The first reported case of erythrodermic sarcoidosis with systemic involvement during COVID-19 vaccination.

Post-vaccinal and parainfectious activation of the immunity with subsequent development of a certain immunological/skinimmunological disease is not rare in clinical practice. This concept is mentioned in relation to molecular/antigenic mimicry. To this day, the pathogenesis of sarcoidosis and sarcoid-type reactions remains a mystery. Moreover, they can be a warning sign of changes in tissue homeostasis, whether they are infectious, noninfectious- immunological, tumor-related, etc. We present a rare form of erythrodermic sarcoidosis with massive systemic involvement (pericarditis, supraventricular tachycardia, hepatitis, iritis/iridocyclitis, pulmonary fibrosis/bihilar lymphadenopathy, and arthritis) developed after receiving the ChadOx1-S vaccine for COVID- 19. Systemic immunosuppressive therapy with Methylprednisolone was introduced according to a scheme (in a reduction mode with an initial dose of 40 mg/day intravenously) in combination with topical Pimecrolimus 1% cream twice a day. Rapid improvement of the symptoms was observed within the first two days of treatment. According to the scientific literature, the presented patient turns out to be the first case of erythrodermic sarcoidosis (with systemic involvement), described as a side effect after vaccination and/or administration of a certain medicinal form.

Kissing atypical melanocytic nevus of genital type of the labia majora in a young Bulgarian patient. What's the best approach?

Melanocytic lesions, especially in delicate anatomical locations such as the vulva, penis, mons pubis etc, are challenging to diagnose. The patients may delay physical examinations due to anxiety or discomfort from the location of the lesion. In terms of therapy options, the surgical approach is not always the preferred one, but it is the one that could lead to a definitive solution to the problem. A limited number of studies do not exclude that atypical nevi of genital type could be considered as melanoma precursors. Single case reports have identified atypical genital nevi of the labia majora as a risk factor for genital melanoma development. Lesions that occupy a larger area than the labia majora and extend into the areas around them are particularly problematic, because the result of a single biopsy could be misleading. Therefore, careful physical examinations are mandatory. Mechanical irritation in the genital area, and in particular in the labia majora region, is an additional reason for choosing the surgical-reconstructive therapeutic option. We present a 13-year-old female with a progressive kissing divided nevus, located in the area of the vulva and labia majora, extending to the mucosa. A biopsy was taken in order to rule out malignancy. Immunohistochemistry was performed with specific melanocyte markers S-100, HMB-45 and SOX confirming the benign origin of the lesion. A diagnosis of atypical melanocytic nevus of genital type was made. For prevention a surgical excision was advised but later on declined by the patient's parents. Further close observation of the lesion was recommended.

Efficacy of a retinoid complex plus anti-inflammatory component cream alone or in combination with prebiotic food supplement in adult acne: A randomized, assessor-blinded, parallel-group, multicenter trial on 184 women.

