Complete Resolution Of Skin Lesions Research Articles

Unique ulcerative autoinflammatory disease mistaken for factitious disorder

Autoinflammatory diseases (AID) are disorders of inappropriate innate immune system activation. Twenty-four percent of patients with AID do not have a well-defined clinical picture or pathogenic mutation and are characterized as having undefined AID. Cutaneous ulcers have been described but not outside of distinct histologic and clinical disorders. We present a unique case of undefined adult-onset AID with ulcers mistaken for a factitious disorder.A 32 year-old female with borderline personality disorder, depression, and anxiety developed recurrent cutaneous MRSA abscesses and recurrent ulcers of the extremities and scalp associated with alopecia. The ulcers were minimally-responsive to IV antibiotics, isotretinoin, oral dapsone, intralesional triamcinolone, and adalimumab. Flares of the skin disease were associated with fevers and elevated inflammatory markers.Scalp ulcer biopsies revealed numerous pigmented hair casts with trichomalacia and perifollicular lymphocytic infiltrate. One hair follicle contained bacterial cocci associated with a perifollicular neutrophilic infiltrate. These findings along with the patient's psychiatric history led to a diagnosis of trichotillomania from multiple dermatology and psychiatry consultations. The patient denied that she was inducing the lesions and sought another psychiatric consultation, who felt this was not a factitious disease. She was referred to immunology for evaluation of a possible IEI. Laboratory evaluation revealed mild hypogammaglobulinemia with IgG of 663 (768–1632) mg/dL, normal antibody response to recall antigens, but absent response to neo-antigen (Salmonella typhi). Peripheral lymphocyte phenotyping was normal except for a relative decrease in the number of memory B-cells with CD27+IgD+% of 6.9 (13.4–21.4) and CD27+IgD-% of 2.5 (9.2–18.9). Neutrophil oxidative burst was normal. Whole exome sequencing did not identify variants in genes associated with her phenotype.The patient was partially responsive to IVIG but highly responsive to high-dose intravenous methylprednisolone. She was subsequently diagnosed with undefined AID based on Eurofever registry criteria matching her clinical course and had near complete resolution of skin lesions and alopecia with the addition of anakinra.Clinicians must be vigilant for atypical presentations of AID. Our patient had signs of undefined AID but had a delayed diagnosis due to her psychiatric disorders, to which her symptoms were attributed, and a previously undescribed presentation of AID.

Enzalutamide-Induced Acute Maculopapular Rash in Treatment of Metastatic Prostate Cancer: First Case Report from a Tertiary Cancer Care Center of North India.

Enzalutamide is a new potent inhibitor of the signaling pathway for the androgen receptor with a half-life of 5.8 days. It has been on the market for the treatment of metastatic castration-resistant prostate cancer since November 2013.We report a case of acute generalized exanthematous maculopapular rash induced by enzalutamide. In summary, newer androgen receptor blockers have a propensity to cause skin related adverse effects. Most common among these are apalutamide. Enzalumatamide, per se, is a safe drug and has not been associated frequently in causing maculopapular rash. Few cases has been reported. In all these cases, the drug was discontinued and 2nd line therapy was instituted. In this report, Enzalutamide was withheld for 10 days and anti-histaminics was instituted. After a full recovery, Enzalutamide was reinstituted in treatment.A 62-year-old male patient with no significant medical history, was diagnosed in March 2020 with metastatic prostatic adenocarcinoma. Baseline PSA was 456 ng/ml. PSMA PET scan showed evidence of multiple bony metastasis. He was started on Degarelix subcutaneous injection with oral abiraterone initially. PSA level showed initial decreasing trend till September 2021 followed by sudden increase. Intramuscular Injection leuprolide was started and initial responses were good followed by later rise of PSA from January. Tab Xtandi (Enzalutamide) was added to the regimen from 31.1.22. Three days after starting enzalutamide treatment, the patient experienced an acute skin reaction. It is about of the plaques covered with widespread millimetric non-follicular papules. Enzalutamide was stopped after appearance of rashes to avoid further serious adverse effects. Anti-histaminics were started. Complete resolution of skin lesions occurred within 10 days. Tab Enzalutamide was reinstituted on 11th day after stoppage and on complete resolution of skin resolutions. According to the CTCAE 5.0 criteria, these skin rash was graded as grade 2. In view of evidence in literature and clinical improvement after stoppage, the acute drug reaction was attributed to enzalutamide.Uro oncologist can be confronted with adverse skin drug reactions attributable to new therapeutic molecules. The slow resolution of symptoms seems be due to the long half-life of enzalutamide. It should not be withdrawn from therapy owing to these effects. Rather, it should be with stopped for 10-14 days. Basic treatment with anti-histaminics or topical steroids may be enough to warranty the resolution of symptoms, and the drug (Enzalutamide) can be continued thereafter.

