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Chemotherapy-associated eccrine hidradenitis: neutrophilic eccrine hidradenitis reevaluated: the role of neutrophilic infiltration.

Two cases of chemotherapy-associated eccrine hidradenitis are presented. Previous cases have been labeled "neutrophilic eccrine hidradenitis" (NEH). Both cases reported herein involve neutropenic patients and are notable for the absence of neutrophils on histologic examination. Oncologists need to be aware of this newly described process, and this rash must be added to the differential diagnosis of the neutropenic oncology patient. The role of neutrophils in this disorder is discussed, and a new term, "chemotherapy-associated eccrine hidradenitis," is suggested for this unusual process.

Antineoplastic Agents↗

Idiopathic plantar hidradenitis: a neutrophilic eccrine hidradenitis occurring primarily in children.

Neutrophilic eccrine hidradenitis (NEH) has been described in a variety of clinical settings but is most often seen in leukemic patients receiving chemotherapy. We have recently encountered 6 healthy individuals, of whom 5 were children, who developed NEH localized to the feet. The patients were from 9 to 21 years of age. The presenting complaint was rapid development of tenderness of the feet with varying degrees of morbidity. At clinical evaluation, tender, erythematous papules and nodules were confined to the feet, primarily the plantar surfaces. The major differential diagnoses were erythema nodosum and vasculitis. Laboratory studies were non-contributory. Histologically, the findings were generally similar to those of NEH with certain exceptions, most notably the absence of syringosquamous metaplasia and the presence, in most cases, of neutrophilic abscesses in eccrine coils. Inflammatory and degenerative changes involved primarily the eccrine duct (coiled and dermal), and tended to spare the secretory apparatus. Stains for microorganisms were negative. There were brief recurrences in some of the patients, but those followed over time have remained well. We suggest the term idiopathic plantar hidradenitis for this condition.

Adolescent↗

Eccrine squamous syringometaplasia. A cutaneous sweat gland reaction in the histologic spectrum of 'chemotherapy-associated eccrine hidradenitis' and 'neutrophilic eccrine hidradenitis'.

A 31-year-old Hispanic man presented in the pancytopenic phase of acute myelocytic leukemia and was treated with the chemotherapeutic agents mitoxantrone and cytarabine. After 5 days, an erythematous, blanching, papular, crusted eruption developed on his forehead, chest, and legs. Some lesions showed confluence and all were at the same developmental stage. Clinical diagnoses included necrotizing vasculitis and sepsis. A biopsy specimen revealed widespread noninflammatory syringometaplasia of eccrine ducts. Well-developed intercellular bridges and eosinophilic cytoplasm were seen within the metaplastic cells; apoptoses and occasional mitoses were present. This process is distinct and probably occurred secondary to direct toxic injury from the chemotherapeutic drugs. Because similar changes have occurred in patients with neutrophilic eccrine hidradenitis, we believe our patient represents an example of the noninflammatory end of the spectrum of chemotherapeutic eccrine gland reactions.

Adult↗

Sebum excretion in hidradenitis suppurativa.

BACKGROUND: Clinical and histological similarities between hidradenitis suppurativa and acne have been pointed out. The possible role of sebaceous glands in hidradenitis has not previously been investigated. Acne treatment, in particular is however not effective in hidradenitis. No previous information was found on regional sebum excretion in hidradenitis. OBJECTIVE: Investigate the sebum excretion (SE) and markers of cutaneous virilization in hidradenitis patients. METHODS: Sebutapes and scores of acne, hirsutes and alopecia in 16 women with hidradenitis suppurativa were compared with 16 healthy controls. RESULTS: The SE and the number of active glands followed a similar pattern in both groups (face > axillae/genitofemoral fold, p < 0.0001) but no significant differences were seen between the two groups. No differences were seen in the median Body Mass Index, number of obese persons in either group or any of the clinical markers of virilization studied. CONCLUSION: No significant axillary, genitofemoral or facial seborrhea was found in hidradenitis. It is suggested that SE is not an important factor in the development of hidradenitis, and this may help explain the generally unsatisfactory therapeutic effect of retinoids in this disease. The results further suggest that general cutaneous virilization is not a feature of hidradenitis.