BACKGROUND: Adult female acne (AFA) nowadays is a very common skin condition affecting mainly women aged between 25 and 40. The treatment of AFA could be challenging. STUDY AIM: We evaluate and compare the efficacy and tolerability of a cream formulation containing two retinoid molecules (hydroxypinacolone/retinyl palmitate) combined with Iris Florentina root extract and a complex of three oligopeptides (C) applied twice a day (morning and evening) alone or in combination (C + O) with a food supplement containing a mixture of prebiotic molecules (FOS&GOS) zinc, lactoferrin, and niacinamide. SUBJECTS AND METHODS: In a multicenter, randomized, assessor-blinded, 12-week trial, we assessed the efficacy of these two regimens in the evolution of AFA lesions (non-inflammatory: NI-L; inflammatory: IL; and total number of lesions: TL). Additional efficacy endpoints were the evolution of the 6-point (from 0 to 5) GEA and Adult Female Acne Scoring Tool (AFAST) scores. RESULTS: One hundred and eighty-four women (mean age 32 ± 6 years) with AFA agreed to participate after obtaining informed consent. They were randomized (2:1) to the topical product (n = 123) (Group C) or to the combination (n = 61) (Group C + O) treatment. All enrolled patients concluded the trial with no drop-out. At baseline, NI-L, IL, and TL acne lesion count were 15 ± 9, 9 ± 5, and 24 ± 14 in the Group C and 19 ± 8, 9 ± 4, and 29 ± 10 in Group C + O. In comparison with the number of the acne lesions at the baseline, both treatment regimens induced a significant reduction (p = 0.0001, ANOVA test) at Week 12 in NI-L, IL, and TL by -54%, -63%, and - 59% in Group C and by -55%, -73%, and - 61% in the Group C + O, respectively. At Week 12, the absolute IL count reduction vs. baseline was significantly (p = 0.0158) greater in Group C + O (-7.0) in comparison with Group C (-5.5). The GEA absolute score reduction in Group C + O group was significantly greater in comparison with Group C (-1.5 vs. -1.1; p = 0.0097). In the Group C + O, a greater percentage of success treatment (defined as a GEA score of 0/1 at Week 12) was observed in comparison with Group C (39% vs. 27%; p = 0.06). AFAST score at baseline was 2.4 ± 0.5 in group C and 2.8 ± 0.6 in group C + O. AFAST score was reduced by 21% and by 51% after 6 and 12 weeks of treatment in group C and by 22% and 55% in group C + O, respectively. Both treatment regimens were well tolerated. Not relevant adverse events were recorded. CONCLUSION: A cream containing retinoid molecules and Iris Florentina root extract is effective and well tolerated in the management of AFA. The treatment combination with a prebiotic and anti-inflammatory food supplement offers an additional clinical benefit mainly in reducing inflammatory lesions and improving the severity acne score.

H syndrome: the first 79 patients.

BACKGROUND: H syndrome is an autosomal recessive genodermatosis with multisystem involvement caused by mutations in SLC29A3. OBJECTIVE: We sought to investigate the clinical and molecular findings in 79 patients with this disorder. METHODS: A total of 79 patients were included, of which 13 are newly reported cases. Because of the phenotypic similarity and molecular overlap with H syndrome, we included 18 patients with allelic disorders. For 31 patients described by others, data were gathered from the medical literature. RESULTS: The most common clinical features (>45% of patients) were hyperpigmentation, phalangeal flexion contractures, hearing loss, and short stature. Insulin-dependent diabetes mellitus and lymphadenopathy mimicking Rosai-Dorfman disease were each found in approximately 20%. Additional systemic features were described in less than 15% of cases. Marked interfamilial and intrafamilial clinical variability exists. Twenty mutations have been identified in SLC29A3, with no genotype-phenotype correlation. LIMITATIONS: In the 31 patients described by others, data were collected from the medical literature. CONCLUSIONS: H syndrome is a multisystemic disease with clinical variability. Consequently, all SLC29A3-related diseases should be considered a single entity. Recognition of the pleomorphic nature of H syndrome is important for diagnosis of additional patients.

Polyamine metabolism changes in psoriasis.

INTRODUCTION: Polyamines - putrescine, spermidine and spermine are polycationic compounds ubiquitous for all living organisms. They are essential for the cell growth and differentiation, the control of cell cycle progress, apoptosis, and cancerogenesis. Accumulated scientific evidence suggests the central role of polyamines in the process of keratinocytic proliferation, differentiation, and regulation. OBJECTIVE: To elucidate the polyamine metabolic changes that occur in benign keratinocytic proliferation. Fifty eight patients were enrolled in the study, 31 with plaque-form of psoriasis vulgaris, which had been referred to as a model of benign keratinocytic proliferation, and 27-healthy controls. MATERIALS AND METHODS: An original, innovative chromatographic method was used to detect the levels of putrescine, spermidine, and spermine in all skin samples. RESULTS: Were significantly proven (P < 0.05). No difference was found between the polyamines levels of non-lesional psoriatic skin and healthy controls. Psoriatic lesions showed a two-time higher concentration of all polyamines in lesional, compared to non-lesional skin. Spermine had the highest concentration and highest proliferation trend, which demonstrated the importance of propylamine synthesis in the pathogenesis of psoriasis. Spermine highest concentrations suggested the leading role of adenosine methionine decarboxylase (AMDC) in the pathogenesis of benign keratinocytic proliferations. CONCLUSIONS: Non-lesional skin in psoriatic patients did not show latent changes in polyamine metabolism. Psoriatic lesions demontrated two-time higher levels of the most essential biogenic polyamines compared to healthy controls. The highest level of spermine proved the crucial role of AMDC in the polyamine metabolism changes in psoriasis. Future therapeutic approaches should be focused on reduction of exogenic spermine intake, utilizing new spermine blockers, and synthesis of AMDC inhibitors.

