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Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking.Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN.Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method.Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements.Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

Introduction: Scalp micropigmentation is a method of concealing alopecia by depositing permanent pigment in a tattoo-like manner. Pigment is deposited between hair follicles in a stippling pattern that resembles closely cut hair. Case Presentation: On trichoscopy, characteristic findings of scalp micropigmentation include homogenous, grey to black circular dots that are evenly spaced and appear larger than adjacent hair follicles. Findings were correlated with histopathology. Conclusion: Trichoscopy is a useful tool to visualize scalp micropigmentation in place of invasive procedures.

Lichen planopilaris (LPP) is a primary lymphocytic cicatricial alopecia for which therapy is often ineffective and there is no cure.Looking for a new targetable molecule in the treatment of LPP, we sought to verify whether IL-17 expression is increased in scalp biopsies from patients with active scalp lesions of LPP.Horizontal sections of hematoxylin and eosin-stained slides from 40 scalp biopsies of active LPP were retrospectively collected and stained with the monoclonal antibody against IL-17 (Abcam, Cambridge, MA; ab79056, dilution 1:100). Twenty biopsies from patients with chronic telogen effluvium served as controls because of their morphological resemblance to the normal scalp. Statistical analysis was performed using IBM SPSS Statistics for Windows (IBM Corporation, Armonk, NY).The main finding was the positive cytoplasmic expression of IL-17 in the perifollicular fibrosis of the affected follicles in LPP which was statistically significant compared with the controls ( P0.0001). The labeled cells were identified as fibroblasts based on their spindle shape and fascicular concentric arrangement in tight perifollicular distribution. Although most of the LPP specimens (n = 35; 87.5%) also revealed cytoplasmic IL-17 expression in the lichenoid inflammatory infiltrate, the results were not statistically significant ( P = 0.1351).Our immunohistochemistry results show that blocking the IL-17 inflammatory pathway may interfere with the progression of the perifollicular fibrosis and inflammation in LPP.

Papuloerythroderma of Ofuji (PEO) is a rare skin condition first described in 1984 and characterized by diffuse erythroderma composed of papules coalescing into plaques with sparing of skin folds, known as the deck-chair sign. The disease is almost exclusively seen in the elderly and affects men more frequently than women. Common laboratory findings include peripheral and tissue eosinophilia, elevated levels of immunoglobulin E, and lymphopenia. The diagnosis entails exclusion of potentially causative pathologies, including drug intake, atopy, malignancy, and infection. These factors have frequently been found in association with PEO, but their role in the etiopathogenesis of the disease is poorly understood. A dysregulated immune system, with particular involvement of T-helper (Th)2 and Th22 cells, seems to be important in the development of PEO. Controversy exists as to whether PEO exists as an independent entity or as a clinical pattern of a variety of distinct conditions. Treatment necessitates first addressing any coexisting circumstances that may have a causal relationship with PEO. In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.

T-cell prolymphocytic leukemia (TPLL) is a rare form of leukemia by T lymphocytes at a post-thymic intermediate stage of development with an α/β immunophenotype. Facial involvement is common in TPLL and displays significant heterogeneity of the lesions' description and location. TPLL also contains a wide array of histology findings, cell cytology, and molecular studies. Here, we describe a TPLL patient who presented with an ill-defined erythematous patch involving the right axilla progressing to the left axilla, upper back, and face that resembled dermatomyositis. The diagnosis of TPLL was established using flow cytometry of bone marrow and peripheral blood, and histopathology of the involved skin. Dermatologists should be aware of these unique features.

