning revealed that all three had previously experienced several attacks of inflammatory nodules in the axillae and/or inguinal folds; they had not mentioned these lesions since they seemed so minor. The fourth patient had lesions typical of HS comprising nodules, sinus, rope-like hypertrophic scars on the face mistaken for acne, epidermal cysts on the scrotum and pubic folliculitis. Treatment with systemic antibiotics resulted in regression of lesions in all 4 patients. DISCUSSION: An acne flare on isotretinoin requires investigations with a view to potential diagnosis of HS. Patients presenting "acne" and atypical features such as involvement of the neck, large U scars and cord-like structures should be questioned about the presence of nodules in the axillae and groin since patients with mild HS may not spontaneously acknowledge such typical symptoms.

European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa.

Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillae, inguinal and anogenital regions. A mean disease incidence of 6.0 per 100,000 person-years and an average prevalence of 1% has been reported in Europe. HS has the highest impact on patients' quality of life among all assessed dermatological diseases. HS is associated with a variety of concomitant and secondary diseases, such as obesity, metabolic syndrome, inflammatory bowel disease, e.g. Crohn's disease, spondyloarthropathy, follicular occlusion syndrome and other hyperergic diseases. The central pathogenic event in HS is believed to be the occlusion of the upper part of the hair follicle leading to a perifollicular lympho-histiocytic inflammation. A highly significant association between the prevalence of HS and current smoking (Odds ratio 12.55) and overweight (Odds ratio 1.1 for each body mass index unit) has been documented. The European S1 HS guideline suggests that the disease should be treated based on its individual subjective impact and objective severity. Locally recurring lesions can be treated by classical surgery or LASER techniques, whereas medical treatment either as monotherapy or in combination with radical surgery is more appropriate for widely spread lesions. Medical therapy may include antibiotics (clindamycin plus rifampicine, tetracyclines), acitretin and biologics (adalimumab, infliximab). A Hurley severity grade-relevant treatment of HS is recommended by the expert group following a treatment algorithm. Adjuvant measurements, such as pain management, treatment of superinfections, weight loss and tobacco abstinence have to be considered.

[Severe tularaemia mimicking glandular tuberculosis during adalimumab therapy]. / Tularémie sévère mimant une tuberculose ganglionnaire au cours d'un traitement par adalimumab.

BACKGROUND: Tularaemia is an anthropozoonosis, transmitted by small mammals (hares) and arthropods (ticks, horseflies). The causative agent is Francisella tularensis, a facultatively intracellular Gram-negative bacillus. We report a case of tularaemia in its ulceroglandular form occurring during methotrexate and adalimumab treatment (Humira) for rheumatoid arthritis. PATIENTS AND METHODS: A 58-year-old man with a history of primary tuberculosis receiving adalimumab in combination with methotrexate for rheumatoid arthritis for almost 1 year consulted for a febrile inflammatory plaque on the left leg with a small central necrotic area. An enlarged left inguinal lymph node was present. Doxycycline has previously been prescribed for a tick bite. The lymphadenopathy gradually became enlarged resulting in skin fistulisation. After surgical excision, histopathology revealed epithelioid granulomas accompanied by giant cells and central necrosis. Mycobacterial cultures were negative. Positive tularaemia serology at significant titres suggested a diagnosis of tularaemia, with probable transmission via a tick bite. The diagnosis was confirmed by F. tularensis DNA amplification using PCR on a lymph node biopsy. Doxycycline was continued for a further 6 weeks. One year later, no relapse had occurred. DISCUSSION: A febrile adenopathy presenting the histological features of necrotic granulomas in a patient receiving anti-TNF alpha treatment initially suggested reactivation of tuberculosis. However, the history of tick bite and failure to isolate mycobacteria from different tissue specimens prompted screening for a tick-borne disease, finally leading to a diagnosis of tularaemia. We discuss the possible relationship between immunosuppression and the clinical course of this rare infection.

Hidradenitis suppurativa.

