Eosinophilic pustule smear in the diagnosis of pediatric post-transplant eosinophilic folliculitis.

Hematologic-associated eosinophilic pustular folliculitis is a subtype of eosinophilic pustular folliculitis (EPF) which develops in patients with underlying hematological malignancies after treatment with chemotherapy, bone marrow transplant (BMT), or stem cell transplant (SCT). Few cases of hematological-associated EPF have been reported in pediatric patients. Skin biopsy is considered the gold standard for diagnosis. We describe a case in which Wright staining of a pustule smear for eosinophils provided data to rapidly support a clinical diagnosis of hematologic-associated EPF.

A 6-Year-Old Boy With Cough, Mucositis, and Vesiculobullous Skin Lesions.

A 6-year-old boy taking no regular medications had persistent fever and cough for 15 days. Physical examination revealed eyelid swelling; vesiculobullous lesions on the palms and soles; vesicles and erosions on the face, trunk, and limbs; erosions on the lips and oral mucosa; and blisters on the anal mucosa. Laboratory testing revealed leukocytosis, and lung auscultation revealed bilateral crackles. What is the diagnosis and what would you do next?

Nonmelanoma skin cancer in the setting of erosive pustular dermatosis of the scalp: A case series and comment on management implications.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory cutaneous disorder typically affecting sun-damaged skin of mature individuals. Clinical features of EPDS include sterile pustules and chronic crusted erosions that can be hyperkeratotic and lead to scarring alopecia, atrophy, and telangiectasia. While the condition occurs on sun-damaged skin, a relationship with non-melanoma skin cancer (NMSC) has not been investigated. OBJECTIVES: Here we attempted to identify cases of NMSC developing in the setting of EPDS. METHODS: Retrospective review of EPDS cases in a dermatology practice. RESULTS: Six patients with mean (range) age 82 (65-92) years that developed NMSC in the setting of EPDS are reported. Five patients had skin phototype I or II associated with substantial solar elastosis. Four patients had history of NMSC. Four patients developed squamous cell carcinoma and two patients basal cell carcinoma on the scalp in the setting of EPDS. A morphologic change in an EPDS lesion, such as a crusted plaque becoming nodular and/or growing significantly within a relatively short period of time, prompted a biopsy that revealed NMSC. CONCLUSIONS: NMSC may develop in the setting of EPDS. Possible mechanisms underlying this association include the chronic inflammation associated with EPDS and ultraviolet light exposure. It is crucial to promptly obtain a biopsy in EPDS cases showing signs suspicious for NMSC. Further studies are required to confirm whether NMSC shows a higher prevalence in the setting of EPDS.

Eosinophilic folliculitis due to wearing protective gear in citizens volunteering for sanitation services during the COVID-19 pandemic - an original epidemiological, clinical, dermoscopic, and laboratory-based study.

BACKGROUND: An association between wearing protective gear and eosinophilic folliculitis has not been reported. We aimed to investigate such during the COVID-19 pandemic. METHODS: In three outpatient clinics, we hand-reviewed records of all patients having consulted us during a Study Period (90 days) in the early phase of the pandemic. Our inclusion criteria for Study Subjects were: (i) clear clinical diagnosis, (ii) dermoscopic confirmation, (iii) differential diagnoses excluded, (iv) eosinophilia, (v) protective gear worn during sanitation services, (vi) temporal correlation, (vii) distributional correlation, (viii) physician-assessed association, and (ix) patient-assessed association. Control Periods in the same season were elected. RESULTS: Twenty-five study subjects fulfilled all inclusion criteria. The incidence was significantly higher than in the control periods (IR: 3.57, 95% CI: 1.79-7.43). Male predominance was significant (P < 0.001). Such for patients in the control periods were insignificant. Study subjects were 21.2 (95% CI: 11.0-31.4) years younger than patients in the control periods. For the study subjects, the distribution of erythematous or skin-colored folliculocentric dome-shaped papules and pustules were all compatible with body parts covered by the gear. Lesional biopsy performed on two patients revealed eosinophilic dermal infiltrates within and around the pilosebaceous units. Polarized dermoscopy revealed folliculitis with peri-/interfollicular vascular proliferation. Lesion onsets were 6.4 (SD: 2.1) days after wearing gear. Remissions were 16.7 (SD: 7.5) days after ceasing to wear gear and treatments. CONCLUSIONS: Wearing protective gear in volunteered sanitizing works could be associated with eosinophilic folliculitis. Owing to the significant temporal and distributional correlations, the association might be causal.

Erosive pustular dermatosis of the scalp following surgical procedures: a systematic review.