Prurigo nodularis (PN) is a chronic skin dermatosis with hyperkeratotic and intensely pruritic nodules. Managing PN-associated itch is difficult because its aetiology is still unknown. This study aimed to investigate the correlation between itch intensity in PN and the expression of a pruritogenic cytokine interleukin (IL)-31, its receptor complex components IL-31 receptor α (IL-31RA) and oncostatin M receptor β (OSMRβ), and oncostatin M (OSM), which is a ligand of OSMR β, through immunofluorescence staining examination. Itch intensity in PN was closely correlated with the number of dermal IL-31(+) cells (Spearman's r = 0.551, p 0.05), dermal IL-31RA(+) cells (r = 0.475, p 0.05) and dermal OSM(+) cells (r = 0.505, p 0.05). In addition, the number of dermal OSMRβ (+) cells was increased in PN (t test, p 0.05), despite not being correlated with itch intensity (Spearman's r = 0.375, p 0.05). Major cellular sources of dermal IL-31 were T cells (27.0% of total IL-31-expressing cells) and macrophages (35.0%), while those of OSM were mainly T cells (49.8%) and mast cells (26.8%). IL-31RA-expressing dermal cells were mostly mast cells (49.3%) and macrophages (36.6%), and OSMRβ was mainly expressed by macrophages (51.8%) in the dermis. These findings indicate that IL-31 (mainly from macrophages and T cells) and OSM (principally from T cells and mast cells) stimulate dermal cells expressing IL-31RA and OSMRβ (e.g. macrophages), which may further promote itch and inflammation in PN. This complex dermal milieu of cell/cytokine/receptor network can be a therapeutic target for PN-associated itch.

Introduction: Reticulated hyperpigmentation is a relatively uncommon dermatologic pattern. It is used to describe brown-colored skin lesions that manifest in a lacy or net-like distribution. When a clinician encounters a patient with reticulated hyperpigmentation, its location is often the most helpful feature in establishing a differential diagnosis. As this pattern is rarely observed on the scalp, this site is currently not included in the diagnostic approach. Case Presentation: In this report, we present a case of lichen planopilaris (LPP) in a black man who presented with reticulated hyperpigmentation over the scalp. Conclusion: We suggest that it may be warranted to add LPP to the differential diagnosis of reticulated hyperpigmentation, especially when arising on the scalp of darker skinned individuals.

Psoriasis is a chronic inflammatory disease affecting over 8 million Americans. Importantly, patients with psoriasis are at an increased risk of developing atherosclerosis, coronary artery disease, and myocardial infarctions. Several studies have suggested that psoriasis may be an independent risk factor for cardiovascular disease given their shared inflammatory properties and pathogenic similarities. Epicardial fat is also linked to cardiovascular disease and may be an independent risk factor for atherosclerosis. It has been proposed that measuring epicardial fat tissue may serve as a useful subclinical measure of cardiovascular disease in psoriasis patients. Echocardiography has been increasingly adopted as an accurate, minimally invasive, and cost-effective measure of determining the volume and thickness of epicardial fat. Using echocardiographic measures of epicardial fat thickness as a marker of cardiovascular disease and therapeutic target in psoriasis patients may provide clinicians with a means to better manage and hopefully prevent deleterious downstream effects.

Bubble hair deformity is an acquired abnormality characterized by air-filled cavity formations within the hair shaft, usually because of heat damage. Traditionally, bubble hair is diagnosed by visualization of characteristic bubbles under light microscopy. The increased utilization of trichoscopy (scalp dermoscopy) has led to its adoption in the diagnosis of many hair and scalp conditions, including bubble hair deformity. We sought to review clinical reports evaluating the patient profile, use of diagnostic imaging, and treatment options for bubble hair deformity. A systematic search of PubMed was performed in February 2021 using various keywords. Titles and abstracts were screened, leading to the selection of 11 case reports or series. The majority of patients were middle-aged Caucasian women who had used a heated tool to dry or style wet hair. Treatment consisted of cessation of heated tool usage. Light microscopy visualization of characteristic hair shaft cavities was used for diagnosis of bubble hair deformity in reports published prior to 2012. Diagnosis by trichoscopy was used in more recent reports. Our findings support the use of trichoscopy as a convenient and noninvasive method of diagnosing bubble hair deformity. More clinical studies are needed to evaluate the development of bubble hair deformity in ethnic hair.