Acne Vulgaris↗

Cigarette smoking as a triggering factor of hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease involving the axillary, inguinal and anogenital regions and sometimes, in addition, the submammary or sacral areas. The etiology of this condition is unknown. OBJECTIVE: A matched-pair case-control study was performed to evaluate the influence of smoking habits on the manifestation of this disease. METHODS: Patients who had received surgical treatment for hidradenitis suppurativa in two dermatological centers completed a questionnaire dealing with family history, course of the disease and smoking habits. To form a randomized matched-pair control group, an equal number of patients admitted for various other skin diseases such as atopic dermatitis, varicose veins, skin tattoos, alopecia areata or melanoma was matched for sex and age and evaluated for smoking habits. Statistical analysis was performed by use of several chi2 tests in a cross-table setting. Moreover, a comparison to the expected smoking prevalence in Germany based on national statistics was performed. RESULTS: Out of 84 patients treated for hidradenitis suppurativa, 63 subjects (27 men, 36 women) completed the questionnaire. The rate of active cigarette smokers was 88.9% (56 patients), whereas 4 subjects (6.4%) had never smoked. 3 patients (4.8%) stated to be ex-smokers, but 2 of these had quit smoking only recently and after onset of the disease. The rate of smokers in the matched-pair control group was 46%. The significantly higher proportion of active smokers among patients with hidradenitis suppurativa can be expressed by an odds ratio of 9.4, the calculated 95% confidence interval was 3.7-23.7 (p < 0.001). The expected smoking prevalence in Germany was 26.7% according to national statistics. 73% of our patients had no family history of hidradenitis suppurativa whereas 27% reported at least one affected first-degree relative. CONCLUSION: From the exceedingly high rate of smokers among patients with this condition we conclude that cigarette smoking is a major triggering factor of hidradenitis suppurativa. Remarkably, the disease can be categorized as a smoking sequel that is neither of vascular nor neoplastic nature. Because familial occurrence was rather rarely reported, and because an environmental factor in the form of cigarette smoking appears to be of crucial importance to trigger the disease, we assume that the genetic basis of hidradenitis suppurativa is polygenic rather than mendelian. Smoking cessation should be encouraged particularly in patients with hidradenitis suppurativa although it is unknown whether this improves the course of the disease.

Adolescent↗

A case-control study of hidradenitis suppurativa in an STD population.

The prevalence of hidradenitis suppurativa has been found to be higher among patients attending an STD clinic than in an unselected general population sample. The aetiology of hidradenitis in unknown, but an association with chlamydial infections has been suggested for perineal lesions. Our aim was to describe the history of STD, STD risk factors and possible current STD in patients with hidradenitis. A case-control study comparing patients with hidradenitis (20) and controls (60) was therefore made in patients attending an STD clinic. Genital HPV infection was found to be more common in patients with hidradenitis (p = 0.036), but no differences were seen in the history of STD, STD risk behaviour or other current STD between the two groups studied. HPV infection appears to be the only STD which is more common in hidradenitis patients, but the biological significance of this finding is not clear. It is speculated that common predisposing factors may be responsible. We were unable to support the previously postulated association between Chlamydia trachomatis infection and hidradenitis.

Adult↗

Recurrent palmoplantar hidradenitis in children.

BACKGROUND: There are several reports of tender, erythematous plantar nodules occurring in pediatric patients. Despite similar morphological features, the histologic findings in these lesions have been quite diverse. A new entity called idiopathic plantar hidradenitis (also termed neutrophilic eccrine hidradenitis in children), which is characterized by tender, red nodules on the feet and unique histopathologic features, was recently described. OBSERVATIONS: We describe two children with these unusual cutaneous lesions and histopathologic findings and compare them with patients with idiopathic plantar hidradenitis. Our patients, in contrast to those with idiopathic plantar hidradenitis, had involvement of the palms as well as the soles. Both children had self-limited recurrent lesions; in one child, the lesions were associated with low-grade fever. Biopsy specimen findings in both cases demonstrated dense neutrophilic infiltrates localized to the eccrine units. CONCLUSIONS: Our patients had clinical and histologic findings similar to those recently reported as idiopathic plantar hidradenitis (neutrophilic eccrine hidradenitis in children). Because palms and soles can be affected and the lesions typically recur, we suggest that this condition be referred to as recurrent palmoplantar hidradenitis.

