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Hidradenitis suppurativa (HS) is an inflammatory disorder that is characterized by chronic deep-seated nodules, abscesses, fistulae, sinus tracts, and scars in the axilla, inguinal area, submammary folds, and perianal area. This disfiguring condition is accompanied by pain, embarrassment, and a significantly decreased quality of life. Although the mechanism of HS has not been entirely elucidated, lesion formation is believed to center around follicular hyperkeratosis within the pilosebaceous–apocrine unit. Recent research has provided new insight into the role of cytokines in the pathogenesis of HS, helping close some existing knowledge gaps in the development of this condition. The first article in this continuing medical education series reviews HS epidemiology, clinical presentation, and classification. We also provide an update on the most recent understanding of HS pathogenesis, including the central role of inflammatory cytokines and other contributing factors, such as genetics, hormones, and pathogenic microorganisms.

Background The permanent disfigurement associated with hidradenitis suppurativa (HS) necessitates early aggressive disease intervention. Although limited data support the use of infliximab (IFX) in HS, the efficacy of high-dose, high-frequency IFX has yet to be defined. Objective To evaluate the efficacy of IFX 7.5 to 10 mg/kg, with a maintenance frequency every 4 weeks. Methods Prospective analysis of 42 patients initiating IFX 7.5 mg/kg every 4 weeks (IFX 7.5) and 16 patients receiving dose escalation to IFX 10 mg/kg every 4 weeks (IFX 10) between March 1, 2018, and February 28, 2019. The primary outcome measure (clinical response) was the proportion of patients with Physician Global Assessment of clear, minimal, or mild (score of 0-2) HS with at least a 2-grade improvement from baseline scores. Results The proportion of patients achieving a clinical response after initiating IFX 7.5 was 20 of 42 (47.6%) at week 4 and 17 of 24 (70.8%) at week 12. For patients receiving dose escalation to IFX 10 because of incomplete initial response, 6 of 16 (37.5%) achieved clinical response at week 4 and 6 of 12 (50%) at week 12. Conclusions Initiation of IFX 7.5 every 4 weeks, with possible dose escalation to IFX 10, if needed, provides optimal mitigation of HS-related disease activity.

Background Few data exist to guide infliximab dosing in hidradenitis suppurativa (HS). Objectives To determine optimal infliximab dosing for patients with HS based on treatment response and achievement of stable dosing. Methods Retrospective cohort study of 52 patients with HS treated with infliximab. Primary outcome was achievement of stable dosing regimen for at least 8 weeks. Secondary outcomes were time to discontinuation, time to titration, changes to inflammatory markers, and clinical response. Results Thirty-five patients (67%) achieved stable dosing, most at a schedule of 10 mg/kg every 6 or 8 weeks. At 1 year from initiation, most patients (64%) required dose escalation. Patients tolerated infliximab well and achieved significant improvements in abscess and nodule count, draining sinuses, and erythrocyte sedimentation rate. Limitations Retrospective nature and lack of HS clinical response data for many patients. Conclusions Infliximab 10 mg/kg every 6 or 8 weeks may be a reasonable starting dosage for most patients.

Background Fistulous tracts in hidradenitis suppurativa (HS) are key signs of severity and their clinical evaluation alone may be limited for assessing their presence and morphology. There is also a need to determine the factors that allow reversibility of the anatomic changes in HS. Objective We sought to categorize fistulous tracts in HS. Methods A retrospective study of color Doppler ultrasound images of cases with positive clinical and sonographic criteria of HS with fistulous tracts was performed. The sonographic staging of HS, location, and anatomic characteristics of the tracts were registered and graded. Statistical analysis for correlating variables was performed using bivariate and multivariate studies. Results In all, 52 patients presenting 96 fistulous tracts met the criteria. Morphology was defined and a sonographic classification into 3 types of fistulae was developed. Type 3 concentrated 71% of the cases presenting communicating tracts, and type 2, 29%. Types 2 and 3 represented 63% of patients with multiple fistulous tracts. Fistulous tracts types 2 and 3 were significantly correlated with age 35 years or older and groin location. Limitations Ultrasound cannot detect lesions less than 0.1 mm. Conclusion Fistulous tracts in HS can be categorized using ultrasound, which may support earlier and more precise management.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The first article in this continuing medical education series explores the pathogenesis of neutrophilic dermatoses and reviews the epidemiology, clinical and histopathologic features, diagnosis, and management of Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.