Etanercept-induced Wegener granulomatosis in a patient with rheumatoid arthritis.

A very rare case of etanercept-induced Wegener's granulomatosis in a patient with long-standing rheumatoid arthritis is reported. A thorough critical analysis on Wegener's granulomatosis pathogenetic mechanisms is done. The peculiarities of etanercept pharmacodynamic features are also presented together with some suggestions of possible induction pathways.

Comparative analysis of polyamine metabolism in benign and neoplastic keratinocytic proliferations.

INTRODUCTION: Polyamines (putrescine, spermidine, and spermine) are polycationic compounds that play a central role in keratinocytic proliferation, differentiation, and regulation. The objective was to elucidate the polyamine metabolic changes that occur in various benign and neoplastic skin proliferations. METHODS: The study included 58 patients: 31 with the plaque form of psoriasis vulgaris and 27 with non-melanoma skin tumors. The levels of putrescine, spermidine, and spermine were detected in lesional and non-lesional skin samples. RESULTS: Findings were representative (p < 0.05). Psoriatic lesions showed a twofold elevation of all polyamines in lesional skin compared to non-lesional skin. Spermine had the highest concentration, which suggested a leading position of propylamine synthesis in psoriatic pathogenesis. Results on the polyamine metabolism of basal cell carcinoma represented basic characteristics similar to those of psoriasis. Conversely, squamous-cell carcinoma lesions showed the highest concentration of putrescine, suggesting a crucial role of spermidine-spermine acetyltransferase in their pathogenesis. DISCUSSION: Our findings showed different polyamine metabolic changes in lesions from benign and neoplastic keratinocytic proliferations. Basal-cell carcinoma polyamine metabolism revealed a closer relationship to psoriasis than to squamous-cell carcinoma, which might explain its long-term benign course and non-metastatic nature.

Recall dermatitis after systemic treatment with paclitaxel.

BACKGROUND: Recall phenomena of the skin at the site of prior radiation treatment are well established after systemic anti-neoplastic therapy. The aim of this report is to describe a rare clinical entity of recall dermatitis after systemic treatment with paclitaxel without a history of previous radiation at the site of reaction. MATERIAL AND METHODS: A 63-year-old Caucasian female patient treated with paclitaxel because of breast cancer (T3 N1 M0) is presented. RESULTS: Five days after the fifth-in-a-row infusion swelling and redness occurred on the left arm, where the drug has been administered. The skin changes were interpreted as erysipelas and the patient was treated with systemic antibiotic. One month later, when a new cycle of chemotherapy with paclitaxel was performed, the medication was administered on the opposite (right) arm. Several days after the procedure, the same changes occurred again on the left arm, as in the previous hospitalization. Based on the clinical features and the laboratory findings, diagnosis of "recall dermatitis" was coined. The presented case serves as a basis for discussion on the etiopathogenetic mechanism of the skin recall phenomenon. The drugs associated with the onset of such a reaction are debated. CONCLUSION: The specificity of this rare dermatological entity is important for setting up the exact diagnosis and therapeutic approach.

Therapeutic Hotline: Cysteinyl leukotriene receptor antagonist montelukast in the treatment of atopic dermatitis.

