Mycosis fungoides (MF) represent the most frequent form of cutaneous T-cell lymphoma (CTCL). Chlormethine gel has been approved as first-line therapy in MF. The classification of early forms of MF is clinically and histologically complex even for experienced clinicians. Ultra-high-frequency ultrasound (UHFUS) is a new non-invasive method that is capable of supporting clinical evaluation, thus providing both a diagnostic guide for distinguishing different stages of the disease and a therapeutic method in terms of monitoring the treatment. In our case report, we clinically and ultrasonographically evaluated the efficacy of chlormethine gel treatment on patches resistant to previous local treatments. A 63-year-old patient was treated for MF stage 1B with cycles of topical clobetasol cream and PUVA and UVB narrow-band phototherapy for 2 years, with a partial therapeutic response. Patches remained at the level of the left and right hips and lower legs. The patient began treatment with chlormethine gel 3 times a week for 2 months. We performed clinical and ultrasound evaluations on the left hip at baseline and after 2 months. The ultrasound examination was performed using (VEVO MD, VisualSonics), both in B-mode and C-mode, with a 70 MHz frequency probe. At baseline, we found a hypoechoic band of 0.94 mm named the Subepidermal Low Echogenity Band (SLEB), localized under the dermo-epidermal junction. Multiple dilated hair follicles with vascular invasion, disrupting the dermo-epidermal junction, and numerous vascular lacunae at the dermal level were found using C-mode. After 2 months of treatment, the lesions had clinically healed with the persistence of a pigmentary outcome. UHFUS identified minimal thickness of the SLEB and minimal vascularization. Chlormethine gel was an effective and well-tolerated treatment in patches unresponsive to other topical treatments. The use of a non-invasive diagnostic technique can allow objective assessment of lesions with a better therapeutic approach.
Pathophysiologically, drug hypersensitive reactions (DHRs) are classified into four types: type I, immediate reactions, and types II, III, and IV, non-immediate reactions. They are further categorized as severe or non-severe based on clinical severity. Genetic predisposition and viral reactivation are cofactors of severe DHR type IV. Rapid identification, cessation, and future avoidance of offending and cross-reactive drugs are the cornerstone of treatment. The diagnosis is established based on the history, physical examination, and subsequent in vivo, in vitro, and drug provocation testing, which are selected based on the severity of DHR. In non-severe DHRs, desensitization to the offending drug is possible.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and aggressive hematologic malignancy, arising from plasmacytoid dendritic cells (pDCs). BPDCN frequently has, at least initially, exclusively cutaneous presentation. We present a 45-year-old woman with a 3-month history of rapidly evolving violaceous patches, infiltrated plaques, and bruise-like tumefactions, disseminated on her face and upper trunk. Histopathology of a lesion, along with Flow cytometry of peripheral blood and cerebrospinal fluid, confirmed the diagnosis of BPDCN. The patient received a hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) regimen and six triple intrathecal chemotherapies. Unfortunately, the patient contracted COVID-19 and died of severe respiratory complications, despite all the available treatment modalities. Our patient presented with typical clinicopathological manifestations of the disease, misdiagnosed for 3 months. The case additionally reflects difficulties in patient management during the COVID-19 pandemic. Dermatologists should be aware of this rare disease, since the early diagnosis and treatment with new emerging drugs may lead to a better prognosis.
Psychological stress may affect skin barrier homeostasis and slow down its recovery. Therefore, we aimed to examine the association between psychological stress levels and natural moisturizing factor (NMF) levels in the stratum corneum (SC). NMF levels were determined in the SC of 25 third-year and 25 sixth-year medical students in two periods (stressful and non-stressful) using the tape stripping method, high-performance liquid chromatography, and spectrophotometry. Additionally, students completed the Perceived Stress Scale questionnaire. Most students had medium stress levels. During the non-stressful period, third-year students were under higher stress than sixth-year students (P<0.001). However, there was no significant difference between the two examined groups in the stressful period. Only among the sixth-year students were significantly higher stress levels found in the stressful period compared with the non-stressful period. In both groups, the levels of NMF did not differ significantly between the examined periods. Regarding the non-stressful period, there was a clear trend of increasing NMF levels among third-year students compared with sixth-year students (P=0.0547), consistent with the higher stress levels among third-year students during this period. Additionally, sixth-year female students had significantly lower NMF levels during the non-stressful period than third-year female students. In conclusion, third-year students were exposed to longer-term stress, while the stress of sixth-year students was associated with the exam-writing period. Additionally, we observed a trend of increasing NMF levels with increasing stress. Further research is needed to determine the mechanism by which stress, as an independent factor, influences NMF levels in the SC.
parts of the world (1,2). CL is characterized by significant clinical variability. An ulcerated nodule on the exposed parts of the body (corresponding to the parasite inoculation site by the vector insect) is the classic presentation. However, other forms of clinical presentations also exist (3,4). CL can be present on unusual locations such as the scalp, the genital region, or palmoplantar areas. Localization in the foot poses a diagnostic challenge due to similarity to disorders which produce ulcerations. The latter include diabetes and leprosy. A 75-year-old Afghani man presented with a large, progressive, cutaneous necrotic ulcer on the left foot (Figure 1) associated with pain, one year in duration. The patient has no history of trauma or any chronic systemic disorders. Additionally, the patient reported no similar diseases in the past. The patient was repeatedly diagnosed with diabetic foot in his village. Dressings were administered several times without any improvement. The patient underwent blood tests, including fasting blood sugar, which were all within normal limits. As the patient lived in an endemic area for CL, CL was suspected and confirmed by slit skin smear and skin biopsy. The latter demonstrated Leishmania amastigotes in the dermal histiocytic infiltrates (Figure 2). The patient is currently under treatment with systemic sodium stibogluconate (pentostam). Leishmaniasis is a major medical issue in several parts of the world. It is transmitted by sandflies. Visceral and cutaneous forms of the disease have been identified. There are more than 1.5 million cases of CL reported annually around the world (1-4). The classical form of CL can be easily diagnosed, particularly in the endemic areas. However, rare and an unusual clinical locations and presentations exist. The latter include annular, chancriform, acute paronychial, palmoplantar, zosteriform, and erysipeloid. Involvement of lower limbs is not common, but can occur (1,2). CL affecting the foot is much less commonly reported (3,4). In this location, CL can be confused with other conditions such as leprosy, vasculitis, neoplastic ulcers, and chronic ulcers due to vascular insufficiency or diabetes. Some authors have stated that routine diagnostic biopsies may be useful in case of clinically suspected wound infections, particularly in patients with deep ulcerations, diabetic foot syndrome, severe soft tissue infection, or fistula tissue. They believed that biopsies are indispensable in the microbiology workup of specific pathogens such as mycobacteria, Leishmania, actinomycetes, Nocardia ssp., or molds (5). The present case highlights the importance of proper investigation of foot ulcer and the importance of considering the diagnosis of leishmaniasis, particularly in endemic areas.
Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery. To the best of our knowledge, this is the first long-term follow-up of an early TNF inhibitor introduction in a patient with acute, severe form of juvenile dermatomyositis and retinal vasculopathy. After 12 years of infliximab therapy, the outcome was excellent, with no side effects throughout the whole treatment.
Eccrine angiomatous hamartoma (EAH) is a rare benign skin neoplasm characterized by an increased size and number of eccrine glands or ducts, along with proliferation of vascular structures in the dermis. This case is unique in its presentation of bilateral symmetrical nodules on both hands and the development of new nodules during puberty. It highlights the need for further research and understanding of this rare condition and its potential progression over time.