Hidradenitis suppurativa is a chronic disease characterized by recurrent, painful, deep-seated, rounded nodules and abscesses of apocrine gland-bearing skin. Subsequent suppuration, sinus tracts and hypertrophic scarring are its main features. Onset is usually after puberty, although it is most common during the third decade and may persist in old age. The disease tends to be chronic and may develop to subcutaneous extension leading to indurations, sinus, and fistula having a profound impact on the quality of life. The prevalence is 1% in several studies. Axillary and inguinal involvement is more common in females; peri-anal and buttocks localizations are prevalent in males. The exact aetiology remains unknown. The primary event is a follicular occlusion with secondary inflammation, infection and destruction of the pilo-sebaceo-apocrine apparatus and extension to the adjacent sub-cutaneous tissue. Infection is common. Smoking may be a triggering factor. Obesity aggravates the discomfort. Differential diagnostic includes Crohn's disease, nodular acne and furonculosis. The main complications are arthropathy, carcinoma. Treatment depends upon the stage of the disease. Early nodular lesions may be treated by antibiotics for acute stage; long-term antibiotics, zinc salts may be useful as maintenance treatment; anti-TNF drugs have been used in severe cases; systemic steroids, estrogens, anti-androgens, retinoids have been used as options with limited success. Surgical treatment includes incision with or without drainage for limited abscesses; limited excisions are used for locally recurring draining sinuses. Total wide excision and healing with secondary intention or flaps and grafts is the only curative procedure in case of advanced disease.

Psoriasis in France and associated risk factors: results of a case-control study based on a large community survey.

BACKGROUND: Risk factors for psoriasis have been identified. OBJECTIVE: To precisely define these associated factors. METHODS: A survey was conducted using a questionnaire on a representative sample of the French population. A case-control study was conducted. Cases were persons who declared having had psoriasis during the previous 12 months. For each case, 3 matched controls were selected. Cases and controls were compared using univariate and multivariate analyses. RESULTS: The questionnaire was filled out and returned by 6,887 (68.9%) of 10,000 subjects aged 15 years and over; 356 cases were identified. In multivariate analysis, a higher body mass index, current and former smoking habits and beta-blocker intake were independently associated with a higher risk of psoriasis; intake of statins was associated with a decreased risk (p < 0.05). CONCLUSIONS: We confirmed the association of overweight, smoking habits and beta-blocker intake with psoriasis and reported a decreased risk associated with statin intake.

[Wells' cellulitis and bacterial necrotizing cellulitis induced by anakinra]. / Cellulite de Wells et dermohypodermite bactérienne nécrosante induites par l'anakinra.

BACKGROUND: Anakinra is a recombinant form of the naturally occurring human interleukin-1 receptor antagonist. It is used in the treatment of rheumatoid arthritis. The most frequent side effects are injection site reactions, which seem to have a toxic mechanism. PATIENTS AND METHODS: We report two unusual cases of injection site reactions with anakinra: a woman presented Wells' cellulitis of the thigh and a man developed serious bacterial cellulitis distinguished by deep necrosis at the site of the latest anakinra subcutaneous injection. DISCUSSION: The cases are examples of serious side effects that can occur during treatment with anakinra and underline the need for careful use of this new biological agent.

What causes hidradenitis suppurativa?

Hidradenitis suppurativa (HS)--a rather common, very chronic and debilitating inflammatory skin appendage disorder with a notoriously underestimated burden of disease--has long been a playground for the high priests of nomenclature: Ask a bunch of eminent dermatologists and skin pathologists to publicly share their thoughts on what causes HS, and they will soon get entrenched in a heated debate on whether this historical term is a despicable misnomer. Fortunately, the recently founded Hidradenitis Suppurativa Foundation (HSF; http://www.hs-foundation.org), to which EXP DERMATOL serves as home journal, has broken with this unproductive tradition and has encouraged publication of the current CONTROVERSIES feature. This is exclusively devoted to discussing the pathobiology of this chronic neutrophilic folliculitis of unknown origin. Although traces of terminological bickering remain visible, it does the HS experts in our virtual debate room credit that they engage in a constructive and comprehensive dissection of potential pathogenesis pathways that may culminate in the clinical picture we know under the competing terms HS or acne inversa. These experts sketch more often complementary than mutually exclusive pathogenesis scenarios, and the outlines of a conceivable consensus on the many open pathobiology questions begin to emerge in these CONTROVERSIES. Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy.

[Lymphedema as a complication of hidradenitis suppurativa in three patients]. / Lymphoedème compliquant une hidradénite suppurée: trois observations.

BACKGROUND: We report three patients (respectively 36, 52 and 52 years old) with a long history of hidradenitis suppurativa associated with pubic lymph oedema. CASE REPORT: Dramatic pubic oedema was noted, with "rugby-ball and bag-like" tumefaction associated with nodules and fistula. There was no evidence of lymph node involvement or abdominal lymphatic blockade. One patient underwent surgical excision combined with split-thickness graft and complete healing ensued. Pathological examination of the excised tissue showed telangiectasia and fibrosis. DISCUSSION: Chronic lymphedema affects patients with severe and long-lasting disease. The complication is caused by chronic and recurrent inflammation due to HS with subsequent blockade or destruction of local lymph drainage routes. Once this complication appears, no medical treatment is effective.

