Acne Conglobata Research Articles

Good Response of Acne Conglobata to Adalimumab in Two Patients with Hidradenitis Suppurativa

Good Response of Acne Conglobata to Adalimumab in Two Patients with Hidradenitis Suppurativa

Efficiency and tolerance of acne therapy using system isotretinoin

Purpose. Determination of efficiency and tolerance of Sotret product in treatment of patients with different clinical forms and severe acne in three clinical investigations. Materials and methods. 103 patients with acne (moderate and severe acne, acne conglobata and acne inversa) were examined and treated. All patients underwent monotherapy with Sotret with clinical effectiveness assessment and records of adverse events. Results. Sotret therapy facilitated clinical remission of acne in 90-100% of cases. Adverse events were easily tolerated and were not serious. Conclusion. Established therapeutic efficiency of Sotret determines its potential in treating various severity and various forms of acne.

Acne Conglobata Associated with Hidradenitis Suppurativa, Disorders of Follicular Occlusion (Case Report)

Abstract The author is presenting the case of a 23-year-old female patient with a severe form of acne conglobata, with the first symptoms of the disease occurring as far back as the prepubertal age. In the past year the disease has combined with hidradenitis suppurativa (to be referred to henceforth as “HS”), Hurley stage I, in the axillae and both sides of the inguinal region, with a family history of acne conglobata (both her mother and brother were affected). Further examinations ruled out inflammatory bowel disease because of a lack of further associated symptoms, except for sideropenic anaemia (lesser form) and lower serum values of vitamin D. Up until now the disease has been resistant to treatment, including the long-term treatment of methylprednisolone in combination with isotretinoid as well as dapsone and antibiotics.

Dapsone Gel for Papulopustular Rosacea

Dapsone gel is used to treat acne. Oral dapsone has been used off-label to treat acne conglobata and rosacea fulminans. It seems reasonable that

Off-label use of TNF-alpha inhibitors in a dermatological university department: retrospective evaluation of 118 patients.

Tumor necrosis factor-alpha (TNF)-alpha inhibitors are licensed for patients with severe refractory psoriasis and psoriatic arthritis. However, TNF-alpha inhibitors have also been used off-label for various recalcitrant mucocutaneous diseases. This study aimed to evaluate the efficacy and safety of TNF-alpha inhibitors used for off-label dermatological indications. We retrospectively evaluated patient records of 118 patients treated off-label with TNF-alpha inhibitors in a dermatological university department. Patients presented with severe aphthous stomatitis/genital aphthous lesions (26), chronic urticaria (25), hidradenitis suppurativa (29), acne conglobata (11), dissecting cellulitis of the scalp (two), orofacial granulomatosis (four), sarcoidosis (four), granuloma annulare (two), granulomatous rosacea (one), granuloma faciale (one), subcorneal pustulosis (one), pyoderma gangrenosum (four), Sweet's syndrome (four), Well's syndrome (one), benign familial pemphigus (one), lichen planus (one), and folliculitis decalvans (one). A significant number of these patients went into remission during therapy with TNF-alpha inhibitors. A total of 11 patients (9%) experienced severe adverse effects during therapy. Off-label therapy with TNF-alpha inhibitors may be considered for selected patients with severe recalcitrant mucocutaneous diseases. The risk of severe adverse effects signals that a thorough benefit-risk assessment should be performed before initiating off-label treatment with TNF-alpha inhibitors for these conditions.

Maladie de Verneuil : aux confins des spondyloarthrites

Hidradenitis suppurativa (HS), or acne inversa, is a chronic inflammatory skin disease, involving skin annexes, located at the axillary, inguinal and perineal sites. It can be associated with acne conglobata and neutrophilic dermatosis, as well as with Crohn's disease. HS should be added to the list of skin diseases associated with spondyloarthritis (SpA). After case reports and short case series, a prospective multicenter national French survey has provided evidence for a 10 times higher prevalence of SpA in HS compared to the general population, namely 3.7% and 0.3%, respectively. Based on this prospective study encompassing 43 SpA out of 640 HS patients, clinical settings are responding to ESSG, Amor and ASAS criteria. In 90% of cases, HS precedes SpA onset, by 3.6 years as a mean. Musculoskeletal involvement includes inflammatory back and buttock pain (80%), enthesitis mainly at the Achilles tendon (46%), peripheral arthritis (25%), and dactylitis (9%). Anterior thoracic pain is present in more than 30% of patients with HS. SAPHO has been described in more than 15 cases in the literature. Besides tobacco and obesity as risk factors in both diseases, common pathogenic mechanism can be postulated with respect to innate immunity and inflammatory cytokine pathways.

Serum levels of hypersensitive-C-reactive protein in moderate and severe acne.

