A recalcitrant case of folliculitis decalvans: imaging and treatment options

Folliculitis decalvans (FD) is a rare form of scarring alopecia, presenting with tufted hairs, follicular papules or pustules. It is the most common of neutrophilic alopecia, often associated with pruritus and pain. Currently, the cause of FD is still unknown. Scalp colonization by staphylococcus aureus seems to induce a severe inflammatory reaction with consequent destruction of hair follicles. All patients with active FD should be treated because the disease destroys hair follicles and causes permanent hair loss. Multiple treatments exist, but often provide only transitory or modest improvement. For this reason FD can negatively affect patient’s quality of life. A 46-year-old man presented with 15-year history of FD characterized by recurrent purulent follicular lesion on occipital side of the scalp and consequent scarring alopecia. His medical history was unremarkable, but he was distressed by the appearance of the lesions and hair loss. He was treated with different therapies such as doxycycline, rifampicin and hydroxychloroquine with poor results. He underwent two sessions of photodynamic treatment with methyl aminolevulinate at 2-week interval. At first, he was achieved benefit, but after 2 months there was recurrence and further progression of the disease. Based on literature reports, we decided to treat with long-pulse ND:Yag laser. We started at 30 J/cm2 and then we improved dose until 80 J/cm2. A total of 7 treatments in an 8-12 weeks inter-treatment interval were performed with successfully outcome, without relapse for more than 18 months of follow up. We monitored scalp inflammation with reflectance confocal microscopy (RCM) and optical coherence tomography (OCT) during patient’s treatment. Both technologies represent non-invasive diagnostic tool and their application on inflammatory skin diseases has increased in the last years. ND:Yag laser is useful therapy option in the treatment of recalcitrant FD. Our experience allowed us to monitor the evolution of inflammatory reaction, confirmed treatment’s efficacy over the clinical examination

Pseudoxanthoma elasticum and reflectance confocal microscopy: report of two affected young sisters

Pseudoxanthoma elasticum (PXE) is a rare inherited multisystem disorder that mainly affects skin, eyes and cardiovascular system. The associated clinical signs are due to progressive calcification of elastic fibres and blood vessels, despite normal levels of calcium and phosphorus in blood and urine. The first clinical description of the disease was done in 1881 by Rigal, and in 1896 it was named PXE by Darier. Transmission of the disease is autosomal recessive. PXE is caused by homozygous or compound heterozygous mutations in the ATP-binding cassette subfamily C member 6 (ABCC6) gene, which encodes a transmembrane transport ADP-dependent protein (MRP6). The gene is expressed predominantly in the liver and kidney, and found in low level in the tissue involved by PXE. The clinical expression of PXE is heterogeneous with considerable variation in age of onset, progression and severity of the disease, even in individuals of the same family with identical mutations. We present the case of two young sisters affected by PXE and the correlation between the histopathology and the reflectance confocal microscopy (RCM). Parents and brother carry one copy of the mutated gene, without showing signs and symptoms of the disorder. We report the main clinical aspects of PXE and we highlight the importance of early diagnosis of the disease for adequate therapeutical management of associated complications

Eccrine poroma: dermoscopical and confocal features of five cases

Background: Eccrine poroma (EP) is a rare benign adnexal tumor arising from the intraepidermal ductal portion of the sweat gland. It commonly occurs as a single, slowly growing, erythematous, skin-colored, cyanotic or pigmented papule, plaques or nodule. EP occurs usually at the acral sites, but it can develop in other cutaneous sites. It may occasionally mimic malignant tumors including basal cell carcinoma, squamous cell carcinoma and melanoma. Objective: The aim of this study was to describe dermoscopical and confocal features of EP. Methods: A retrospective analysis of the dermoscopical and confocal characteristics of EP was performed. All diagnosis were confirmed by histological examination. Results: A total of 5 cases of non-pigmented EP was analysed. Dermoscopic evaluation found in all lesions a polymorphous vascular pattern, including at least two type of vessels: hairpin (80%), linear (60%), leaf-like (60%), flower-like (40%) and glomerular (40%) vessels. A white-to-pink halo surrounding the vessels was found in 40% of the lesions. Multiple pink-white structureless areas were found in 4 out of 5 (80%) cases. Only in 2 cases irregular haemorrhagic and blue-white areas were also observed. Reflectance Confocal Microscopy (RCM) revealed the presence of well-demarcated hyporefractile tumor nests, dark holes corresponding to areas of ductal differentiation within the tumor and highly vascularized stroma in all 5 lesions. Conclusions: The great clinical variability of EP gives reason of the appellative of “big simulator”. Dermoscopy does not revealed univocal features except from “leaf-flower-like” vessels that have not been described in other types of skin tumors. This characteristic, when presents, may be considered an useful clue for the diagnosis. RCM examination of EP revealed features (hyporefractile tumor nests and dark holes) that relate with their histopathological findings. Dermoscopy and RCM improve the diagnostic accuracy and help for diagnosis, although they cannot replace histology that is still required