Child↗

Ultrasound examination of hair follicles in hidradenitis suppurativa.

OBJECTIVE: To describe the in vivo skin echostructure, hair follicle shape, and dermal thickness in hidradenitis suppurativa. DESIGN: Qualitative and quantitative assessment of high-frequency (20-MHz) B-mode ultrasound images of lesional and paralesional skin. SETTING: University hospital. PATIENTS: Age- and sex-matched outpatients with hidradenitis suppurativa (n = 15) and healthy control subjects (n = 13). Median age was 34 years (range, 31-38). RESULTS: Clinically normal paralesional hair follicles in hidradenitis have an abnormal shape. The follicles appear to be wider in the deep dermis, the difference being statistically significant in the genitofemoral region (P = .007). Patients with hidradenitis have larger follicles in the axilla than controls (P = .002). Mature acne and hidradenitis lesions are indistinguishable, but both are different from epidermal cysts. Mean axillary and genitofemoral skin was significantly thicker in patients than in controls. CONCLUSIONS: In vivo ultrasonography shows characteristic differences in the shape of hair follicles in hidradenitis. The general underlying abnormalities appear to occur in the deep part of the follicle. The mature lesions are indistinguishable from acne, but are clearly different from epidermal cysts. A thickened skin may play a pathogenic role in the development of hidradenitis.

Adult↗

The homogeneity of hidradenitis suppurativa lesions. A histological study of intra-individual variation.

Recent data suggest that hidradenitis suppurative is a disease of the follicle, but the histological homogeneity of findings over time and in different anatomical regions has not been verified. Its description may help towards a better classification of the follicular diseases. Correct classification provides both knowledge by interference and a way of generalizing with respect to the significance of specific findings. The intra-individual variation of hidradenitis was described through classification of specimens taken from patients with multiple simultaneous or consecutive excisions. A total of 51 specimens from 11 patients were examined; of these 30 were from synchronous biopsies, and 21 from consecutive biopsies (range 2 months to 6 years). The majority of specimens (44/51) contained poral occlusion, sinus tracts or cysts. This pattern was present in 50-85% of the specimens from any given patient with hidradenitis. No primary apocrine involvement was seen. Fibrosis occurred often (33/51 specimens), and eccrine involvement was seen more often than apocrine involvement (10 vs 7 specimens). The homogeneous histology of hidradenitis supports its reclassification as a follicular disease. The reproducibility of the findings further suggests that the changes are specific and that hidradenitis is therefore a definite disease entity within the spectrum of follicular diseases. Additional functional studies may help classify hidradenitis more precisely in relation to other follicular diseases.

Adult↗

The symptomatology of hidradenitis suppurativa in women.

In order to establish the possible role of androgen in the development of hidradenitis suppurativa the symptomatology of the disease was studied in a group of 70 female patients. The results were compared with those obtained from an age-matched control group of healthy women. The incidence of women with signs of androgenization in the two groups did not differ significantly. The only significant differences found were a shorter menstrual cycle and a longer duration of menstrual flow in the women suffering from hidradenitis, and that women with hidradenitis were more likely to have a positive family history of hidradenitis. The prevalence of hidradenitis in the control group was 4%. The results show that hidradenitis is not accompanied by other signs of androgenization. The disease may be due to local changes in the apocrine glands of predisposed individuals.

Adolescent↗

Verrucous carcinoma arising in hidradenitis suppurativa.

There are many reported cases of squamous carcinoma complicating hidradenitis suppurativa, but only one previous mention of verrucous carcinoma in this setting. We describe a case of verrucous carcinoma arising in hidradenitis suppurativa of the anal margin in a non-immunosuppressed man. This is the second report of verrucous carcinoma arising in a lesion of hidradenitis suppurativa. Although hidradenitis suppurativa can involve multiple intertriginous sites, malignant degeneration occurs mostly in the anogenital region. This suggests a role for a regional factor which, when combined with chronic inflammation, predisposes to malignant degeneration. A likely candidate for this factor is human papillomavirus; our case showed histologic evidence for this, but the specimen did not show viral DNA by polymerase chain reaction in situ hybridization. The ability of anogenital hidradenitis suppurativa to form squamous and verrucous cancers reinforces the argument for early and complete resection.