Background:Elevation of C-reactive protein (CRP) has been reported to occur in psoriasis, urticaria, acne, rosacea and many other dermatological and nondermatological conditions. Chronic systemic inflammation has been implicated in the development of neuropsychiatric/degenerative disorders, atherosclerosis, coronary artery disease, diabetes mellitus and even carcinogenesis. The present study is designed to determine whether the level of inflammation created by acne vulgaris could be high enough to raise the serum levels of high-sensitive CRP.Materials and Methods:Forty-two patients with moderate and severe acne vulgaris were enrolled, along with 44 age and sex matched healthy blood donors as controls. Hypersensitive-CRP (Hs-CRP) was measured in both groups.Results:Hypersensitive-C-reactive protein levels in the case group varied between 0 and 28.1 μg/ml with an average of 2.24 ± 4.87 μg/ml (mean ± standard deviation) and a median of 0.6 μg/ml (interquartile range [IQR] =0.3, 1.4 μg/ml). Hs-CRP levels of the control group varied between 0 and 14 μg/ml with an average of 3.12 ± 3.67 μg/ml and a median of 1.5 μg/ml (IQR = 0.55, 5.0 μg/ml). No significant difference of Hs-CRP level between the two groups was seen (t = –0.961, 95% confidence interval: Lower = –2.6942, upper = 0.9377; P = 0.339). Additionally, no significant difference in the level of Hs-CRP was noted between the moderate and severe acne groups (95% confidence interval: Lower = –5.2495, upper = 1.6711; P = 0.165).Conclusion:Acne vulgaris, even in its severe grades (excluding acne fulminans and acne conglobata), does not induce significant inflammation at the systemic level.

Acne conglobata and adalimumab: use of tumour necrosis factor-α antagonists in treatment-resistant acne conglobata, and review of the literature

Acne conglobata (AC) is a chronic, severe, inflammatory variant of acne characterized by development of cystic nodules, abscesses and sinus tracts. AC may prove resistant to conventional acne therapy. The off-label use of adalimumab for the treatment of AC has been reported recently. We present a 26-year-old man with AC resistant to conventional treatment, who was treated with 40 mg adalimumab every other week, with significant clinical improvement. We review the evidence for the use of tumour necrosis factor antagonists in AC and related conditions. This case provides further evidence supporting the role of adalimumab in the treatment of AC.

The SAPHO syndrome revisited with an emphasis on spinal manifestations.

The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis.

Palmoplantar pustules and osteoarticular pain in a 42-year-old woman

Key teaching points • Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is characterized by distinctive osteoarticular manifestations and a spectrum of neutrophilic dermatoses. • The most common dermatologic manifestations include palmoplantar pustulosis, acne conglobata, and acne fulminans. • SAPHO syndrome should be considered in patients presenting osteoarticular pain, particularly involving the anterior chest wall and/or spine, and neutrophilic skin lesions.

SAPHO Syndrome in an Adolescent: A Clinical Case With Unusual Severe Systemic Impact

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes both dermatological and rheumatologic symptoms. Being a rare condition, the diagnosis is frequently late. The authors report a case of a 13-year-old boy diagnosed with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome with unusual severe systemic repercussions. The patient presented with acne conglobata, inability to walk due to pain and weakness and weight loss. Bone scintigraphy was suggestive of sacroiliitis, and lumbar spine x-ray showed signs of hyperostosis. His clinical state improved after treatment with nonsteroidal anti-inflammatory drugs, methotrexate, clindamycin, and isotretinoin. A review of the clinical aspects of this syndrome is presented, emphasizing how this underdiagnosed syndrome can lead to severe weight loss and significant functional and psychological impairment at an early age.

G49 Acne fulminans induced by oral isotretinoin

A 13 year-old male presented with a one year history of acne vulgaris, unresponsive to six months of lymecycline, with comedones, pustules and a few small scarring cysts of his...