Background Hand, foot, and mouth disease is a contagious viral infection usually affecting children. A resurgence of cases in adults, mainly caused by coxsackievirus A6 and with an atypical and more severe presentation, has taken place. Objective The goal was to examine the clinical, histologic, and immunohistochemical features of this disease in adults. Methods This is a retrospective study on documented cases of adult hand, foot, and mouth disease from France's Dermatology Department of Strasbourg University Hospital and Bel-Air Hospital in Thionville. Results Six patients with severe and atypical presentation were included, 4 caused by coxsackievirus A6. The histologic features were: spongiosis, neutrophilic exocytosis, massive keratinocyte necrosis, shadow cells in the upper epidermis, vacuolization of basal cells, necrotic cells in follicles and sweat glands, dense superficial dermal infiltrate of CD3+ lymphocytes, and strong granulysin expression. Limitations This is a retrospective case series. Conclusion In adult patients presenting with atypical hand, foot, and mouth disease caused by coxsackievirus A6, biopsy specimens show distinctive changes in the epidermis but also in adnexal structures. The inflammatory infiltrate is made of T cells with a cytotoxic profile, with numerous granulysin-positive cells, as observed in severe drug-induced eruption with necrosis of keratinocytes.

Background Medical therapies for hidradenitis suppurativa (HS) are often ineffective. Tumor necrosis factor-α inhibitors may be a potential treatment for patients with moderate to severe HS. Objectives We sought to evaluate the safety and efficacy of etanercept for patients with severe HS. Methods We conducted a phase II clinical trial of etanercept (50 mg/wk subcutaneously) in patients with moderate to severe HS. Efficacy was measured using a Physician Global Assessment and several secondary physician- and patient-reported outcome measures. Responders were classified as those achieving at least a 50% reduction on the Physician Global Assessment score at week 12 compared with baseline. Results Only 3 of the 15 patients who entered the study were classified as responders (response rate of 20%; 95% confidence interval: 4.3-48.1) based on the intention-to-treat analysis. Dermatology Life Quality Index scores improved slightly from a median of 19 to 15 ( P = .02). Comparison of baseline with week-12 Physician Global Assessment scores, and secondary outcome measures of lesion counts and patient pain scores, failed to show statistically significant improvement. Etanercept was generally well tolerated; however, two patients discontinued the study as a result of skin infections at the site of hidradenitis lesions requiring oral antibiotics. Limitations Lack of a control group and a small number of participants are limitations. Conclusions Our study demonstrated minimal evidence of clinically significant efficacy of etanercept (50 mg/wk subcutaneously) in the treatment of hidradenitis. Future studies using higher doses of etanercept are indicated; however, patients need to be carefully monitored for infection and other adverse events. Randomized, controlled trials will be necessary to demonstrate the risk-to-benefit ratio of tumor necrosis factor-α inhibitors in the treatment of hidradenitis.

Neutrophilic eccrine hidradenitis typically manifests as erythematous plaques on the face, trunk, or extremities. This eruption has been associated with numerous factors, but most commonly is seen with chemotherapy, particularly cytarabine. We report a 73-year-old woman with acute myelogenous leukemia who developed rapidly expansive neutrophilic eccrine hidradenitis mimicking facial cellulitis only after a course of cytarabine was followed by granulocyte-colony stimulating factor. Prompt diagnosis is imperative to prevent prolonged antimicrobial therapy.