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, neutrophilic, autoinflammatory skin disease characterised by recurrent flares of generalised sterile pustules and associated systemic features. Inconsistent diagnostic criteria and a lack of approved therapies pose serious challenges to GPP management. Our objectives were to discuss the challenges encountered in the care of patients with GPP and identify healthcare provider (HCP) educational needs and clinical practice gaps in GPP management.On 24 July 2020, 13 dermatologists from 10 countries (Brazil, Canada, China, Egypt, France, Germany, Japan, Malaysia, the UK and the USA) attended a workshop to share experiences in managing patients with GPP. Educational needs and clinical practice gaps grouped according to healthcare system level were discussed and ranked using interactive polling.Lack of experience of GPP among HCPs was identified as an important individual HCP-level clinical practice gap. Limited understanding of the presentation and pathogenesis of GPP among non-specialists means misdiagnosis is common, delaying referral and treatment. In countries where patients may present to general practitioners or emergency department HCPs, GPP is often mistaken for an infection. Among dermatologists who can accurately diagnose GPP, limited knowledge of treatments may necessitate referral to a colleague with more experience in GPP. At the organisational level, important needs identified were educating emergency department HCPs to recognise GPP as an autoinflammatory disease and improving communication, cooperation and definitions of roles within multidisciplinary teams supporting patients with GPP. At the regulatory level, robust clinical trial data, clear and consistent treatment guidelines and approved therapies were identified as high priorities.The educational imperative most consistently identified across the participating countries is for HCPs to understand that GPP can be life-threatening if appropriate treatment initiation is delayed, and to recognise when to refer patients to a colleague with more experience of GPP management.

A 64-year-old man was referred to our dermatology clinic with a diagnosis of Muir-Torre syndrome (MTS), he had a history of multiple sebaceous carcinomas and sebaceous adenomas removed over the years. The patient has also had visceral cancer and had undergone a colon resection 17 years before to treat colon cancer and was recently diagnosed with invasive high-grade urothelial carcinoma of the right ureter. In addition, the patient has an extensive family history of cancer; a pedigree was constructed to document this history (Figure 1). Of note is that the patient's mother and father were second cousins. The patient's father was diagnosed with lung cancer at age 57 and died of colon cancer at the age of 72. The patient's mother died of colon cancer at age 74. The patient has three siblings: a sister and two brothers. The sister died of bone cancer at age 42. One brother had a number of cancers including colon, kidney, and skin cancers and died at age 53. His other brother is alive and has a history of colon cancer, kidney cancer, and ureteral cancer. The patient has five children. He has a 40-year-old son who, at the age of 30, was diagnosed with testicular cancer. His daughters are 47, 44, 39, and 34, with no history of malignancy to date. The patient had three maternal aunts, all of whom succumbed to colon cancer, as well as two paternal uncles who died of lung cancer. The patient's maternal grandfather was a smoker and he also died of lung cancer.