Anus Neoplasms↗

The prevalence of hidradenitis suppurativa and its potential precursor lesions.

BACKGROUND: The morbidity of hidradenitis suppurativa can be considerable, but little is known about its epidemiology. OBJECTIVE: Our purpose was to describe the 1-year and point prevalences of hidradenitis suppurativa and its potential precursor lesions. METHODS: We obtained the histories and examined an unselected sample (599 persons) of the general population (1-year prevalence), and we performed physical examinations for a consecutive sample of 507 persons undergoing screening for sexually transmitted diseases (point prevalence). RESULTS: The point prevalence was 4.1% (95% confidence interval [CI] = 3.0-6.0) on the basis of objective findings. The 1-year prevalence of hidradenitis was 1.0% (CI = 0.4-2.2) on the basis of subject recollection only. The patients in the sample on which point prevalence is based were younger than those in the unselected sample of the general population (p < 0.001). Hidradenitis was significantly more common in women (p = 0.037), which may result from a female preponderance of genitofemoral lesions (odds ratio [OR] = 5.4; CI = 1.5 - 19.3). No sex difference was found in the prevalence of axillary lesions. CONCLUSION: Hidradenitis suppurativa is significantly more common than hitherto estimated. The female preponderance of patients is confirmed, except for patients with axillary lesions. Additional longitudinal studies are necessary to assess the importance of potential precursor lesions such as noninflamed nodules or comedones.

Adolescent↗

Treatment of hidradenitis suppurativa with carbon dioxide laser excision and second-intention healing.

BACKGROUND: Hidradenitis suppurativa is a disabling disease. Although medical management can at times control the disease, the treatment of choice is surgical excision. Surgical defects have been managed by second-intention healing, simple surgical closure, or complex surgical repairs. OBJECTIVE: Our purpose was to determine the benefit of carbon dioxide laser excision with healing by second intention to treat lesions of hidradenitis suppurativa. METHODS: We treated seven patients who had axillary and inguinoperineal hidradenitis suppurativa with carbon dioxide laser excision followed by second-intention healing. A total of 12 procedures were performed (five bilateral and two unilateral). Follow-up periods were 10 to 27 months. RESULTS: After a healing time of 4 to 8 weeks, scars were flat and linear. Only one recurrence of hidradenitis suppurativa has been observed; this occurred along the margin of the surgical scar in a previously treated area. Some patients, however, had continued disease activity in untreated areas. All subjects were satisfied with the results of the procedure, and complications were minimal. CONCLUSION: Carbon dioxide laser excision with second-intention healing is safe and effective for hidradenitis suppurativa.

Adult↗

Palmoplantar eccrine hidradenitis: three new cases and review.

Palmoplantar eccrine hidradenitis (PEH) is characterized by painful erythematous papules and nodules of abrupt onset on the soles of young individuals. The histologic hallmark is a predominant neutrophilic infiltrate surrounding the eccrine gland apparatus. A total of 28 cases have been published since 1988, with a broad variation in the age of patients, symptomatology, associated diseases, clinical course, and histologic features. These different histologic pictures have resulted in several names for this entity, including idiopathic plantar hidradenitis, neutrophilic eccrine hidradenitis, recurrent palmoplantar hidradenitis in children, and idiopathic palmoplantar hidradenitis. We present three cases of this disorder and review the literature on the varying symptomatology, clinical course, and histology that have led to the myriad of names for this disease.

Adult↗

Hidradenitis suppurativa--characteristics and consequences.