An atlas of the morphological manifestations of hidradenitis suppurativa

This article is dermatological atlas of the morphologic presentations of Hidradenitis Suppurativa (HS). It includes: superficial abscesses (boils, furnucles, carbuncles), abscesses that are subcutaneous and suprafascial, pyogenic granulomas, cysts, painful erythematous papules and plaques, folliculitis, open ulcerations, chronic sinuses, fistulas, sinus tracts, scrotal and genital lyphedema, dermal contractures, keloids (some that are still pitted with follicular ostia), scarring, skin tags, fibrosis, anal fissures, fistulas (i.e. circinate, linear, arcuate), scarring folliculitis of the buttocks (from mild to cigarette-like scarring), condyloma like lesions in intertrigous areas, fishmouth scars, acne inversa, honey-comb scarring, cribiform scarring, tombstone comedones, and morphia-like plaques. HS can co-exist with other follicular diseases such as pilonidal cysts, dissecting cellulitis, acne conglobata, pyoderma gangrenosum, and acanthosis nigricans. In sum, the variety of presentations of HS as shown by these images supports the supposition that HS is a reaction pattern.HS is a follicular based diseased and its manifestations involve a multitude of follicular pathologies [1,2]. It is also known as acne inversa (AI) because of one manifestation that involves the formation of open comedones on areas besides the face. It is as yet unclear why HS is so protean in its manifestations. HS severity is assessed using the Hurley Staging System (Table 1). It also remains unclear why hidradentitis may remain limited to Hurley Stage 1, evolve to the more confluent (Hurley Stage 2), or progress even further to the fully confluent (Hurley Stage 3).In addition, HS can be associated with other follicular based diseases such as pilonidal cysts (PCs) of the sacrum and buttocks, dissecting cellulitis (DC), and acne conglobata (AC), which usually involves the face, chest, When HS occurs with PCs, DC, and/or AC it is referred to as the follicular occlusion triad or tetrad [2]. HS can more rarely be associated with pyoderma gagrenosum (PG) or Crohn disease (CD), other inflammatory diseases of the skin that are not follicular. The reason for this is unclear [2]. What AC, DC, HS, CD, and PG share is that they occur in bacterially rich environments. HS probably occurs with acanthosis nigricans because many HS patients are obese [2]. This concurrence seems under reported.

A case of a patient with stage III familial hidradenitis suppurativa treated with 3 courses of infliximab and died of metastatic squamous cell carcinoma.

Although rare, severe hidradenitis suppurativa (HS) of the anal, perianal, gluteal, thigh, and groin regions can evolve into squamous cell carcinoma (SCC). This usually does not occur until the HS has been present for more than 20 years. Malignant degeneration of HS in the axilla has not been reported. SCC has developed in dissecting cellulitis, acne conglobata, and pilonidal cysts (other members of the follicular tetrad). Whereas the male to female ratio of HS is 1:3, SCC in HS has a male to female ration of 5:1. The reasons behind malignant degeneration in HS are complex and might differ from the malignant degeneration causing Marjolin ulcers. It likely involves the presence of human papilloma virus (HPV) in affected areas (a rarity in the axilla), and impaired defensins, which combat HPV, in the skin of Hurley Stage III HS. In familial HS, the odds of developing SCC are likely greater because of independent loss-of-function mutations in the γ-secretase multiprotein complex, which regulates the Notch signaling pathway. Compromise of the Notch signaling pathway can undermine immune function and increase the risk of neoplastic development. Coincident SCC with use of tumor necrosis factor α blockers has been reported. I report a patient with long standing Hurley Stage III, familial HS, wwho developed metastatic SCC after 3 courses of infliximab and expired 11 months after the infliximab was started. A 47-year-old male presented with progressive HS since early adulthood. His stage III hidradenitis suppurativa (HS) involved his groin, legs buttocks, and perineal areas. Interestingly, his HS was familial; one daughter also suffered from HS. A pilonidal cyst had been excised in the past. He suffered from hypertension for which he took ramipril, 2.5 mg per day. He did not admit to smoking. He had undergone numerous surgeries and courses of clindamycin with rifampin and clindamycin with minocycline. He used pregablin among other stronger medications for pain control. He had also taken isotretinoin years before without substantial long-term benefit. The various treatments were palliative but the HS always returned. He expressed that the pain from his HS was not bearable. He decided in consultations with his doctors to try infliximab owing to the positive clinical data for its efficacy in HS. He took 3 courses of infliximab 500mg, each of which was followed by surgical debridement of the perineal and anal areas. At the 3rd surgical debridement his physician noted the presence of squamous cell carcinoma (SCC) on July 28, 2008. The infliximab was stopped. However, the patient developed the patient underwent scanning soon after that showed soon after that the SCC had metastasized. He expired in June of 2009.

Impact of TNF Haplotypes in the Physical Course of Acne Vulgaris

Background: The role of single-nucleotide polymorphisms (SNPs) of the TNF gene in acne vulgaris remains controversial. Methods: Genomic DNA was isolated from 185 patients with acne vulgaris and 165 healthy controls. SNPs at positions -376, -308 and -238 of the promoter region of TNF were defined. Results: The frequency of the GAG haplotype was greater among patients (16.8%) than among controls with borderline significance (9.7%, p = 0.059). Male carriers of haplotypes other than GGG presented acne vulgaris at a later age than carriers of the GGG haplotype. No effect of the GAG haplotype on the frequency of acne conglobata was found among women with polycystic ovary syndrome. Conclusions: Carriage of the GAG haplotype of TNF is linked with borderline susceptibility to acne vulgaris. The GGG haplotype is related with earlier disease onset in male patients.

