Intertriginous Areas Research Articles

Treatment of acanthosis nigricans with sequential salicylic acid-mandelic acid combination peel and maintenance with glycolic acid-urea combination cream: A retrospective pilot study.

Acanthosis nigricans (AN) is a common dermatosis that presents with hyperpigmented, velvety thick plaques over intertriginous areas. Though a number of treatment modalities including chemical peels have been used, none provide long-term and sustained improvement. Our study evaluated the efficacy and safety of regular sessions of salicylic acid-mandelic acid peeling over axillary AN lesions, which was followed by daily application of a topical combination of glycolic acid, urea, and cetylated fat esters for maintenance of effect for 9months. A retrospective pilot study was conducted in Indian patients (Fitzpatrick skin type 4 or 5), aged 18-50 with benign hereditary AN involving the underarms, with or without affection of other typical sites. Data were retrieved of seventeen patients with AN involving the axillae. Patients were started on combination salicylic-mandelic acid peel given every 2weeks for a total of 6sessions. Maintenance was done by night application of combination cream of glycolic acid, urea, and cetylated fat esters, which was continued for 9months after completion of peeling sessions. Lesions were evaluated every 3months of 9months for improvement in pigmentation and skin thickening. All the patients (100%) showed significant improvement in both pigmentation and thickening of lesions. In terms of improvement in skin thickening, very good improvement was seen in 41%, while 29% patients had moderate improvement. In terms of improvement in pigmentation, 35% each had very good and moderate improvement. Post peel erythema (100%) and burning sensation (90%) were the most common encountered adverse effects which lasted for only 1-2days. Patients were followed up for another 9months during which no relapses were seen. Combination of keratolytic chemical peels and topical mild keratolytic application ensures better therapeutic outcome in patients of AN with long lasting effect.

AEROBIC – ANAEROBIC CUTANEOUS MICROBIOTA OF HIDRADENITIS SUPPURATIVA. A CASE REPORT

Hidradenitis suppurativa (HS) is a chronic inflammatory disease affecting intertriginous skin areas of young adults. Although HS was historically defined as an infectious disease, the role of microorganisms in its pathogenesis is now controversial. We investigated the lesion microbiota of a recently diagnosed 24-year-old male patient with axillary HS, who shared features of the metabolic syndrome. The microbiological analysis of the patient’s lesion was indicative of colonization with bacteria including aerobes – Staphylococcus (S.) aureus, S. lugdunensis, S. epidermidis, anaerobes – Anaerococcus (A.) octavius, and microaerophiles – Cutibacterium (C.) acnes; this last one was also isolated in monoculture from a patient's healthy skin control sample. In vitro antibiotic susceptibility evaluation revealed Methicillin-resistant S. aureus (MRSA) and highly susceptible S. lugdunensis and S. epidermidis. Profiles of A. octavius and C. acnes showed that both strains were susceptible to Vancomycin, Clindamycin, Doxycycline and Linezolid; the anaerobe was Benzylpenicillin-resistant and Tetracycline and the microaerophile was intrinsically Metronidazole-resistant. We conclude that the intralesional microbial diversity in this case shows low variety of species and similarities to healthy skin microbiota, although the presence of S. aureus, particularly MRSA, S. lugdunensis or C. acnes – frequently involved in chronic skin pathologies – may be relevant for the disease severity. MRSA carriage in the patient’s lesion represents a significant epidemiological risk for the healthcare environment. Antibiotic treatment in the early stages of HS should be administered with caution. Extended studies of HS microbiota could identify relevant colonization patterns for the outcome of the disease and offer important keys for selecting personalised, efficient therapeutic schemes. Keywords: hidradenitis suppurativa, cutaneous microbiome, bacterial pathogens, MRSA, Staphylococcus aureus, Cutibacterium acnes, anaerobes

Two Novel ATP2C1 Mutations in Portuguese Patients with Hailey-Hailey Disease


 
 
 Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis. It is characterized by a recurrent eruption of vesicles, erosions, and scaly erythematous plaques involving intertriginous areas and first occurring after puberty, mostly in the third or fourth decade. In 2000, mutations in the ATP2C1 gene on band 3q22.1, encoding the secretory pathway Ca2+/Mn2+-ATPase protein 1(hSPCA1), have been identified as the cause of HHD. We report the identification of two novel mutations of ATP2C1 gene in two Portuguese patients, which expands the spectrum of ATP2C1 mutations underlying HHD and provides useful information for genetic counseling.
 