Symptom Resolution in Acyclovir Treated Hand, Foot, and Mouth Disease

Background: Hand, Foot, and Mouth Disease (HFMD) is a highly contagious virus primarily affecting young children, but may also impact older children and adults. Initial symptoms typically manifest as fever, malaise, and pharyngitis, followed by eruption of erythematous, papular lesions on the palms and soles. HFMD is typically treated with supportive care, as there is no current gold standard therapy for HFMD. However, based on previous case studies, acyclovir has demonstrated its potential as a viable treatment for HFMD. Case Presentation: A 37-year-old female patient presented to the clinic with a two-day history of fever, malaise, pharyngitis, and papulovesicular skin lesions on her hands and feet. A clinical diagnosis of HFMD was made, and the patient was promptly started on oral acyclovir 800 mg, three times daily for seven days. Within two days of acyclovir treatment, the patient’s fever subsided, and the skin lesions on the hands and feet were fully resolved. Conclusion: Our case report showcases the benefits of acyclovir therapy in treating HFMD. Acyclovir treatment provided our patient symptomatic relief, resulting in defervescence and complete resolution of skin lesions. Symptom duration and severity were lessened significantly within the first couple days of therapy. Acyclovir therapy has shown promising results for potential HFMD treatment, and we encourage additional studies to further evaluate its efficacy.

A new siloxane embedded benzalkonium chloride-based skin protectant (fiteBac Germicidal Skin Softening Gel) for use against insect bites and related dermal infections

fiteBac Skin Softening Gel, widely used as a cosmetic, contains the anti-infective benzalkonium chloride embedded in siloxane quaternary ammonium compounds, but does not contain alcohol. It thus serves well for skin protection combined with local anti-infective activity. As it does not penetrate the skin, no systemic toxic side effects are expected, and more than 3 years of practical use have not revealed any adverse reactions.We have tested in a pilot study the usefulness of fiteBac Skin Softening Gel against skin lesions following insect bites various in nature as well as a few other ailments (e.g. fungus-related intertrigo). Of the 23 cases tested, all but one responded to the treatment with complete resolution of skin lesions within a few days up to a week. The only exception was a case of suspected tick bite with local arthritic symptoms. In the latter, a combination of the fiteBac gel treatment with subsequent local administration of ibuprofen gel also resulted in complete remission of dermatitis and arthritis.We thus recommend wider use of this cosmetic/anti-infective agent for the prevention and treatment of insect bites to prevent subsequent infectious complications (such as, for instance, Lyme disease).

Canine pemphigus folliaceus: report of five clinical case

Pemphigus folliaceus (PF), which is considered the most common autoimmune skin disease in the dog, is associated with the formation of autoantibodies directed against the desmosomes of the upper third of the epidermis, that result in loss of keratinocyte adhesion (acantholysis) and subsequent epidermal cleft formation. The clinical and histopathological findings, along with the response to treatment in 5 PF cases, either idiopathic (3/5) or secondary to trimethoprim-sulfonamides (1/5) and to allergic dermatitis (1/5), are presented and discussed, accordingly. All the 5 dogs exhibited hypotrichosis-alopecia (4/5), erythema (4/5), vesicles (1/5), pustules (5/5), crusts (5/5), epidermal collarettes (3/5), excoriations (1/5), erosions (3/5), ulcers (1/5), fissures (1/5), lichenification, thickening and folding of the skin (1/5), and footpad hyperkeratosis (1/5), that were pruritic in 3 of them. These lesions were located on the head (4/5), pinnae (4/5), limbs (3/5), body trunk (3/5) and the footpads (2/5). Diagnosis was based on the results of lesionai histopathology where the most striking finding was the presence of subcorneal pustules filled with normally looking neutrophils and acanthocytes. Moreover, pustule or crust cytology that was carried out in 3 cases, revealed clusters of acantholytic cells in 2 dogs. The treatment, that was instituted in 2/5 dogs and resulted in a complete resolution of skin lesions, was based on prednisolone in immunosuppressive dosage along with azathioprine (PF secondary to allergic dermatitis), or on a combination of dapsone, Oxytetracycline and vitamin E (idiopathic PF). The remaining 3 cases were left untreated, because one showed a spontaneous remission following withdrawal of the offending medication and the other 2 were lost to follow-up.