Adalimumab for the Treatment of Refractory Acne Conglobata

Tumor necrosis factor (TNF) inhibitors have become important components of the treatment of multiple chronic inflammatory disorders such as psoriasis, psoriatic arthritis, rheumatoid arthritis, ankylosing spondylitis, juvenile idiopathic arthritis, Crohn disease, and ulcerative colitis. Furthermore, off-label use of TNF inhibitors has been reported for a variety of other diseases. We report a case of refractory acne conglobata treated with the TNF inhibitor adalimumab.

SAPHO syndrome associated with acne conglobata successfully treated with etanercept

A 42-year-old Taiwanese male presented to our dermatology department with complaint of chronic acne on the face, back and buttocks with severe sinus tracts and depressed scar formation for 20 years (Fig. 1A). Long-term oral minocycline provided some relief, but new crops of lesions continued to develop periodically. He started to suffer from intermittent chest pain and palpitation during the past 5 years. Repeated 24-hour-electrocardiography monitoring and echocardiography were unremarkable. In addition, episodic polyarthralgia over the bilateral temporomandibular joint, left shoulder, and left ankle were noted in recent years. Laboratory studies revealed a mild increase in the erythrocyte sedimentation rate (11 mm, normal: <10 mm) and mild decreases in immunoglobulin G (656 mg/dL, normal: 751e1560 mg/dL), immunoglobulin M (44.4 mg/dL, normal: 46e304 mg/dL), and complement 3 (72.7 mg/dL, normal: 79e152 mg/dL). X-ray of the chest, left ankle, and skull, as well as computed tomography of the head and anterior chest wall were all unremarkable. Bone scintigraphy showed uptake in the left parietal skull,

Mutations in SH3PXD2B cause Borrone dermato-cardio-skeletal syndrome

Borrone Dermato-Cardio-Skeletal (BDCS) syndrome is a severe progressive autosomal recessive disorder characterized by coarse facies, thick skin, acne conglobata, dysmorphic facies, vertebral abnormalities and mitral valve prolapse. We identified a consanguineous kindred with a child clinically diagnosed with BDCS. Linkage analysis of this family (BDCS1) identified five regions homozygous by descent with a maximum LOD score of 1.75. Linkage analysis of the family that originally defined BDCS (BDCS3) identified an overlapping linkage peak at chromosome 5q35.1. Sequence analysis identified two different homozygous mutations in BDCS1 and BDCS3, affecting the gene encoding the protein SH3 and PX domains 2B (SH3PXD2B), which localizes to 5q35.1. Western blot analysis of patient fibroblasts derived from affected individuals in both families demonstrated complete loss of SH3PXD2B. Homozygosity mapping and sequence analysis in a second published BDCS family (BDCS2) excluded SH3PXD2B. SH3PXD2B is required for the formation of functional podosomes, and loss-of-function mutations in SH3PXD2B have recently been shown to underlie 7 of 13 families with Frank-Ter Haar syndrome (FTHS). FTHS and BDCS share some overlapping clinical features; therefore, our results demonstrate that a proportion of BDCS and FTHS cases are allelic. Mutations in other gene(s) functioning in podosome formation and regulation are likely to underlie the SH3PXD2B-mutation-negative BDSC/FTHS patients.

Short‐term clinical effects of photodynamic therapy with topical 5‐aminolevulinic acid for facial acne conglobata: an open, prospective, parallel‐arm trial

Acne conglobata is hardly curable and easily leads to scar formation after treatment using traditional methods. To develop a novel way to treat acne conglobata. Seventy-five patients with facial acne conglobata were included in this clinical study and divided into either a treatment group (n = 35) to receive photodynamic therapy (PDT) with topical 5% 5-aminolevulinic acid and red light once every 10 days for a month or a control group (n = 40) to receive a Chinese herbal medicine mask plus red light once per week for the same duration. Patients in both groups were given oral viaminate capsules, doxycycline, zinc gluconate, and topical metronidazole. Efficacy was evaluated with respect to symptom score, cure rate, and response rate up to 2 weeks following the final treatment, and time points for assessment included baseline (D0 ), the visit before each treatment (D10 and D20 for the treatment group, and D7 , D14 , and D21 for the control group), and 2 weeks after treatment (D34 for the treatment group and D35 for the control group). Safety was assessed by recording adverse effects. Treatment with PDT significantly improved acne lesions and reduced scar formation. The treatment group had a significantly lower symptom score, a higher cure rate, and response rate than the control group. No systemic side effects occurred. The treatment of acne conglobata with PDT is associated with a high cure rate, short treatment period, few side effects, and reduced scar formation. To the best of our knowledge, this is the first report on the treatment of acne conglobata with PDT.

Spondyloarthritis Associated with Acne Conglobata, Hidradenitis Suppurativa and Dissecting Cellulitis of the Scalp: A Review with Illustrative Cases

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.