 

Dermatologic Manifestations of Systemic Diseases in Childhood

Dermatologic Manifestations of Systemic Diseases in Childhood

Lichen planus pigmentosus in a 7-year-old girl.

LPP is a rare variant of LP that is infrequently encountered in children. In a retrospective analysis of 316 pediatric patients with LP, only nine (2.8%) were diagnosed with LPP (4). Similarly, in a recent retrospective study of 76 pediatric patients with LP in South India, only one (1.3%) was diagnosed with LPP (6). It is commonly seen in middle-aged patients with high skin phototypes. LPP presents as chronic, acquired, violaceous to brownish-gray, oval macules, with poorly-defined borders (7). The lesions appear more frequently in the neck and face, but they can also affect the upper and lower extremities, and torso as well. LPP rarely affects the oral mucosa, scalp, palms, soles, and nails. Flexural involvement is seen in around 20% of cases with a concurrent photo-exposed distribution (5). LPP-inversus is a variant of LPP, limited to intertriginous and flexural areas, and sparing sun-exposed areas (3, 5). A skin biopsy will confirm the diagnosis. The histopathology findings include hyperkeratosis and vacuolar degeneration of the basal layer, dermal band-like or perivascular infiltrate, and melanophages. Superficial pigmentary incontinence can be seen (7). The main differential diagnosis of LPP is erythema dyschromicum perstans (EDP) or ashy dermatosis. EDP presents as non-pruritic, blue-gray macules and patches, with or without raised erythematous borders in photo-exposed areas and trunk. Histopathology reveals lichenoid features within the peripheral active border. A perivascular infiltrate and numerous melanophages can be seen. The clinical features and histopathology of LPP and EDP can overlap, so much so as to suggest that the two conditions can be part of the spectrum of a unique entity. Other differential diagnoses include post-inflammatory hyperpigmentation, pigmented contact dermatitis, fixed drug eruption, and urticaria pigmentosa, among others (2, 7). LPP treatment is difficult, with limited results in most cases. The available topical treatments include medium to high potency corticosteroids, calcineurin inhibitors (mostly tacrolimus 0.1% ointment) and skin lightening creams containing hydroquinone and retinoids in conjunction with an anti-inflammatory treatment (3, 7, 9). Some Authors (3) recommend that in order to prevent steroid atrophy, corticosteroids should be used for a short period (2-4 weeks). Later on they could be followed by a calcineurin inhibitor over a longer period (3-6 months). In recalcitrant or widespread cases, systemic treatments with or without topical therapy can be used. They include oral corticosteroids, dapsone, and isotretinoin (7, 9, 10). The use of narrow band ultraviolet B (NB-UVB) phototherapy in LPP has been recently reported. Complete resolution of lesions was achieved after 20 exposures and no adverse effect was observed (1).

Light and Laser-based Treatments for Hidradenitis Suppurativa: A Systematic Review

Background: Hidradenitis suppurativa (HS) is characterized by painful, recurrent lesions occurring mainly in intertriginous areas. The pain, odor, and disfigurement caused by HS significantly impacts quality of life and is challenging to treat. A comprehensive systematic review evaluating the use of light and laser-based treatments for HS is lacking. Methods: We performed a systematic review by searching Cochrane, MEDLINE and Embase. Title, abstract and full text screening, and data abstraction were done in duplicate. Results: Forty studies met the inclusion criteria, representing a total of 821 patients. Included studies were comprised of 5 randomized within-patient controlled trials, 1 randomized controlled trial, and 34 case series. Overall, treatments with the most reported cases were laser surgery, photodynamic therapy (PDT), and laser field treatments which showed a response in 80% (n=344/431), 73% (n=122/167) and 71% (n=84/101) of treated patients respectively. The pooled response rate for psoralen plus ultraviolet A was 69% (n=9/13). Conclusion: Our results suggest that laser surgery using carbon dioxide (CO2) laser or a combination of CO2 and Nd:YAG lasers has a moderate response rate for HS with the most reported cases. Laser for field treatment and PDT also had moderate response rates with a large number of reported cases. However, extrapolation of these results may be limited due to the majority of the studies being case series, lack of standardized outcomes being assessed, and insufficient long term follow up results.