Background Neutrophilic eccrine hidradenitis (NEH) is occasionally reported in patients who have not received chemotherapy. Objective The purpose of this study was to describe the clinicopathologic features of NEH occurring in healthy children and to investigate the interleukin (IL)-8 expression in the cutaneous lesions. Methods Ten children with characteristic histologic features of NEH were collected from the Chang Gung Memorial Hospital. Their formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for IL-8. Results The age of first presentation at our clinic ranged from 6 months to 14 months with a median age of 9.1 months. The onset of the disease clustered in the summertime. The most common clinical appearance was multiple erythematous papules and nodules on the limbs. Two of 7 biopsy specimens grew coagulase-negative Staphylococcus . None of patients had underlying systemic disease and all had complete resolution of the lesions within 3 weeks. Immunohistochemical staining for IL-8 was negative in the 10 cases studied. Conclusion Childhood NEH appears as urticaria-like erythematous nodules and plaques on the limbs, trunk, or scalp. This benign and limited disease occurs with a predilection for summer months. In our study, onset was in children less than 15 months of age. IL-8 was not detected in the cutaneous lesions.

Background Hidradenitis suppurativa (HS) is a chronic inflammatory disease involving intertriginous skin. Previous epidemiologic studies have been limited by small sample size. Objective We sought to describe the prevalence and comorbidities of HS in a large patient care database. Methods In this retrospective case-control study, we chart-validated all patients within a hospital database who received at least 1 billing code for HS between 1980 and 2013. Verified cases were matched with controls based on age, gender, and race. Prevalences of a priori selected comorbidities were compared between HS and control groups. Results A total of 2292 patients at Massachusetts General Hospital received at least 1 code for HS. A total of 1776 cases had a validated diagnosis of HS, yielding a prevalence of 0.08%. In unadjusted analysis, all comorbidities were diagnosed significantly more in HS compared with control including (in rank order of likelihood): smoking, arthropathies, dyslipidemia, polycystic ovarian syndrome, psychiatric disorders, obesity, drug dependence, hypertension, diabetes, thyroid disease, alcohol dependence, and lymphoma (all P Limitations Control subjects were not validated for absence of HS and comorbidity validation was not performed for either group. Conclusions Our results highlights the high comorbidity burden of patients with HS compared with matched control subjects.

A 15-year-old boy experienced a macular serpentine erythematous eruption that subsided with a persistent hyperpigmentation overlying the superficial venous network of the left forearm. This reaction occurred at the injection site of a chemotherapy regimen that combined actinomycin and vincristine a few hours after the first course. After a single injection of actinomycin in the right arm, a similar reaction occurred, implicating it as the responsible agent. A skin biopsy specimen demonstrated a cell-poor interface tissue reaction associated with an eccrine neutrophilic hidradenitis. To our knowledge, this is the first case of persistent supravenous serpentine hyperpigmented eruption reported in a child treated with this particular drug combination.

Objective We sought to determine quality of life impairment in hidradenitis suppurativa. Methods Questionnaires were administered to 61 patients. Results Quality of life impact in hidradenitis was much greater than that of several other dermatologic conditions. Limitation This hospital-based population may not be representative. Conclusion Hidradenitis is one of the most distressing conditions observed in dermatology.

Background: Antibiotics are often used to treat hidradenitis, but only topical clindamycin has been shown to be effective in a randomized controlled trial. The paucity of these trials may be the result of difficulties in disease assessment. Objective: We compare topical clindamycin with systemic tetracycline in the treatment of hidradenitis suppurativa, and study clinical disease assessment. Methods: A total of 46 patients with stage 1 or 2 hidradenitis suppurativa were treated in a double-blind, double dummy controlled trial. Results: No significant difference was found between the two types of treatment. Patients' global assessment of disease was significantly worse than physician's assessment in 3 of 5 evaluations ( P = .0096 to .015), but the correlation between patients' and physicians' assessments was satisfactory after only one visit (rs = .761 to .895). Soreness was the key factor in patients' overall assessment of the disease. Conclusion: Systemic therapy with tetracyclines did not show better results than topical therapy with clindamycin. Subjective factors, particularly soreness, appear to be a key factor in patients' assessment of the disease and should, therefore, be included as an outcome variable in future therapy studies. (J Am Acad Dermatol 1998;39:971-4.)