Drug survival analysis of biologic agents in psoriasis is of extreme importance, as it allows not only the evaluation of objective clinical outcomes (such as effectiveness and safety) but also of factors that are associated with patients’ adherence to treatment. The aim of this study was to evaluate and compare the drug survival of the most recent biologic agents approved for the treatment of moderate-to-severe psoriasis—ustekinumab, secukinumab, ixekizumab, brodalumab, guselkumab, and risankizumab—and to identify clinical predictors that can influence the drug survival of these drugs. This retrospective multicentric cohort study from 16 dermatology centers in Portugal, Spain, Italy, Switzerland, Czech Republic, Canada, and the United States included patients that started IL-12/23, IL-17 (IL-17A and IL-17R) and IL-23 inhibitors for the treatment of psoriasis between January 1, 2012 and December 31, 2019. Survival analysis was performed using a Kaplan-Meier estimator, to obtain descriptive survival curves, and proportional hazard Cox regression models. A total of 3312 treatment courses (total patients: 3145) were included in the study; 1118 (33.8%) with an IL-12/23 inhibitor (ustekinumab), 1678 (50.7%) with an IL-17 inhibitor [911 (27.5%) on secukinumab, 651 (19.7%) on ixekizumab, 116 (3.5%) on brodalumab], and 516 (15.5%) with an IL-23 inhibitor [398 (12.0%) on guselkumab, 118 (3.5%) on risankizumab]. At 18 months, the cumulative probability of survival was 96.4% for risankizumab, 91.1% for guselkumab, 86.3% for brodalumab, 86.1% for ustekinumab, 82.0% for ixekizumab, and 79.9% for secukinumab. Using ustekinumab as reference, drug survival of guselkumab was higher (HR 0.609; 95% CI 0.418–0.887) and that of secukinumab was lower (HR 1.490; 95% CI 1.257–1.766). In the final multivariable model, secukinumab, female sex, higher BMI, and prior exposure to biologic agents significantly increased the risk of drug discontinuation, whereas risankizumab was protective. In this multinational cohort with 8439 patient-years of follow-up, the cumulative probability of drug survival for all drugs was >79% at 18 months. Prescribed biologic, female sex, higher BMI, and previous exposure to biologic agents were predictors of drug discontinuation. Drug survival of guselkumab and risankizumab was higher than that of ustekinumab, and secukinumab was lower.

Frontal Fibrosing Alopecia (FFA) is a chronic lymphocytic cicatricial alopecia that usually involves the fronto-temporal hairline and the eyebrows in women but other presentations such as involvement of the sideburns, occipital scalp, eyelashes and body hair are also possible. Further, its presentation comes in typical and atypical patterns. FFA can be associated with lichen planus pigmentosus, facial papules, red dots, prominent veins, and increases preauricular wrinkles. It is of unknown etiology and there is no cure. Recent works have suggested association with leave in cosmetic products such as sunscreens, and possible sensitization due to cosmetic ingredients. Histopathologic and trichoscopic findings are the mainstay in obtaining an accurate diagnosis. Treatment and prognosis vary depending on severity at presentation, however, reduced progression with treatment has been demonstrated in most patients within a year.

Lichen Planus (LP), the prototype of lichenoid dermatoses, is an idiopathic, T cell-mediated, autoimmune, inflammatory disease. It may affect the skin, hair, nails, and mucous membranes. Many clinical variants of LP have been described, including lichenoid drug eruption or drug induced LP, associated with a myriad of culprit medications. We describe a 63-year-old woman with longstanding psoriasis effectively controlled with ixekizumab, who developed lichenoid drug eruption . Her lichen planus lesions improved after treatment discontinuation and the patient was started on an IL23 inhibitor to treat her psoriasis through an alternative mechanism of action. Our report adds to the literature and provides insight into the complex pathophysiology of lichen planus.

Cutaneous mucinoses are a heterogeneous group of disorders having as a common denominator the deposition of an excess of mucin (acid glycosaminoglycans, mostly hyaluronic acid) in the dermis or in the hair follicles. They are divided into primary and secondary forms. Primary cutaneous mucinoses are characterized by specific clinical lesions in which mucin deposition is the distinctive histological sign, while in secondary mucinoses, mucin deposition is simply an accessory histological sign. Disorders of collagen and elastic fibers are divided into hereditary and acquired forms. Both forms can be further divided into those diseases characterized by an increase and those by a decrease in collagen and elastic tissue. A peculiar variant of disorders of dermal connective tissue includes the primary perforating dermatoses.

Soft tissue amyloidoma is a rare condition that presents primarily in the abdomen and/or mediastinum and more uncommonly on the extremities. Soft tissue amyloidomas on the extremities have been associated with chronic inflammation, particularly when accompanied by AA-type amyloid deposition as seen in local trauma, surgery, hypertension and diabetes. To our knowledge, this is the first reported case of nodular cutaneous amyloidoma in the setting of systemic and cutaneous sarcoidosis. A 65-year-old woman presented with an asymptomatic subcutaneous nodule above her left lateral malleolus. Histopathology of the lesion showed an inconspicuous epidermis with amorphous eosinophilic material deposited in masses within the entire dermis. Congo red and crystal violet stains were positive. Based on the clinical and pathologic findings she was diagnosed with nodular cutaneous amyloidoma. We hypothesize that this process developed secondary to the chronic granulomatous inflammation of sarcoidosis.

Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. Each subtype can present as a solitary lesion or multiple lesions. They could present as part of systemic diseases or inherited disorders. This article provides a comprehensive literature review of the different types of CTN, their clinical presentations, associations, and treatment options. Treatment options for 56 lesions were reviewed. Fifty-two percent of lesions were present in males, and the age range at the time of presentation was wide (1.6-80 years). Management varied according to CTN subtypes. Most lesions (14) received topical or intralesional treatment with corticosteroids, followed by surgical removal of lesions (12), whereas the remaining lesions were clinically monitored.

Scalp morphea presents as a scarring alopecia in en coup du sabre pattern. We report an unusual presentation of a round hairless patch of morphea on the occipital scalp present for 15 years. The scalp lesion aligned with 2 other hyperpigmented lesions of biopsy-proven morphea in the lower back. Pathology of horizontal sections from the scalp lesion showed follicular dropout, thickening of the collagen bundles, and preserved eccrine and follicular structures. Marked lymphocytic perineural infiltrate, a reported clue to the diagnosis of scalp morphea, contributed to the diagnosis. This case is unusual due to its rare clinical presentation. It also highlights the importance of recognizing histopathological clues for the diagnosis of uncommon scalp disorders.

Carcinosarcomas are rare malignant tumors derived of both epithelial and mesenchymal elements. Herein, we report an elderly man originally diagnosed with a squamous cell carcinoma of the hand. Upon excision, the tumor was found to be a more aggressive carcinosarcoma. Immunohistochemical stains revealed that the sarcoma component of the lesion was vimentin positive, whereas the primary carcinoma tumor cells were positive for p63 and CK903. Both components were negative for CD34 and D2-40. This tumor was found to have angiolymphatic invasion and eventually metastasized to the axillary lymph nodes and lungs.

Carcinosarcomas are rare malignant tumors derived of both epithelial and mesenchymal elements. Herein, we report an elderly man originally diagnosed with a squamous cell carcinoma of the hand. Upon excision, the tumor was found to be a more aggressive carcinosarcoma. Immunohistochemical stains revealed that the sarcoma component of the lesion was vimentin positive, whereas the primary carcinoma tumor cells were positive for p63 and CK903. Both components were negative for CD34 and D2-40. This tumor was found to have angiolymphatic invasion and eventually metastasized to the axillary lymph nodes and lungs.

Primary cutaneous diffuse large B-cell lymphoma is an uncommon and aggressive lymphoproliferative disorder with a rapid growth rate and dismal prognosis. We present the case of a 91-year-old female with an unusual manifestation of primary cutaneous diffuse large B-cell lymphoma, mimicking other more prevalent diseases like chronic non-healing venous ulceration. Dermatopathologic evaluation rendered the correct diagnosis. A discussion of this rare presentation is important for clinician consideration to prevent misdiagnosis and prolongation of proper management in patients with chronic non-healing leg ulcers.

Clear cell acanthoma (CCA) is a rare, benign cutaneous condition most often seen on the lower extremities. Lesions are of variable morphology and have been described as polypoid, pigmented, giant, and cystic lesions. Although no racial or gender predilection has been reported, CCA on the breast is a very rare finding that has been observed mainly in young Korean women. Herein, we describe a case of CCA of the areola in an elderly woman with metastatic renal cell carcinoma. Physical exam revealed a pink plaque with central erosions on the left areola. Given the concern for cutaneous metastasis, excisional biopsy was performed, which showed pale glycogenated epithelium consistent with CCA. No evidence of recurrence or new lesions was observed after 6 months of follow-up. Our case exemplifies that clinicians should consider CCA in the differential diagnosis for a new eczematous lesion involving the breast, even in the setting of malignancy.