[Severe necrotic and haemorrhagic edema of the upper limbs revealing remitting seronegative symmetrical synovitis with pitting edema]. / Rhumatisme inflammatoire de type RS3PE révélé par un oedème nécrotique et hémorragique grave des membres supérieurs.

BACKGROUND: RS3PE (Remitting Seronegative Symmetrical Synovitis with Pitting Edema) syndrome is characterized by bilateral and symmetrical tenosynovitis of the distal extremities. It occurs with acute onset in older patients aged over 50 years. This heterogeneous entity can be isolated or can reveal various rheumatic diseases and neoplastic conditions. We report a case of RS3PE syndrome associated with unusually severe cutaneous necrotic and haemorrhagic lesions, and revealing malignant monoclonal IgM proliferation. CASE REPORT: A 62-year-old man was admitted for acute and symmetrical synovitis of both hands and forearms associated with fever and increased acute phase reactants. Severe necrotic and haemorrhagic edema developed simultaneously. Laboratory tests ruled out infectious disease and collagen vascular disorder. Clinical symptoms responded promptly to corticosteroids to reveal severe erosive arthropathy of both wrists. The monoclonal IgM proliferation discovered during the acute phase evolved into a malignant medullary plasmocytosis. After adequate treatment of this proliferation, no haematological, cutaneous or articular relapse occurred during the two-year follow-up period. DISCUSSION: This type of cutaneous symptoms has never been reported during the course of RS3PE syndrome. It may be supposed that the severity of the initial clinical picture was linked in this patient to the paraneoplastic nature of this.

[Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma with aggressive course]. / Lymphome T cutané épidermotrope CD8+ cytotoxique d'évolution très agressive.

BACKGROUND: Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the literature. Below we report a case involving unusual association of cutaneous vasculitis and lymphoproliferation. CASE REPORT: A 42-year-old senegalese man was hospitalized for cutaneous nodular lesions, which rapidly spread and became necrotic and ulcerated. he had recent weight loss with fever and multiple enlarged lymph nodes. Cutaneous histological analysis showed epidermotropic dermal infiltrate comprising medium and large cd8+ cytotoxic t-cells of unusual angiocentricity with cutaneous vasculitis and fibrinoid necrosis. the patient died 4 months after initiation of treatment with multi-agent chemotherapy. DISCUSSION: This patient presented the characteristics of primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma described by Berti. The clinical findings in most cases consist of nodular and ulcerative cutaneous lesions. Histologically, the cutaneous infiltrate is composed of pleomorphic lymphocytes with marked and constant epidermotropism. Immunohistochemistry shows lymphocytes expressing a CD8+ phenotype and cytotoxic proteins, which probably accounts for the local and systemic aggressiveness of the disease, as well as the angiodestructive nature of the infiltrate and the necrotic lesions.

Association between benign and malignant peripheral nerve sheath tumors in NF1.

OBJECTIVE: People with neurofibromatosis type 1 (NF1) have a 10% lifetime risk of developing a malignant peripheral nerve sheath tumor (MPNST). MPNSTs are often metastatic and are a frequent cause of death among people with NF1. Clinical evidence suggests that most MPNSTs in people with NF1 develop from preexisting plexiform neurofibromas. However, it is not known whether an individual's risk of developing an MPNST is associated with the burden of benign neurofibromas. The authors conducted a study to determine whether people with NF1 who have benign neurofibromas of various kinds are at greater risk of developing MPNSTs than patients with NF1 who lack these benign tumors. METHODS: Clinical information on 476 NF1 probands in the Henri Mondor Database was analyzed by logistic regression to examine associations between MPNSTs and internal plexiform, superficial plexiform, subcutaneous, and cutaneous neurofibromas. RESULTS: Individuals with subcutaneous neurofibromas were approximately three times more likely to have internal plexiform neurofibromas or MPNSTs than individuals without subcutaneous neurofibromas. Individuals with internal plexiform neurofibromas were 20 times more likely to have MPNSTs than individuals without internal plexiform neurofibromas. When this analysis was done with both subcutaneous and internal plexiform neurofibromas as explanatory variables, only the association of MPNSTs with internal plexiform neurofibromas remained significant. CONCLUSIONS: The observation that malignant peripheral nerve sheath tumors are strongly associated with internal plexiform neurofibromas suggests that patients with neurofibromatosis type 1 with these benign tumors warrant increased surveillance for malignancy.