Congenital Glioblastoma multiforme and eruptive disseminated Spitz nevi

Background: Glioblastoma multiforme (GBM) is the deadliest malignant primary brain tumor in adults. GBM develops primarily in the cerebral hemispheres but can develop in other parts of the central nervous system. Its congenital variant is a very rare disease with few cases described in literature. Case presentation: We describe the case of a patient with congenital GBM who developed eruptive disseminated Spitz nevi (EDSN) after chemotherapy. Few cases of EDSN have been described in literature and this rare clinical variant, which occurs predominantly in adults, is characterized by multiple Spitz nevi in the trunk, buttocks, elbows and knees. There is no satisfactory treatment for EDSN and the best therapeutic choice is considered the clinical observation of melanocytic lesions. Conclusion: We recommend a close follow-up of these patients with clinical observation, dermoscopy and reflectance confocal microscopy (RCM). However, we suggest a surgical excision of the lesions suspected of being malignant

Utilizzo della tomografia ottica computerizzata (SV-OCT) per la valutazione del coinvolgimento ungueale nell’acrodermatite continua di Hallopeau

Recentemente è stato proposto l’utilizzo della Tomografia Ottica Computerizzata (SV-OCT) nella valutazione delle lesioni psoriasiche. Tale metodica è in grado evidenziare in modo accurato alterazioni cutanee ed ungueali clinicamente non valutabili. Presentiamo la nostra esperienza preliminare nell’utilizzo della SV-OCT in una donna di 75 anni affetta da una grave Acrodermatite continua di Hallopeau delle mani. Le valutazioni cliniche e strumentali sono state eseguite al basale e dopo 4 settimane di trattamento con 40 mg di adalimumab ogni 2 settimane e 25 mg di prednisone/die. Prima del trattamento le dita delle mani mostravano una grave onicodistrofia con pustole dolorose che coinvolgevano il letto ungueale, circondate da aree di intenso eritema ed ipercheratosi. SV-OCT evidenziava un marcato ispessimento ed irregolarità dello strato superficiale e ventrale della lamina ungueale; inoltre, si osservavano alcune aree iperriflettenti. L’analisi delle immagini mostrava la presenza di diffuse aree rosse nel letto ungueale, corrispondenti alla flogosi. Dopo 4 settimane di terapia abbiamo osservato la scomparsa delle pustole con una iniziale ricrescita delle unghie. SV-OCT mostrava un aspetto regolare dello strato superficiale e ventrale della lamina ungueale ed una marcata riduzione delle aree rosse corrispondenti all’infiammazione

The combination of oral and topical photoprotection with a standardized Polypodium leucotomos extract is beneficial against actinic keratosis

IntroductionThis study describes a prospective, multicentre, randomized controlled, open-label study with three arms aimed at studying the differences between: [Cnt], self-administered sun protection; [T], topical treatment; and [TO], topical + oral treatment; for the management of Actinic Keratosis (AK) in a cohort of subjects of advanced age displaying severe actinic damage (SAD). MethodsTreatments administered to groups [T] and [TO] had a common component, which is a botanical extract, Fernblock, with demonstrated photoprotective activity. ResultsIn total, 131 subjects were distributed randomly in the three groups, and followed up clinically at three separate time points, beginning of the study (t = 0) and after 6 and 12 months. Analysis of clinical data and examination using reflectance confocal microscopy (RCM) revealed that group [T] and [TO] displayed decreased clinical AK and field cancerization parameters, including the number of new lesions, and reduced the need for additional interventions in these patients. RCM revealed normalization of the keratinocyte layer. Improvements in AK and field cancerization parameters were greatest in the group [TO], suggesting that topical and oral photoprotection improves the clinical and anatomical outcome compared to control conditions. ConclusionsThe combination of topical and oral immune photoprotection provides an advantage compared to topical photoprotection alone

Uso della tomografia a coerenza ottica nella valutazione dell’efficacia del trattamento in paziente con acrodermatite di Hallopeau

Si presenta il caso di una donna di 75 anni affetta da una grave forma di acrodermatite di Hallopeau delle mani trattata con 40 mg di adalimumab ogni 2 settimane e 25 mg di prednisone al giorno. Prima del trattamento le dita mostravano una grave onicodistrofia con pustole dolorose che coinvolgevano il letto ungueale, circondate da aree di intenso eritema ed ipercheratosi. La tomografia a coerenza ottica evidenziava marcato ispessimento della lamina ungueale ed irregolarità degli strati dorsale e ventrale; inoltre, si osservavano alcune aree iperriflettenti. L’analisi delle immagini mostrava la presenza di diffuse aree rosse nel letto ungueale corrispondenti alla flogosi. Dopo 4 settimane dall’inizio della terapia si è osservata la scomparsa delle pustole con un’iniziale ricrescita delle unghie. La tomografia a coerenza ottica mostrava un aspetto regolare degli strati superficiale e ventrale della lamina ungueale ed una marcata riduzione delle aree rosse corrispondenti all’infiammazione

“Lambs” in wolves’ clothing: when basal cell carcinoma mimics melanoma, but it is detected by the use of reflectance confocal microscopy