Eccrine glands are uniquely susceptible to a variety of pathologic processes. Alteration in the rate of sweat secretion manifests as hypohidrosis and hyperhidrosis. Obstruction of the eccrine duct leads to miliaria. The excretion of drugs into eccrine sweat may be a contributory factor in neutrophilic eccrine hidradenitis (NEH), syringosquamous metaplasia (SSM), coma bulla, and erythema multiforme (EM). Alterations in the electrolyte composition of eccrine sweat can be observed in several systemic diseases, most notably cystic fibrosis. This article summarizes current knowledge of eccrine gland pathophysiology.

Hidradenitis suppurativa, also known as acne inversa, is a chronic, often debilitating disease primarily affecting the axillae, perineum, and inframammary regions. Prevalence rates of up to 4% have been estimated. Our understanding of the disease has changed over time. It is now considered a disease of follicular occlusion rather than an inflammatory or infectious process of the apocrine glands. Clinically, the disease often presents with tender subcutaneous nodules beginning around puberty. The nodules may spontaneously rupture or coalesce, forming painful, deep dermal abscesses. Eventually, fibrosis and the formation of extensive sinus tracts may result. The location of the lesions may lead to social embarrassment and the failure to seek medical treatment. Therapies in the past have consisted of long-term antibiotics, antiandrogens, and surgery. New treatments like tumor necrosis factor-alfa inhibitors have given clinicians more options against this difficult disease.

Neutrophilic eccrine hidradenitis is an uncommon, self-limited dermatosis with a variable clinical presentation. It seems to be due to chemotherapeutic drugs in most cases. Necrosis of the eccrine gland associated with a neutrophilic infiltrate is the histologic hallmark of this disease. We report two additional cases in neutropenic patients with acute myelogenous leukemia in which there was a striking lack of neutrophil infiltration. A new term, drug-associated eccrine hidradenitis, is suggested.

A 12-year-old boy had painful lesions on both soles. He had had a similar episode several months before that resolved spontaneously. Physical examination showed erythematous, extremely tender nodules on both plantar surfaces and the toes. The second and third left toes had small pustules on top of the nodules. There was no palmar involvement. The clinical features, pathologic findings, and self-limited course suggested recurrent palmoplantar hidradenitis. This distinctive entity of unknown origin appears during childhood and is characterized histologically by a neutrophilic infiltrate affecting the eccrine glands. We report the case of a patient with unusual clinical features. (J Am Acad Dermatol 2002;47:S263-5.)

Neutrophilic eccrine hidradenitis has been described in patients with acute myelogenous leukemia and other malignant diseases, usually during chemotherapy. We describe a 46-year-old man with Hodgkin's disease in whom neutrophilic eccrine hidradenitis developed after each of the first two treatments with lomustine. Dapsone, 100 mg daily, was initiated 48 hours before the patient's third treatment with lomustine and was continued for 14 days. This regimen was successful in suppressing the reaction during the first course and three subsequent courses of lomustine.