Erosive pustular dermatosis of the scalp (EPDS) occurs in elderly individuals with significant actinic damage. EPDS also occurs in association with surgery; however, significant studies determining an association of EPDS with type of surgical closure is absent. This review examines whether the closure method following cutaneous surgery performed on the scalp is associated with development of EPDS. Databases were reviewed and studies describing EPDS after cutaneous surgery met inclusion criteria. Articles were excluded if EPDS developed after trauma or non-surgical procedures. Descriptive analyses were performed on the data. Thirteen case reports and 6 case series involving 32 patients met inclusion criteria. Fourteen articles (73.7%) stated that EPDS developed in the same location as, or near to, the closure site. Thirteen patients (40.6%) developed EPDS following skin grafting. Three patients (9.4%) developed EPDS following secondary intention healing, two patients (6.3%) following repair by primary intention, and one patient (3.1%) following repair with a local skin flap. Thirteen cases (40.6%) did not specify closure type. This review revealed that surgical procedures performed on the scalp utilizing skin grafts for closure may be increasingly associated with the development of EPDS compared to other closure types.

A case of bullous Sézary syndrome.

Sézary syndrome is a rare leukemic subtype of cutaneous T cell lymphoma that is characterized by erythroderma, lymphadenopathy, and malignant T cells in the peripheral blood. Poor prognostic factors of Sézary syndrome include advanced disease stage, older age at onset, and large cell transformation. Presentation with bullous lesions, though rare, has been reported in a few patients. We present an elderly woman with bullous Sézary syndrome who presented with a two-month history of progressive rash. Upon admission, the patient had pruritic, erythematous, edematous plaques with overlying flaccid bullae and erosions involving the scalp, neck, torso, and extremities. Despite treatment, the patient died two months after presentation. Although rare, bullous lesions associated with Sézary syndrome may indicate poor prognosis.

Upregulation of IL-36 cytokines in folliculitis and eosinophilic pustular folliculitis.

BACKGROUND: Members of the interleukin (IL)-36 family, IL-36α, IL-36ß and IL-36γ, are potent chemoattractive cytokines for neutrophils and eosinophils. IL-36 receptor antagonist (IL-36Ra) inhibits IL-36α, IL-36ß and IL-36γ activity. However, the immunohistological expression of IL-36α, IL-36ß, IL-36γ and IL-36Ra has never been addressed in normal follicles, folliculitis or eosinophilic pustular folliculitis (EPF). METHODS: We performed immunohistochemical staining for IL-36α, IL-36ß, IL-36γ and IL-36Ra using 10 cases of EPF, nine of non-specific folliculitis, 10 normal skin samples and 10 samples of normal follicles adjacent to a sebaceous naevus as a control. Two dermatologists, who were blind to the patient records, evaluated all of the slides. RESULTS: The immunoreactive IL-36α was hardly detected in the follicular epithelium and epidermis in the normal skin, folliculitis or EPF. The expression of IL-36ß, IL-36γ and IL-36Ra was augmented in both folliculitis and EPF compared with that in normal follicles. Negative correlations were detected between IL-36ß and IL-36Ra and between IL-36γ and IL-36Ra in normal follicles; however, these were absent in folliculitis. In contrast to normal follicles and folliculitis, a significant positive correlation between IL-36ß/γ and IL-36Ra was shown in EPF. CONCLUSIONS: The overexpression of IL-36ß, IL-36γ and IL-36Ra is an integral part of the inflammatory response of folliculitis and EPF. The coordinated expression of IL-36γ and IL-36Ra may be related to the pathomechanism of EPF.

Neonatal vesiculopustular eruption in Down syndrome and transient myeloproliferative disorder: A case report and review of the literature.

Transient myeloproliferative disorder (TMD) is a spontaneously resolving clonal myeloid proliferation characterized by circulating megakaryoblasts in the peripheral blood that is restricted to neonates with Down syndrome (DS) or those with trisomy 21 mosaicism. Cutaneous manifestations of TMD are observed in only 5% of affected neonates and present as a diffuse eruption of erythematous, crusted papules, papulovesicles, and pustules, often with prominent and initial facial involvement. We describe the case of a male infant with DS and TMD, associated with a vesiculopustular eruption, which appeared on day 36 of life, and review previous cases.

Mantle cell lymphoma presenting with exaggerated skin reaction to insect bites.

We present the case of a 62-year-old African-American woman with medical history of hypertension and hyperlipidaemia who presented to dermatology clinic for 'bug bites'. Skin examination showed resolving bullae on the shins and postinflammatory pigment changes. Histopathology showed eosinophilic spongiosis and direct immunofluorescence (DIF) was negative for IgG, IgM, IgA and C3. After returning to clinic with recurrent severe bullous eruptions, the patient presented with anaemia, lymphocytosis, posterior cervical lymphadenopathy and weight loss. An exuberant bite reaction in the setting of lymphoma was suspected. Further workup with haematology revealed elevated IgG level and total protein levels. Flow cytometry showed a B cell lymphoma subtype. Extensive imaging was positive for diffuse lymphadenopathy, with accompanying evidence of Ebstein-Barr virus infection. Our case highlights the importance of considering exuberant arthropod bite reaction in the setting of undiagnosed lymphoma in a patient with bullous eruption and negative DIF.