Leukotrienes are potent proinflammatory mediators derived from arachidonic acid through the 5-lipoxygenase pathway. Experimental data suggest a role for cysteinyl leukotrienes in the pathogenesis of atopy giving a rationale for its use in asthma, allergic rhinitis, and chronic urticaria management. A few clinical observations and small trials suggest that montelukast may be used in an adjunctive manner as an effective therapeutic option for all age categories affected by moderate-to-severe atopic dermatitis. Our own observations proved that montelukast as a prospective corticosteroid-sparing option in the complex therapeutic strategy of corticosteroid-dependent atopic dermatitis patients, even in the severe erythrodermic cases.

Verrucous systemic lupus erythematosus.

Few patients with systemic lupus erythematosus have features of verrucous (hypertrophic) lupus erythematosus. A 29-year-old Caucasian woman with a 7-year history of systemic lupus erythematosus developed painful verrucous plaques on the nose. Erythematous, raised, indurated, hyperkeratotic plaques localized on the dorsa of the distal parts of the toes and over the interphalangeal joints of her fingers were also noted. A large, dull-red, indurated plaque with rolled borders on the bridge of the nose was most characteristic. Rapid therapeutic effect was obtained by systemic corticosteroid regimen. This verrucous variant of lupus erythematosus, sometimes clinically resembling actinic keratosis, keratoacanthoma and squamous cell carcinoma, is reviewed.

The H syndrome is caused by mutations in the nucleoside transporter hENT3.

The H syndrome is a recently reported autosomal-recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, short stature, hallux valgus, and fixed flexion contractures of the toe joints and the proximal interphalangeal joints. Homozygosity mapping in five consanguineous families resulted in the identification of mutations in the SLC29A3 gene, which encodes the equilibrative nucleoside transporter hENT3. Three mutations were found in 11 families of Arab and Bulgarian origin. The finding of several different mutations in a small geographic region implies that the H syndrome might be rather common. The identification of mutations in the SLC29A3 gene in patients with a mild clinical phenotype suggests that this is a largely underdiagnosed condition and strongly suggests that even oligosymptomatic individuals might have the disorder.

POEMS in childhood.

A 17-year-old boy had a 3-year history of diabetes mellitus, malabsorption syndrome, and skin changes consisting of induration, hyperpigmentation, and hypertrichosis on the anterior aspect of both thighs, lower abdomen, and scrotum. Physical examination found hypogonadism, hepatomegaly, gynecomastia, growth retardation, and ankle edema. There was no neuropathy or plasma cell dyscrasia. However, the characteristic skin changes and the combination of symptoms suggest polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes syndrome. This is a rare multisystemic disorder of obscure pathogenesis and no conspicuous heredity. Overproduction of vascular endothelial growth factor is thought to cause microangiopathy, neovascularization, and accelerated vasopermeability causing the multiorgan deterioration. Cyclophosphamid cytostatic therapy seems beneficial.

Vesicular mycosis fungoides.

A 62 years old patient presented with rapidly progressive mycosis fungoides. Lesions of the face, head, trunk and limbs exhibited numerous vesicles and erosions. Histopathology showed marked spongiosis and intraepidermal blisters, as well as invasion of the epidermis by atypical lymphocytes, which was confirmed by immunohistochemistry. The vesicular variant of mycosis fungoides is rare and associated with a poor prognosis.

Dermatological aspects of cerebrovascular diseases.

Neurological symptoms are sometimes triggered by the same mechanisms as are skin manifestations. They include genetic conditions like the epidermal nevus syndrome, the Sneddon syndrome, Fabry disease and others, as well as certain inflammatory disorders like erythematous lupus, Bechet disease. Basically all conditions giving rise to anticoagulation processes may cause simultaneously neurological and cutaneous manifestations. Cerebrovascular stroke is the third most common condition of death in the developed world after cancer and ischemic heart disease. The mechanisms responsible for development of skin manifestations in patients afflicted by stroke are shortly reviewed. Stroke may also influence the already existent skin diseases.