New Topical Therapies for Psoriasis.

Psoriasis is a chronic immune-mediated skin disease with a significant impact on patients' quality of life. Mild-to-moderate forms of the disease usually require long-term topical treatment, but prolonged use of corticosteroids and vitamin D analogues is limited by adverse effects. With further understanding of psoriasis pathogenesis, new molecules are emerging aiming to fulfil these clinical needs. Tapinarof, an aryl hydrocarbon receptor modulator, has completed a phase III study and demonstrated good efficacy results, even in long treatment courses, with a favourable safety profile. It additionally appears to have a promising remitting effect as patients presented with an average relapsing time of over 3 months. Roflumilast, a phosphodiesterase type 4 inhibitor, also underwent a phase III study with significant lesion improvement and notable pruritus management, and with no reported side effects. Roflumilast was evaluated as an option for intertriginous areas with good outcomes in a small sample, but larger trials are required. The Janus kinase-signal transducer and activator of transcription pathway has been targeted in recent clinical investigations with promising options, currently with brepocitinib pending phase IIb results. Ongoing preclinical studies involving interleukin-2 inhibition, RNA modulators and amygdalin analogues may lead to forthcoming clinical trials. New topical drugs are successfully emerging and future research comparing them to classical options will dictate their clinical role in the treatment of psoriasis.

Lichen Planus Pigmentosus Inversus: Two Case Reports

Abstract Introduction: Lichen planus pigmentosus inversus (LPPI) is a rare a rare variant of lichen planus characterized by hyperpigmented patches with predominating localization in intertriginous areas. Due to its rarity, only a few LPPI cases are reported. We herein describe two rare cases of LPPI. Case presentation: The two patients were all with a brownish macular lesion on the intertriginous area. A diagnosis of LPPI was made based on their clinical manifestations, dermoscopic features, and histopathologic features, which revealed an interface change, lichenoid infiltration, and pigmentary incontinence. Discussion: LPPI is pruritic or asymptomatic, hyperpigmented macules and patches on the flexural folds. The axillae and flanks were the most commonly affected areas, followed by the groin and genitalia. About half of the female patients had inframammary fold lesions. LPPI shows higher female predominance than Lichen planus pigmentosus. There were fewer cases that lasted more than 3 years compared to LPP. Conclusion: LPPI is a rare variant of lichen planus, with a locational characteristic and female predominance. Therefore, in the case of a pigmented disease occurring in the flexural folds, it should be placed in the differential diagnosis.

Histopathologic features of inverse psoriasis.

Inverse psoriasis represents a less commonly described form of psoriasis in intertriginous areas. The pathologic findings of inverse psoriasis are typically grouped in with those of plaque psoriasis, as the histopathologic features specific to inverse psoriasis have not received significant investigation. A single institution, retrospective cohort study was performed to review biopsy slides for psoriasis occurring in typical intertriginous areas. Patient's charts were reviewed and only those where the clinical diagnosis of inverse psoriasis was also favored were included. Twelve patients met inclusion criteria: 58% male and 42% female, 18 to 86 years of age. Classic features of psoriasis such as hypogranulosis, confluent parakeratosis, and thinning of the suprapapillary plate were seen in 100%. Regular psoriasiform acanthosis and dilated tortuous dermal vessels were seen in 92%. Neutrophils were present in the scale in 83% and in the dermis in 100%. Features considered atypical for psoriasis included spongiosis in 83%, eosinophils in 67%, and focal serum in the scale in 42%. While inverse psoriasis commonly exhibits features considered to be classic for psoriasis, it is not unusual for inverse psoriasis to show features considered atypical for plaque psoriasis such as dermal eosinophils, epidermal spongiosis, and focal serum in the scale.