Visceral leishmaniasis with cutaneous involvement caused by Leishmania infantum-chagasi

We report the case of a 66 year-old woman who presented nodular skin lesions on her back and upper extremities. Biopsy revealed amastigotes that were identified as Leishmania infantum-chagasi by PCR. Evaluation also showed hepatomegaly and pulmonary nodules. Treatment with amphotericin B led to complete resolution of skin lesions.

Drugs for discoid lupus erythematosus.

Drugs for discoid lupus erythematosus.

Steven Johnson Syndrome and Toxic Epidermal Necrolysis in a burn unit: A 15-year experience

IntroductionThe diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is similar to skin loss in second degree burns, and many of these patients are referred for treatment at burn centers. Treatment can differ markedly from center to center, and mortality can range from 25% to 70%, including a considerable morbidity. However, our experience over a 15-year period from 2000 to 2015 with 40 patients found a mortality rate of only 10% (4/40). The purpose of this paper is to discuss our treatment algorithm as a model for other centers treating SJS/TENs patients. MethodsRecords were reviewed for all patients admitted to the LAC+USC burn unit between 2000 and 2015 and 40 patients were identified with biopsy-proven SJS or TENS. These cases were reviewed for age, gender, initial and greatest TBSA, causative drug, pre-existing medical conditions, and morbidity and mortality. All data were entered into the SPSS statistical software package and all statistical analyses were performed using this program. ResultsOur treatment algorithm focused on early referral to a specialty burn unit, immediate discontinuation of the offending drug, fluid resuscitation, nutritional supplementation, and meticulous wound care. Average time to transfer to a burn unit was 3.36 days. Silver-releasing antimicrobial dressings were applied to the affected skin surface and changed every 3 days. Mupirocin coated petroleum gauze was used for facial involvement. Steroids were tapered and discontinued if initiated at an outside facility (58% of patients), and starting after 2001, all patients received a course of IVIG. All patients received fluid resuscitation and the majority received supplemental tube feedings (69%). Average length of total stay was 17.1 days and length of ICU stay 15.9 days. While 44% were transferred to another facility for further rehabilitative care, 37% of patients discharge to home. In patients discharged home with complete resolution of skin lesions, time to healing was an average of 14 days. DiscussionWith our 10% mortality rate in 40 patients, our study represents a relatively large study population while maintaining a relatively low mortality rate. The demographic data from our study largely aligns with the existing literature, and we therefore feel that our low mortality rate is due to our treatment algorithm, rather than to a less severe pathology in our patient population. This claim is supported by a standard mortality ratio of 1.68. This ratio proves a significantly improved mortality than would be expected based on disease severity on admission.

Case report of blastic plasmacytoid dendritic cell neoplasm (BPDCN) a rare entity

Aim/Background: BPDCN is a rare aggressive haematological malignancy in the elderly, with high frequency of cutaneous and bone marrow involvement and a poor prognosis. Our aims are (1) to increase awareness of this rare entity and (2) discuss the value of different investigation tools in diagnosis and response assessment.Method: A 75-year-old man presented with widespread nodular violaceous rash. Full blood count showed Hb 112 g/L, WCC 16.5×109/L, platelets 162×109/L and circulating blasts. Skin biopsy, bone marrow biopsy and flow cytometry were consistent with BPDCN (CD4+, CD56+, CD38+, CD45dim, CD123bright, negative for lineage markers). He was commenced on induction chemotherapy using a modified acute lymphoblastic leukaemia (ALL) protocol for the elderly.Results: Chemotherapy was well tolerated with complete resolution of skin lesions by day 15. Repeat bone marrow biopsy (day 30) revealed significant reduction but persistence of blasts (9%) detected by flow cytometry.Conclusions: Ancilliary investigations in particular flow cytometry and immunohistochemistry are essential in the diagnosis of BPDCN and to distinguish this from other haematological malignancies. Bone marrow biopsy together with flow cytometry and/or immunohistochemistry to assess remission status should be considered post induction chemotherapy even if cutaneous manifestations have resolved. Aim/Background: BPDCN is a rare aggressive haematological malignancy in the elderly, with high frequency of cutaneous and bone marrow involvement and a poor prognosis. Our aims are (1) to increase awareness of this rare entity and (2) discuss the value of different investigation tools in diagnosis and response assessment. Method: A 75-year-old man presented with widespread nodular violaceous rash. Full blood count showed Hb 112 g/L, WCC 16.5×109/L, platelets 162×109/L and circulating blasts. Skin biopsy, bone marrow biopsy and flow cytometry were consistent with BPDCN (CD4+, CD56+, CD38+, CD45dim, CD123bright, negative for lineage markers). He was commenced on induction chemotherapy using a modified acute lymphoblastic leukaemia (ALL) protocol for the elderly. Results: Chemotherapy was well tolerated with complete resolution of skin lesions by day 15. Repeat bone marrow biopsy (day 30) revealed significant reduction but persistence of blasts (9%) detected by flow cytometry. Conclusions: Ancilliary investigations in particular flow cytometry and immunohistochemistry are essential in the diagnosis of BPDCN and to distinguish this from other haematological malignancies. Bone marrow biopsy together with flow cytometry and/or immunohistochemistry to assess remission status should be considered post induction chemotherapy even if cutaneous manifestations have resolved.