Clear cell acanthoma (CCA) is a rare, benign cutaneous condition most often seen on the lower extremities. Lesions are of variable morphology and have been described as polypoid, pigmented, giant, and cystic lesions. Although no racial or gender predilection has been reported, CCA on the breast is a very rare finding that has been observed mainly in young Korean women. Herein, we describe a case of CCA of the areola in an elderly woman with metastatic renal cell carcinoma. Physical exam revealed a pink plaque with central erosions on the left areola. Given the concern for cutaneous metastasis, excisional biopsy was performed, which showed pale glycogenated epithelium consistent with CCA. No evidence of recurrence or new lesions was observed after 6 months of follow-up. Our case exemplifies that clinicians should consider CCA in the differential diagnosis for a new eczematous lesion involving the breast, even in the setting of malignancy.

Multiple Sclerosis (MS) is a chronic autoimmune disease that presents with a wide variety of sensory and motor deficiencies. New medications targeting B cells have been approved to treat MS, but the side effect profile has not been widely explored. Herein, we report a case of drug-induced psoriasiform dermatitis following ocrelizumab treatment. Physicians should be cognizant of this possible side effect in patients receiving treatment for MS.

Venous leg ulcers (VLUs) have higher tumor necrosis factor-α (TNF-α) levels compared with normal skin. Refractory VLUs of long duration have higher TNF-α levels compared with VLUs of shorter duration. As up to 75% of VLUs fail to heal with standard care, we sought to evaluate the role of anti-TNF-α therapy for patients with refractory VLUs. Evaluable data were obtained in four of five subjects with recalcitrant VLUs treated with 80 mg of subcutaneous adalimumab at week 0 and with 40 mg at week 2 along with compression therapy and were followed-up for 6 weeks. Wound biopsies taken at weeks 0 and 4 were stained with anti-TNF-α antibodies. Average 4-week percent wound size reduction was 20.5% ± 6.4%. Two patients had wound size reduction more than 25%, and their percent wound size reduction correlated to percent TNF-α staining score reductions (P = 0.02, R(2) = 0.999). VLU TNF-α level decrease 4 weeks post-adalimumab treatment correlated with wound healing.

Chromoblastomycosis (CBM) is a slowly progressive cutaneous and subcutaneous mycosis mostly seen in tropical and subtropical areas and Fonsecaea pedrosoi is the most common cause. The authors describe the case of a diabetic Haitian woman, presenting with a chronic verrucous plaque without any history of trauma. Her histopathologic results showed epidermal hyperplasia and sclerotic bodies, which are diagnostic for CBM. Her therapy began with itraconazole 200 mg tablets twice a day. The unique feature of this patient is the coincidence of diabetes and CBM. However, to the best of our knowledge, this is the first documented case of human CBM in Miami, FL, which develops the awareness regarding this diagnosis among doctors in this area. There should be a close communication between dermatologists and pathologists to make an early diagnosis of CBM and also adequate therapy, which both are fundamental to improve patient's quality of life.

Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis that can be associated with malignancy and medications. A 60-year-old man presented with erythematous, edematous, and ulcerated plaques in the extensor surface of the upper extremities, after a ketoconazole course due to pityrosporum folliculitis. Skin biopsy showed a dense dermal neutrophilic infiltrate, associated with marked papillary dermal edema. Blood count showed leukocytosis and neutrophilia. Skin lesions resolved spontaneously with discoloration after 2 weeks of discontinuation of ketoconazole. Although most cases of drug-induced SS are associated with granulocyte colony-stimulating factor, other medications need to be considered. This is the first reported case of ketoconazole-induced SS despite its widespread use.