A safety review of biologic therapies for the management of hidradenitis suppurativa and unmet needs

ABSTRACT Introduction Hidradenitis suppurativa (HS) is a chronic, debilitating inflammatory skin disorder characterized by nodules, abscesses, fistulae, and significant scarring in intertriginous areas rich in apocrine glands. Immunomodulator drugs, including biologics, are a mainstay of treatment for this disease. Areas covered This review details the safety profiles of various biologic therapies currently available commercially that have been tried for HS as assessed in clinical trials and observational studies. As the only Food and Drug Administration (FDA)-approved medication for the treatment of moderate-to-severe HS, adalimumab is discussed in the most detail. Additional biologic medications, including tumor necrosis factor α (TNFα) inhibitors, interleukin 1 (IL-1) inhibitors, IL-12 and IL-23 inhibitors, IL-17 inhibitors, and IL-23 inhibitors are discussed as well. Safety concerns in special populations, including pregnant women and children, are outlined. Expert opinion Existing data supports excellent short-term and long-term safety profiles for adalimumab, although caution must be taken with use in high-risk patient populations, including those with chronic infections or increased risk of malignancy. Based on their safety data for other indications, additional biologic agents appear safe in HS as well. However, further research is needed to fully understand the safety profiles of these medications in the HS population.

Wound dressings improve quality of life for hidradenitis suppurativa patients

To the Editor: An important challenge that patients with hidradenitis suppurativa (HS) face daily is wound care. Dressings are often designed for flat surfaces, making it difficult to fix dressings over dynamic contours of intertriginous areas. Frequent dressing changes for exudative, odorous wounds significantly detract from the quality of life (QOL) for HS patients.1 However, no standard wound care protocol exists for addressing HS lesion variety.2 Therefore, we designed a prospective cohort pilot study to determine the impact on QOL of wound dressing type and access in HS patients.

Darier and Hailey‐Hailey disease: update 2021

The autosomal-dominant genodermatoses Darier disease and Hailey-Hailey disease present special challenges to dermatologists. Despite their similar pathogenesis featuring impaired adhesion of suprabasal keratinocytes as a result of defective ATPases in epidermal calcium channels, the two diseases differ considerably in clinical presentation and therapeutic options. Darier disease is characterized by reddish brown, keratotic papules in seborrheic and intertriginous areas, which may coalesce into extensive lesions. Individuals affected with Hailey-Hailey disease primarily develop intertriginous papulovesicles and small blisters, which often evolve into erythematous plaques with erosions and painful fissures. Quality of life is significantly reduced because of complaints (itch, burning sensation, pain), body malodor and chronicity. Therapeutic options remain limited. Antiseptics and intermittent topical corticosteroids are a cornerstone of therapy, and systemic anti-infective treatment is often required in cases of superinfection. Ablative surgical interventions such as dermabrasion and CO2 laser surgery can lead to long-term remissions in intertriginous Hailey-Hailey disease, while temporary relief may also be achieved by intralesional injections of botulinum toxin. Of the systemic medications available for Darier disease, acitretin, which is approved for this purpose, has the best supporting evidence. The efficacy of immunosuppressants and immune modulators is inconsistent. Low-dose naltrexone produces more satisfactory results in Hailey-Hailey than Darier disease. The present CME article summarizes current knowledge of the two dermatoses, taking recent developments into account.