Cutaneous Manifestations of Toxoplasmosis: a Case Report

Abstract Although toxoplasmosis is one of the most widely spread infections in the world, types that involve the skin are extremely rare. However, skin lesions are not specific; moreover, they are quite diverse, which makes the diagnosis of cutaneous toxoplasmosis rather difficult. Thus, differential diagnosis should include a number of other diseases. We present a case of a 43-year-old immunocompetent man with multiple livid erythematous papules and nodules with yellowish discharge that involved the skin of the body and the extremities. By using electro-chemiluminescence immunoassay, immunoglobulin G antibodies to Toxoplasma gondii were detected in the serum, confirming the diagnosis of toxoplasmosis. The treatment with pyrimethamine and trimethoprim-sulfamethoxazole led to complete resolution of skin lesions. In conclusion, although rare in the dermatological practice, cutaneous toxoplasmosis should be considered in all patients presenting with lymphadenopathy, non-specific skin eruptions, especially nodular and colliquative, blood eosinophilia and histological findigs revealing abundant eosinophilic inflitrations.

First description of Merkel Cell polyomavirus DNA detection in a patient with Stevens–Johnson syndrome

Merkel Cell polyomavirus (MCPyV), a ubiquitous DNA tumor virus, has been found to be associated with Merkel cell carcinoma and chronic lymphocytic leukaemia while other associations are still being explored. MCPyV sequences have also been detected in normal tissues of tumor patients and in the blood of healthy donors. This report documents a new MCPyV association with the Stevens-Johnson syndrome, a rare immune-modulated mucocutaneous process particularly associated with specific drugs and infective agents. A high MCPyV viral load was detected simultaneously in fluid from skin lesions (2.0 × 10(4) copies/ml) and in matched blood (7.4 × 10(5) copies/ml) from a young adult patient after bone marrow transplant for a relapsed T-cell acute lymphatic leukaemia. MCPyV clearance concurred with the complete resolution of skin lesions after 5 days of cidofovir treatment. DNA sequencing classified the amplicons as the European/Italian MKL-1 strain. Given its ubiquitous nature, MCPyV could account for part of Stevens-Johnson syndrome idiopathic cases.

Application of IFN‐γ releasing assay for the diagnosis of erythema induratum of Bazin

Erythema induratum of Bazin (EIB) is regarded to be a hypersensitive reaction to the concomitant tuberculosis. Recently, interferon-γ releasing assay (IGRA) has been focused as a promising tool in the diagnosis of latent tuberculosis. However, there has been no large scale study to investigate the usefulness of IGRA in the diagnosis of EIB. To evaluate the diagnostic performance for the detection of EIB. We retrospectively reviewed medical records of all patients with EIB, in the Department of Dermatology, at the Seoul National University Hospital, between April 2009 and September 2011. We analysed clinicopathological features, responses to IGRA and the treatment courses. In addition, we compared positive rate of IGRA in patients with other diseases during the same period. All of the 22 patients demonstrated a positive response to IGRA (100%) and showed a good response to anti-tuberculosis treatment. In contrast, positive rate was 63.64% and 66.67% in patients with psoriasis and other vasculitis respectively. We observed complete resolution of skin lesions in 14 patients. Partial resolution was attained in one patient and the other seven patients are currently on the medication and are showing good responses. We verified that IGRA has an excellent diagnostic performance in EIB, through this observational study. It is strongly suggested that if EIB is clinicopathologically suspected, IGRA should be performed.