Tyrosine kinase inhibitors have revolutionized the chemotherapy arena as targeted therapies for a multitude of malignancies. They are more selective than conventional chemotherapy, and often elicit fewer systemic adverse events, however toxicities still exist. Cutaneous toxicities are common and their management presents a novel challenge to physicians and patients. Ponatinib is a third-generation tyrosine kinase inhibitor increasingly reported to cause cutaneous eruption. A 50-year-old woman with a history of chronic myelogenous leukemia presented with a 4-month history of worsening atrophic and ichthyosiform pink plaques involving the axillae, thighs and abdomen; red patches were also observed on the cheeks and forehead. She was started on the third-generation, ponatinib, 5 months earlier because of disease refractory to previous therapies including interferon, imatinib, dasatinib and bosutinib. A skin biopsy revealed perifollicular fibrosis, alternating orthokeratosis and parakeratosis, and a sparse perivascular lymphocytic infiltrate consistent with a pityriasis rubra pilaris-like reaction. Topical tretinoin 0.025% cream was initiated, resulting in resolution within 3 weeks without discontinuation of ponatinib. A review of previous reports identified significant similarities among the ponatinib-induced drug reactions. Here, we highlight not only that cutaneous eruptions occur on ponatinib therapy, but that the dermatologic manifestations are characteristic and unique, and benefit from retinoid therapy, without requiring interruption of vital chemotherapy. Electronic supplementary material The online version of this article (doi:10.1007/s40800-017-0055-y) contains supplementary material, which is available to authorized users.

A 50-year-old diabetic woman developed multiple abscesses after daily injections of subcutaneous regular human insulin. Initial cultures from the draining fluid revealed no organisms, and the patient was unresponsive to multiple courses of antibiotics including doxycycline, amoxicillin/clavulanic acid, and intravenous vancomycin and piperacillin/tazobactam. After consulting dermatology, the patient underwent punch biopsy and tissue culture. The tissue culture grew mycobacterium fortuitum after 22 days of incubation. The patient was diagnosed with non-tuberculous mycobacterial infection and began to improve after treatment with ciprofloxacin. Non-tuberculous mycobacteria are common in the environment, although systemic manifestations of infection are rare in healthy individuals. In this paper, we discuss the risk factors, diagnostic methods, and treatment recommendations for these types of infections.

The rough coat (rc) spontaneous mutation causes sebaceous gland hypertrophy, hair loss and extracutaneous abnormalities including growth retardation. The rc mice have a missense mutation in the predicted immunoglobulin protein Mpzl3. In this study, we generated Mpzl3 knockout mice to determine its functions in the skin. Homozygous Mpzl3 knockout mice showed unkempt and greasy hair coat and hair loss soon after birth. Histological analysis revealed severe sebaceous gland hypertrophy and increased dermal thickness, but did not detect significant changes in the hair cycle. Mpzl3 null mice frequently developed inflammatory skin lesions; however, the early onset skin abnormalities were not the results of immune defects. The abnormalities in the Mpzl3 knockout mice resemble closely those observed in the rc/rc mice, as well as mice heterozygous for both the rc and Mpzl3 knockout alleles, indicating that rc and Mpzl3 are allelic. Using a lacZ reporter gene, we detected Mpzl3 promoter activity in the companion layer and inner root sheath of the hair follicle, sebaceous gland, and epidermis. Loss of MPZL3 function also caused a striking reduction in cutaneous and overall adipose tissue. These data reveal a complex role for Mpzl3 in the control of skin development, hair growth and adipose cell functions.