Background Basal cell carcinoma (BCC) is the most frequent non-melanoma skin cancer. There are some cases in which clinical and dermoscopic examinations do not allow to formulate a unique diagnosis and in particular can be difficult the differential diagnosis with melanoma. Reflectance confocal microscopy (RCM) is a non-invasive technology, which allows an in vivo imaging of the skin with high resolution. Objectives We addressed our research to evaluate the reliability of the well-known RCM criteria for classic BCCs in a group of lesions with atypical dermoscopy presentation, possibly mimicking melanoma. Methods We retrospectively analyzed at RCM excised lesions presenting in dermoscopy ≥1 score at revisited 7-point checklist. The study population consisted of 177 cases showing no melanocytic RCM findings. Lesions were investigated for distinct non-melanocytic RCM features, while blinded from histopathology. Histopathology matching was performed before statistical analysis. Results Among the lesions classified at RCM with no-melanocytic characteristics, we recognized 34 cases, histopathological confirmed as BCCs (21 nodular BCCs and 13 superficial BCCs) and 143 cases classified as other lesions (DFs, SebKs, SCCs and others). The main features of nBCCs (with histopathological confirm) at RCM are peritumoral clefts (20/21 95,2%; p=0,037), peripheral palisading (19/21 90,5%; p=0,001), increased vascularization (20/21 95,2%; p=0,004). In sBCCs we found mild keratinocytic atypia (13/13 100%; p=<0,001), solar elastosis (12/13 92,3%; p=0,002), cords connected to epidermidis (9/13 69,2%; p=<0,001). Dendritic structures, nests of basaloid cells, inflammatory infiltrate can be seen in all tumors. Conclusions RCM classification proved high agreement with histopathology for BCCs with atypical dermoscopy presentations, allowing an early differential diagnosis and even identification of BCCs subtypes. RCM features in this group of lesions were similar to those described for typical cases of BCCs, and may drive clinicians decisions, helped them in the recognition of melanocytic and non melanocytic lesions, increasing the rate of accurate diagnoses and allowing better therapeutic management

Organ culture and Reflectance Confocal Microscopy as new integrated tools for barrier rescue studies in inflammatory skin diseases

Here we present a new integrated approach to understand skin barrier recovery after physical (tape stripping, TS) or chemical (SDS) injury by combining human skin organ culture and Reflectance Confocal Microscopy (RCM). TS in vitro produced a complete removal of stratum corneum and lipids, a drastic decrease of structural and adhesion proteins, and an increase in cell proliferation. Epidermal recovery with either proliferation or differentiation rescue was observed after 18 hours, with no apoptotic cell detection. On the other hand, when skin organ cultures were exposed to 2% SDS, cellular junctions were disrupted and the expression of late differentiation markers decreased. Junctions repair was detected 24 hours after treatment, with the restoration of epidermal integrity. Both models (TP or SDS) showed the induction of immune-inflammatory markers, such as psoriasin, keratin 16, and the increase in Langerhans cell number. RCM confirmed all the morphological and structural features presented by the organ cultures, thus making this technique fast and easily applicable in the context of dermatological research. These results indicate that combination of skin organ models and RCM can be successfully used for the study of barrier perturbation in skin diseases, for toxicology tests, and for evaluating novel therapies

Reflectance confocal microscopy and optical coherence tomography for the diagnosis of bullous pemphigoid and pemphigus

Introduction & Objectives: Bullous pemphigoid (BP) and pemphigus (P) are autoimmune diseases characterized by the presence of blisters on the skin and/or the mucous membranes. The diagnosis of these bullous diseases is based on a combination of criteria encompassing clinical features, histology, immunofluorescence and laboratory data. The aim of this study was to evaluate features of BP and P at reflectance confocal microscopy (RCM) and optical coherence tomography (OCT) in order to provide a rapid non-invasive bed-side diagnosis. Secondary objective was to evaluate the detectability of clinically non-visible lesions. Material & Methods: This was an observational, retrospective, multicentre study (University of Modena, Italy and University of Saint-Etienne, France) in which patients with suspicious lesions for BP or P underwent clinical assessment, RCM, OCT, blood tests and skin biopsy for histological and direct immunofluorescence examinations. A total of 72 lesions in 24 patients (16 with PB and 8 with P) were evaluated. Apparently unaffected skin was examined in order to test sub-clinical lesion detectability in all patients. Data analysis was performed from January 2014 to December 2015. Results: RCM was able to detect sub-epidermal and intra-epidermal blisters respectively in 75% and 50% of the patients affected by BP and P. At OCT the exact blister level was identified in all BP and P cases’. Acantholytic cells were observed only at RCM in P (62.5%). Fibrin deposition inside the blisters was only found in PB, evidenced both at RCM and OCT. Subclinical bullae were revealed on clinically healthy skin at OCT in some cases of BP and P. Conclusions: RCM and/or OCT can assist the clinician in providing rapid information through a non-invasive procedure for a rapid diagnosis of BP and P. Combined use of RCM and OCT for a real-time examination of the skin lesions associates the higher resolution of RCM with the greater penetration depth in cross-sectional view of OCT, providing in vivo quasi-histologic information