Phantom Limb Pain in Patients with Severe Cryoglobulinemic Vasculitis: Usefulness of Long-Term Pregabalin Treatment

BackgroundPhantom limb pain [PLP] may complicate limb amputation. This symptom has been never described in patients with mixed cryoglobulinemia [MC] syndrome.FindingsWe observed three patients with hepatitis-C virus-related MC complicated by severe skin vasculitis responsible for gangrene needing limb amputation. After surgery, they developed refractory PLP and stump pain, which was successfully treated with pregabalin.ConclusionsA clear improvement of PLP was observed in all patients during pregabalin; in addition, this treatment permitted an effective bed preparation and intensive medications of relapsing vasculitic ulcers in the stump, followed by complete resolution of skin lesions. At the same time, traditional analgesic therapies, potentially responsible for relevant side effects, were progressively tapered until discontinuation.

Paraneoplastic Pemphigus: A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.

Successful treatment of refractory cutaneous infection caused by Mycobacterium marinum with a combined regimen containing amikacin

Background:The incidence of Mycobacterium marinum infection has been increasing. First-line antituberculous drugs and other common antibiotics are effective for most cutaneous M. marinum infections; however, treatment failure still occurs in some rare cases. We report a case of a 70-year-old man with refractory cutaneous infection caused by M. marinum. Reasons for delayed diagnosis and related factors of the refractory infection are also discussed.Methods:Samples of lesional skin were inoculated on Löwenstein–Jensen medium for acid-fast bacilli. Species of mycobacterium were identified by polymerase chain reaction–restriction fragment length polymorphism (PCR-RFLP) analysis. We then carried out genotyping by using mycobacterial interspersed repetitive units and sequencing of heat shock protein 65 (hsp65) and 16S rDNA genes.Results:Tissue cultures for acid-fast bacilli were positive. PCR-RFLP analysis and sequencing of hsp65 and 16S rDNA genes confirmed the isolated organisms to be M. marinum. Systemic therapy with rifampicin, clarithromycin, and amikacin empirically over 6 months led to complete resolution of skin lesions leaving only some residual scars.Conclusion:Key diagnostic elements for M. marinum infections include a high index of suspicion raised by chronic lesions, poor response to conventional treatments, and a history of fish-related exposure. Strong clinical suggestion of M. marinum infection warrants initial empirical treatment. The duration of therapy is usually several months or even longer, especially for elderly patients. Amikacin can be considered in multidrug therapy for treatment of some refractory M. marinum infections.

Acitretin in management of diffuse common warts: a case report

Warts are among the most commonly observed dermatological diseases, caused by human papilloma virus (HPV), usually HPV1-2 subtypes; HPV4-7 are rarely found and mostly related to professional exposure (butchers and dairy workers). Different therapeutical approaches are possible, depending on extension and severity of lesions. The present authors describe the case of 32-year-old Caucasian man, who came to our attention for the presence of numerous exophytic papules on the back of both hands and over periungual regions, which appeared about 6 months before. Histological examination confirmed the clinical suspicion of common warts (HPV4). The patient underwent therapy by acitretin for 12 weeks, obtaining during the 8th week of therapy complete resolution of skin lesions. The present authors present this case for the unusual local aggressiveness of viral warts in an immunocompetent patient successfully treated with acitetrin.

P107 Clinical, immunophenotypical and molecular analysis of a series of patients with poikilodermatous mycosis fungoides