Pigmented casts have been reported with variable frequency in scalp biopsies from alopecia areata, trichotillomania, chemotherapy-induced alopecia and postoperative (pressure induced) alopecia. Their presence and morphology in other scalp disorders has not been described. The authors assessed for the presence and morphology of pigmented casts in 308 transversely bisected scalp biopsies from nonscarring and scarring alopecia, referred to the Department of Dermatology, University of Miami within a year. The pigmented casts were present in 21 of 29 cases of alopecia areata (72%), 7 of 7 cases of trichotillomania (100%), 1 case of friction alopecia, 4 of 28 cases of central centrifugal cicatricial alopecia (14%), and 4 of 4 cases of dissecting cellulitis (100%). They did not show any distinguishing features except for the morphology in trichotillomania, which included twisted, linear (zip), and "button"-like pigment aggregation. The linear arrangement was found also in friction alopecia and dissecting cellulitis. Pigmented casts in the hair canals of miniaturized/vellus hairs was a clue to alopecia areata. Pigmented casts can be observed in biopsies of different hair disorders, but they are not specific for the diagnosis. Horizontal sections allow to better assess their morphology and the follicular level of presence of pigmented casts, which in the context of the other follicular findings may be a clue to the diagnosis.

Summary Background Post-kala-azar dermal leishmaniasis (PKDL) is an uncommon complication of visceral leishmaniasis (VL) but is emerging as an increasingly frequent and serious complication of acquired immunodeficiency syndrome (AIDS). It manifests as a macular, morbilliform, or nodular eruption in a patient who has recovered from VL. Methods We present two cases of PKDL in the setting of AIDS. Results These two cases are notable because they demonstrate the severe course of VL, the rare appearance of PKDL in the USA, and the recurrence of disease after >2 years of remission in the second case. Options for treatment include amphotericin B and pentavalent antimonials along with first-line antiretroviral therapy. Conclusions The prevention of VL relapses, and the development of PKDL in HIV patients with Leishmania co-infection remains challenging. The vast differential diagnosis of other HIV-related cutaneous conditions, along with a lack of experience with this disease, often delays diagnosis. Therapeutic dilemmas concerning drug selection, dosage, scheduling regimen, and the respective durations of initial and maintenance therapy for PKDL need to be addressed. Treatment should aim to target the latent infection and prevent posttreatment VL relapses that may present in an unpredictable fashion. Leishmaniasis remains a recognized but uncommon opportunistic disease in the setting of HIV, which can be associated with atypical features including the appearance of skin lesions years after the treatment of VL.

Primary cutaneous diffuse large B‐cell lymphoma is an uncommon and aggressive lymphoproliferative disorder with a rapid growth rate and dismal prognosis. We present the case of a 91‐year‐old female with an unusual manifestation of primary cutaneous diffuse large B‐cell lymphoma, mimicking other more prevalent diseases like chronic non‐healing venous ulceration. Dermatopathologic evaluation rendered the correct diagnosis. A discussion of this rare presentation is important for clinician consideration to prevent misdiagnosis and prolongation of proper management in patients with chronic non‐healing leg ulcers.

Summary Background Esthetic augmentation of the gluteal region can lead to complications including debilitating pain, infections, wounds, and scars. To our knowledge, a general consensus of staging and treatment guidelines for managing gluteal foreign body reaction to injectables has not yet been established. Objective The objective of this study was to develop a reliable staging system that can be used to implement a treatment algorithm for gluteal foreign body reactions. Methods A retrospective review of 40 patients treated for complications of gluteal injections between September 2010 and May 2014 was performed. Patient symptoms, imaging, and photographs were used to develop a staging system of disease. Institutional review board approval was obtained from the University of Miami Miller School of Medicine. Five independent observers reviewed the patients' documented symptoms and photographs. Using our staging system, the independent observers reviewed the patient cases at two separate time intervals. Intra- and interclass correlation coefficients (ICCs) were computed to assess the reliability for each of the observers. Results Seven patients were classified as Stage I, fifteen as Stage IIa, nine as Stage IIb, and nine as Stage III. The mean patient age was 34 years (21–50). Analysis of the independent reviewer results revealed ICC for each rater to range from 0.96 to 0.98, demonstrating high indexes of intra-rater reliability. Conclusions Based on our statistical analysis, we found an excellent inter- and intra-observer reliability, indicating that the staging system is reproducible and reliable. A treatment strategy dependent on the stage can be implemented as a guideline to optimize functional and esthetic outcomes.