The general characteristics of patients with hidradenitis suppurativa have not been previously described in detail. Although rare complications of the disease have been described, little is known of the average impact of hidradenitis suppurativa. We have now aimed to describe the general characteristics of the disease and its impact on patients with an established diagnosis of hidradenitis (n = 68) and compare these with those of an unselected general population sample (n = 523). Pierced earlobes were more common in patients than in the controls (P < 0.02). Female patients were younger (P = 0.0002) and better educated (P < 0.0001), and fewer had been pregnant (P = 0.0006). The median number of treatments prior to referral was two, and an average of 2.7 work days/patient per year were lost due to hidradenitis. The self-reported health of patients was significantly inferior to that of the general population (P < 0.001), mainly because of soreness and restriction of movement. Low-grade hidradenitis suppurativa therefore has a considerable impact on the health of patients. Previously implied associations with the disease such as the use of cosmetics or oral contraceptives, menstrual cycle and body mass index (BMI) were not confirmed in our study, although they may influence the course pre-existing disease.

Adult↗

Granulomatous hidradenitis suppurativa and cutaneous Crohn's disease.

Three patients with concurrent hidradenitis suppurativa and Crohn's disease are presented. The notable histological feature in each hidradenitis resection was the presence of numerous discrete epithelioid granulomas in areas of non-inflamed dermis. The purpose of the study was to determine the incidence of epithelioid granulomas in 101 hidradenitis patients and their significance in relation to systemic granulomatous disease. Discrete epithelioid granulomas were identified in 8% of the resections (10 patients). One patient had Crohn's disease and one other pulmonary sarcoidosis. Seven patients with granulomatous hidradenitis neither had nor developed any other disease during the 3-year follow-up period. Clinical review identified a further two patients with Crohn's disease but associated with non-granulomatous changes in the skin resections. Foreign body type granulomas were identified in 25% of the resections adjacent to ruptured hair follicles, sinus tracts or nearby degenerate sweat glands. The study shows that, although foreign body type granulomas are a common finding in hidradenitis, the presence of discrete epithelioid granulomas in the dermis away from the site of active inflammation is unusual and should alert the pathologist to the possibility of systemic granulomatous disease such as Crohn's disease or sarcoidosis.

Adolescent↗

[Prevalence of hidradenitis suppurativa in Denmark].

The morbidity of hidradenitis suppurativa can be considerable, but little is known about its epidemiology. Our purpose was to describe the one-year and point prevalences of hidradenitis suppurative and its potential precursor lesions. We obtained the histories and examined an unselected sample (599 persons) of the general population (one-year prevalence), and we performed physical examination for a consecutive sample of 507 persons undergoing screening for sexually transmitted diseases (point prevalence). The point prevalence was 4.1% (95% confidence interval [CI] = 3.0-6.0) on the basis of objective findings. The one-year prevalence of hidradenitis was 1.0% (CI = 0.4-2.2) on the basis of subject recollection only. The patients in the sample on which the point prevalence is based were younger than those in the unselected sample of the general population (p < 0.001). Hidradenitis was significantly more common in women (p = 0.037), which may result from a female preponderance of genitofemoral lesions (odds ratio [OR] = 5.4; CI = 1.5-19.3). No sex difference was found in the prevalence of axillary lesions. Hidradenitis suppurativa is significantly more common than hitherto estimated. A female preponderance of patients is confirmed, except for patients with axillary lesions. Additional longitudinal studies are necessary to assess the importance of potential precursor lesions such as non-inflamed nodules or comedones.

Adult↗

Hidradenitis suppurativa of the anogenital region: response to isotretinoin.

Hidradenitis suppurativa, a chronic relapsing disease of apocrine gland-bearing areas, most frequently occurs in the axillae, groin, perineal, and perianal regions. Hidradenitis of vulva is frequently misdiagnosed and inadequately treated. The case of a 15-year-old nulliparous black female adolescent referred for evaluation of multiple draining fistulas of the anogenital region is presented. Diagnostic studies for granulomatous disease were negative. Results of a barium enema were normal and biopsies were compatible with the diagnosis of hidradenitis suppurativa. She was treated for 22 weeks with isotretinoin, 1 mg/kg daily, with an excellent response. Side effects were minor and included cheilitis, mild xerosis, and a transient elevation of serum alkaline phosphatase levels. Few patients with severe hidradenitis have been responsive to this synthetic vitamin A derivative. A review of the literature indicates that the results of treatment with isotretinoin for hidradenitis have been at best equivocal. Isotretinoin should never be used during pregnancy because of known teratogenic effects. Women of childbearing age must use effective contraception during treatment.

Adolescent↗