Introduction Poikilodermatous mycosis fungoides (PFM) is an uncommon variant of MF, characterized by poikilodermia, atrophy, mottled dyspigmentation and telangectases. Aim To analyze cases of PMF diagnosed at the lymphoma clinic of Andreas Sygros Hospital, in relation to their clinical presentation, immunophenotypic and molecular characteristics and disease course. Methods All patients with PFM were retrospectively evaluated. Patients with predominantly poikilodermatous lesions of MF were selected. Information on clinical, histological, immunohistochemical and molecular data were recorded, in addition to information on treatment and follow up. Results Eleven patients (4 males and 7 females) with PMF were identified. The mean age at diagnosis was 33.5 years (range 10–75 years). Ten out of 11 patients had early stage disease (IB) and one patient presented with tumors accompanying widespread poikilodermia (stage IIB). Two patients had associated lesions typical of lymphomatoid papulosis (LyP). In 45.5% of patients (5 of 11), the atypical lymphocytes were predominantly CD4-positive, whereas the same proportion of patients (5 of 11) had a cytotoxic phenotype. Finally, aberrant CD4-CD8- phenotype was found in one patient, while in seven patients there was a loss of CD7 antigen. PCR analysis disclosed the presence of clonal rearrangements of the genes of TcRγ receptor in 8 out of 11 skin biopsies. Following diagnosis, 8 patients were treated with PUVA. Of these, six showed complete resolution of skin lesions, one partial remission and one progressive disease. Two patients showed stable disease after UBV-NB and acitretin 35 mg/day respectively. Finally, patient with tumor stage MF underwent electron beam radiotherapy on tumors followed by PUVA plus interferon a-2b 5 MU tiw, with disease progression after 5 months of treatment. Conclusion Our data suggest that PMF is commonly diagnosed at a young age and can be frequently accompanied by LyP lesions. A cytotoxic immunophenotype is also more common in PMF. Patients with PMF respond well to phototherapy and may have a good overall prognosis.

Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation

To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.

Steroid‐induced Periorificial Dermatitis in Children—Clinical Features and Response to Azelaic Acid

Periorificial dermatitis, a common skin disease in young women, has been occasionally reported in children. This study elaborates the clinical features of periorificial dermatitis in children as well as possible pathogenetic factors and the response to 20% azelaic acid cream. A total of 10 children aged 3 to 12 (mean 7.7) years suffering from nongranulomatous periorificial dermatitis for 3 to 7 (mean 4.9) months were evaluated, and dermatologic examination was carried out. Pretreatment was documented. Skin prick tests with a panel of six common inhalative allergens and patch tests with the European Standard Series were performed. An association between atopy and periorificial dermatitis was evaluated, and patients were screened for skin colonization by fungi, bacteria, and Demodex mites. They were treated with 20% azelaic acid cream, which was topically applied twice daily on all affected areas, until complete resolution was achieved. Treatment period was followed by an individual observation period. Periorificial dermatitis had developed in typical distribution and morphology. In all patients, low- to high-potency topical corticosteroids had been used on the face prior to manifestation. Atopy was found in half of the patients. Allergological, bacteriological, and mycological examinations did not reveal pathologic results. Demodex mites could not be demonstrated by skin surface biopsy. Treatment with 20% azelaic acid cream led to complete resolution of skin lesions after 4 to 8 (mean 5.4) weeks in all patients. Transient exacerbation of skin condition with a peak between the 2nd and 6th day of treatment could be observed in three patients. Side effects of 20% azelaic acid cream were registered in six patients and were predominantly present in the first 2 weeks of treatment. Side effects were minimal and became rarer with ongoing treatment. No recurrences were seen within a follow-up period of 2 to 8 (mean 4.4) months. Treatment with 20% azelaic acid cream could provide an effective and safe alternative therapeutic option in children with nongranulomatous periorificial dermatitis.

Scurvy diagnosed in a pediatric liver transplant patient awaiting combined kidney and liver retransplantation

First described in the 1500 s, scurvy is infrequently seen in industrialized countries today, although vulnerable patient groups remain. A 15-yr-old girl underwent liver transplantation at age 26 months for a primary diagnosis of biliary hypoplasia, and subsequently developed late allograft failure and progressive renal insufficiency culminating in listing for combined liver retransplantation and kidney transplantation at age 13 yr. She required regular hemodialysis treatment for 12 months prior to deceased donor organ availability, with a complicated clinical course including recurrent septic episodes and severe cachexia. Ten months after initiation of hemodialysis, she presented with severe bone pain, purpura, ecchymoses, gingival hyperplasia, mucosal bleeding, and subconjunctival hemorrhages. Serial serum ascorbic acid levels were found to be extremely low (<10 micromol/L) despite routine supplementation both in her dialysate and via regular oral supplementation. Histopathology from skin biopsy revealed purpura, hyper- and parakeratosis, and follicular plugging. She had ECG and 2D echocardiogram disturbances, as well as osteopenia and sclerosis of the extremities on radiological evaluations. Therapy with high-dose ascorbic acid (1 g/day orally) led to complete resolution of skin lesions. This case highlights the importance of awareness and recognition of this historic diagnosis, and particularly in children with end-stage organ disease with severely compromised nutrition.