Bullous lupus erythematosus with an erythema gyratum repens-like pattern.

Bullous lupus erythematosus is a rare clinical form of lupus. The diagnosis is challenging and involves the exclusion of other subepidermal bullous dermatoses. We present a 21-year-old woman with erythematosus, polycyclic plaques with vesiculobullae along the periphery, creating an erythema gyratum repens-like pattern on acral regions. The cutaneous biopsy, analytical, and autoimmune studies support the diagnosis of systemic lupus erythematosus. Dapsone and glucocorticosteroids were given with prompt resolution of the lesions within two weeks. To our knowledge this is the first case of bullous lupus erythematosus with this atypical acral presentation.

[Self-induced dermatosis on the shoulder]. / Une dermatose auto provoquée de l'épaule.

Self-induced dermatoses are more and more frequent in adolescent substance abusers with adaptation problems. We here report a case of self-induced bullous lesions on the shoulder in an adolescent with major depression and suicidal ideations. The study involved a 22-year old man, who was a chronic smoker, an occasional user of cannabis and alchool with no particular past medical history, with divorced parents, in conflict with his father. He presented with bullous eruption on the left shoulder made of bubbles, post-bullous skin erosions and scabs on a healthy skin. The monomorphic appearance of lesions, their accessibility (on the left shoulder in a right-handed patient), patient's history and his psychiatric problems have immediately suggested the investigation of self-induced dermatosis or factitious disorder. The patient admitted that he had taken psychotropic drugs and that the self-induced lesions had been caused by cigarette burns. This first psichologic evaluation also showed that the patient had suicidal ideations and a self-and hetero-aggressive behavior. The patient was treated with emollients and healing creams and referred to the Department of Psychiatry for complementary therapies.

Eosinophilic pustular folliculitis with underlying mantle cell lymphoma.

Eosinophilic pustular folliculitis (EPF) is a noninfectious condition characterized by folliculocentric papules, pustules, and plaques on the head, trunk, and extremities. Three subtypes of EPF have been described. Histopathology predominantly shows abundant eosinophils concentrated at the follicle, and treatment typically consists of topical corticosteroids or oral indomethacin. We present an unusual case of EPF in a 52-year-old man that preceded the diagnosis of mantle cell lymphoma.

Acquired bullous acrodermatitis enteropathica as a histologic mimic of pemphigus foliaceus in a patient on parenteral nutrition.

The original article was published on July19, 2017 and corrected on May 15, 2018. The revised version of the article includes a funding source for Dr. Maija Kiuru's participation in this case report, awarded by the National Cancer Institute, National Institutes of Health grant K12CA138464. This change appears in the revised online PDF copy of this article.

Treatment outcome and prognostic factors of tonsillectomy for palmoplantar pustulosis and pustulotic arthro-osteitis: A retrospective subjective and objective quantitative analysis of 138 patients.

Pustulosis palmaris et plantaris (PPP) and pustulotic arthro-osteitis (PAO) are tonsil-related diseases. Treatment outcome of tonsillectomy and prognostic factors influencing the outcome have not been analyzed quantitatively. We evaluated those using the Palmoplantar Pustulosis Area and Severity Index (PPPASI). At 1, 3, 6, 12, 24 and more than 24 months post-tonsillectomy, 20 (31%), 34 (48%), 70 (60%), 57 (80%), 36 (95%) and 23 (96%) patients realized 80% or more improvement of PPP skin lesions, respectively, and eight (17%), 23 (36%), 30 (50%), 38 (79%), 12 (100%) and four (100%) patients showed 80% or more improvement of PPPASI (i.e. PPPASI% ≥ 80%), respectively. At 1, 3, 6, 12 and more than 12 months post-tonsillectomy, 19 (73%), 21 (66%), 27 (73%), 19 (79%) and 15 (83%) patients realized a disappearance of PAO-induced arthralgia, respectively. Kaplan-Meier analysis of 80 patients with PPP revealed that, at 12 and 24 months post-tonsillectomy, lesions disappeared (i.e. PPPASI = 0) in 38% and 66% of patients, respectively, and lesions improved by 80% or more (i.e. PPPASI% ≥ 80%) in 71% and 95% of patients, respectively. The log-rank test and univariate and multivariate analyses showed that smoking cessation post-tonsillectomy and PAO were significant predictive factors for the early disappearance of skin lesions. This report is the first demonstrating objective evidence of the great efficacy of tonsillectomy to improve PPP skin lesions. Even post-tonsillectomy, smoking inhibited the early disappearance of the lesions.