Background: Hidradenitis suppurativa is traditionally classified as a disease of the apocrine gland. However, different histologic descriptions exist. Objective: Our purpose was to describe prospectively the histopathologic characteristics of hidradenitis. Methods: We systematically described and classified 60 consecutive biopsy specimens from patients with hidradenitis and compared them with 33 specimens from clinically noninvolved regional controls. Results: A heterogeneous histologic picture was found. Apocrine glands were involved in a minority of the 60 specimens, 17 showed poral occlusion, 17 simple folliculitis without poral occlusion, 9 sinus tracts, 6 epithelial cyst, 5 abscess, 3 apocrinitis, 2 diffuse dermal inflammation, and 1 pyogenic granuloma and scarring. Secondary involvement of apocrine glands was found in 12% of all specimens, and secondary involvement of eccrine glands was found in 25%. Sinus tracts were found significantly more often in the presence of poral occlusion or epithelial cysts. Control specimens frequently revealed changes compatible with early stages of follicular involvement. Apocrine glands were observed significantly more often in the axillae than in the groin. Conclusion: The clinical picture of hidradenitis suppurativa covers a broad histologic spectrum. This may help explain the therapeutic problems posed by this disease. The disease appears to be predominantly follicular, and apocrine glands appear to be primarily involved in only a minority of axillary lesions.

Acneiform eruptions can be caused by contact with oils,' tars,2 waxes, and halogenated phenols.' We describe a patient with severe occupational acne caused by contact with diesel oil who had an excel­ lent response to isotretinoin. A 41-year-old man had a severe and persistent cutane­ ous eruption for 5 months on the face, back, and upper extremities. The patient was previously healthy, did not take any medications, and had never had acne before. He had begun working as a laborer 2 months before the ap­ pearance of the skin lesions. His work involved spraying diesel oil onto the walls of large molds for concrete pan­ els. He wore no protective clothing and his clothes became soaked in oil. Despite progression of his disease, he con­ tinued to work under the same conditions for 5 months. Examination revealed innumerable open and closed comedones, papules, pustules, nodules, and cysts, mainly on the back (Fig. I) but also on the chest, extremities, and face. Almost all follicles on his back and most follicles on the arms and malar areas contained a comedo, giving the skin a gray appearance. Fine follicular hyperkeratosis on the trunk and a slight brown diffuse hyperpigmentation of the face were also seen. There were bilateral inflamed cysts of the meihomian glands. In the axillae, small erythematous nodules, compatible with hidradenitis sup­ purativa were observed. Routine laboratory tests were within normal limits ex­ cept for mild leukocytosis (I 1,720 ttl) with 12% eosine­ phils. Multiple cultures from the pustules grew Staphylo­ coccus epidermidis. A biopsy specimen of a papulopus­ tular lesion on the back showed a slightly acanthotic and spongiotic epidermis with moderate parakeratosis. The main pathologic changes were confined to the mid dermis, in which a ruptured hair follicle with abscess formation

I. Mitsuhashi Y, Hohl D.Dermatitis herpetiforrnis in a patient with acquired immunodeficiency syndrome-related complex [Letter]. J AM ACAD DERMATOL 1988;18:583. 2. Kaplan MH, Sadick, N, McNutt NS, et al. Dermatologic findings and manifestations of acquired immunodeficiency syndrome (AIDS). J AM ACAD DERMATOL 1987;16:485506. 3. Janier M. Manifestations cutanees du SIDA. Ann Dermatol Venereol 1987;114:1487-8. 4. Kotler DP, Gaetz HP, Lange M, et al. Enteropathy associated with the acquired immunodeficiency syndrome. Ann Intern Med 1984;101:421-8.

A 12-year-old healthy female added on urgently to clinic for red, painful feet. Three days prior she developed fever, myalgias, fatigue, diarrhea, nausea and vomiting, and painful red bumps on the feet. All symptoms resolved after two days except foot pain, which progressed. The patient complained of burning, aching foot pain with severe TTP and inability to walk. In the past three weeks she had ridden her horse into a cold river on numerous occasions, most recently two days before her skin eruption. She had no prior history of skin disease. Examination revealed swollen, shiny, tense plantar skin bilaterally with numerous ill-defined bluish-red exquisitely tender nodules and macules on the plantar surfaces and sides of her feet. There was a suggestion of blanching around the lesions. The exam was otherwise normal.