197 Revertant mosaicism as a clue for the role of keratinocytes in innate immunity against Candidaspecies

The role of epidermal keratinocytes (KC) in the innate immunity against pathogens has not been proven in human, aside from several lines of evidence in vitro. Cutaneous Candidiasis is limited to intertriginous area in immune-competent individuals. In absence of systemic immune-deficiency, however, patients with keratitis-ichthyosis-deafness (KID) syndrome caused by mutations in GJB2 coding a gap-junction protein Connexin (Cx) 26 develop chronic cutaneous Candidiasis (CCC) in extremities or trunk. A patient with KID syndrome harboring a germline heterozygous mutation in GJB2 (p.D50N) developed numerous patches of apparently normal skin only after the remission of CCC. We collected 5 revertant patches and separated the epidermis. Exome sequencing identified an additional mutation in GJB2 in each sample. These five mutations were independent to each other, suggesting emergence of independent clones harboring additional mutations in GJB2. All of the four mutations that were phase-able were in cis with D50N. Given that D50N causes hyperactivation of Cx26, these mutations are likely to dampen the effect of D50N in KC. The appearance of revertant patches indicates the impaired survival of KC with D50N in CCC, compared to revertant KC. Since revertant KC are predicted to be similar to wild type KC in the channel function of Cx26, KC with D50N can also be suspected to have impaired survival ability in CCC than wild type KC. Thus, we hypothesized that CCC in KID syndrome is based on the vulnerability of KC with germline mutations to Candida sp. We are currently addressing this hypothesis by analyzing the in vitro survival of KC with different genotypes of GJB2 under the presence of Candida sp. and establishing an in vivo model of CCC using inducible KID mouse model using K14-CreERT mice. In conclusion, we suspected the impaired survival of KC against Candida sp. in KID syndrome through the analysis of revertant patches. The presented case might be the first human phenotype showing the role of wild type KC in the resistance against Candida sp. in unaffected individuals.

Vulvoperineal Hidradenitis Suppurativa: Diagnosis, Treatment, and Management of Deformities.

Hidradenitis suppurativa (HS) is a chronic, inflammatory disorder affecting skin of intertriginous areas that is often encountered and treated by nondermatologic specialists. The purpose of this literature review is to provide a comprehensive, clinical source of information on HS as it relates to incidence of disease, pathophysiology, diagnosis, and overall management of this condition. Sources were obtained through a comprehensive literature search using PubMed and PMC. Various terms were used to query the database, including "hidradenitis suppurativa," "pathogenesis," "prevalence," "management," "surgery," "perineal," and "vulva." Underreported prevalence and unknown pathogenesis have subsequently led to variable approaches in clinical management, often employing a combination of medical and surgical management. Early diagnosis and treatment of HS may lead to better disease control and minimize patients' associated morbidity related to disease. Knowledge of vulvoperineal hidradenitis is necessary for gynecologists and primary care physicians to ensure early diagnosis, management, and referral for optimal patient outcomes.

Evolving Scalp Lesions in an 8-day-old Infant

Evolving Scalp Lesions in an 8-day-old Infant

Asymptomatic Hyperpigmented Rash in a Healthy Adolescent Female.

Asymptomatic Hyperpigmented Rash in a Healthy Adolescent Female.

The Temporal Evolution of Distinct Skin Surface Microbiome in Asian Patients with Severe Hidradenitis Suppurativa during Effective Adalimumab Treatment

Hidradenitis suppurativa (HS) is a debilitating, chronic recurrent inflammatory disease primarily involving the folliculopilosebaceous units of the intertriginous areas. Abscesses, sinus tracts, fistulas, and scarring may occur in severe cases and negatively impact the QOL of the patients (Hunger et al., 2017; Saunte and Jemec, 2017). Bacteria have been implicated in the pathogenesis of the disease, but their role remains unclear (Nikolakis et al., 2017).

LB751 COVID-19 complications in patients with Hidradenitis Suppurativa: A multicenter study

Hidradenitis Suppurativa (HS) is a chronic inflammatory skin disorder that causes abscesses in intertriginous areas and is also associated with numerous other conditions. There is limited literature on the outcomes of COVID patients with HS so the goal was to investigate the impact of AD on COVID outcomes. A retrospective cohort study was done using TriNetX, a federated real time database of 63 million records. COVID patient cohorts were identified by validated ICD-10 and serology codes per CDC guidelines from 1/20/2020 to 2/23/2021.

27945 Biologics-resistant areas in Korean patients with moderate to severe psoriasis

Background: Psoriasis in certain body sites such as palmoplantar, nail, scalp and intertriginous areas has been known to be difficult to treat. However, to date, there have been no studies for biologics-resistant areas in patients with moderate to severe psoriasis in Korea.

28340 Identification of flare triggers in hidradenitis suppurativa

Background: Patients with hidradenitis suppurativa (HS) experience painful nodules and abscesses in the intertriginous areas. Repeated flares in HS lead to progressive worsening of disease. We aimed to identify common triggers for HS exacerbation from the patients’ perspective.