Mucormycosis of the scalp is a rare cutaneous presentation of the disease. It is also an unusual infection in children. We present the case of a 4-year-old girl with acute lymphoblastic leukemia, who presented with atypical cutaneous mucormycosis simulating an ecthyma gangrenosum lesion. Risk factors for the infection are diabetes, neoplastic diseases, immunosuppression in organ transplant recipients, and neutropenia. The cutaneos forms have been associated with trauma, burns and surgical wounds. First line treatment is amphotericin B. Posaconazole was recently approved to treat invasive mucormycosis. Surgical removal of the infected tissue is indicated.
BACKGROUND:Topical corticosteroids have become available as over the counter drugs and are widely misused for various conditions.OBJECTIVE:The aim of this study is to assess the clinical and epidemiological aspects of the unjustified use of topical corticosteroids for facial skin.METHODS:A total of 200 patients with facial dermatoses and topical corticosteroid misapplication daily over face for not less than 30 days were included in the study. This was a prospective study conducted in a tertiary care dermatology outpatient centre of the Jammu region. A detailed clinical history regarding topical corticosteroid use was taken and adverse effects analysed.RESULTS:A total of 166 patients were women and 34 were men. The predominant age was 31-40 years. A total of 170 patients (85%) were in the age group of 21-50 years. Duration of application was over 1 month up to 3 years, daily. Betamethasone or clobetasol ointments were used in 75 patients (37.5%) and momatasone was used in 15 patients (7.5%). Indication for using steroids were: general / cosmetic purposes (72 patients; 36.0%), acne (59; 29.5%), hyperpigmentation (41; 20.5%), tinea (6; 3%), undiagnosed dermatoses (28; 14.0%). The use of corticosteroids was attributed to the advice of pharmacists (69; 34.5%), friends and relatives (61; 30.5%), cosmetologists (22; 11.0%), non-dermatology physicians (30; 15.0%) and dermatologists (18; 9%). Adverse effects included acneiform lesions, telengiectasias, dyspigmentation, hypertrichosis, perioral dermatitis and tinea incognito. A total of 89 (44.5%) patients fulfilled the criteria of "topical steroid dependent face". These patients reported erythema, burning and itching on stopping the application of topical corticosteroids.CONCLUSION:In most cases the use prolonged use of topical corticosteroids on facial skin was recommended by non-professional persons. The adverse events ranged from transient to permanent. The results of this study underline the indispensable role of dermatology specialists in diagnosing and treating cutaneous disorders.
BACKGROUND Skin metastases occur in 0.7% to 9% of all patients with cancer and are usually considered a late event in the evolution of most visceral carcinomas. The development of a nodular metastatic lesion on the nasal tip is known as clown nose sign. MAIN OBSERVATION We report a 64-year-old man that developed a nodular lesion on his nasal tip as first manifestation of squamous lung carcinoma. CONCLUSION The biopsy of the cutaneous metastasis may be helpful to histopathologically confirm the suspected primary tumour avoiding invasive diagnostic procedures.
Background: Grover's disease is a pruritic papular dermatosis of transient nature, mainly located in the thorax and abdomen. The etiology is undefined and it has been occasionally associated with cases of immunosuppression. Histopathology is not specific and patterns of other dermatoses may be found. Main observations: We report a rare case of a 58-year-old liver transplant patient with unilateral Grover's disease. Conclusions: It may be suspected that Grover's disease was triggered in our patient by immunosuppressive therapy. Our case suggests that Grover's disease has to be considered as a differential diagnosis to dermatoses that are present along the Blaschko lines.P
Background: Kimura's disease is a rare, chronic inflammatory disease, which typically affects young Asian men. The disease is characterized by the triad of: lymphadenopathy of the head and neck, eosinophilia and increased serum IgE. The etiology of the disease is unknown.Main observations: We present a case of this disease in 25-year-old Caucasian man with enlarged, cohesive and painless lymph nodes in the submandibular, axillary and supraclavian region. Previous histopathology results indicated a reactive inflammatory process. Detailed anamnesis revealed contacts with Asian and eating original Chinese food 3 years ago. Laboratory results showed eosynophilia (11%) and elevated IgE level (211,2 IU/ml).Conclusion: The diagnosis of Kimura's disease can be very difficult and misleading. During differential diagnosis chronic inflammatory diseases (e.g. tuberculosis), malignances (e.g. acute lymphocytic leukemia, T-cell lymphoma, Kaposi's sarcoma, Hodgkin's disease, or cancer of the salivary glands), lymph node metastases, granulomatosus diseases and angiolymphoid hyperplasia with eosinophilia have to be taken into consideration. It may be hypothesized that contacts with persons of Asian origin or eating original Chinese food may have some role in the development of the disease.
Background: Frontal fibrosing alopecia (FFA) is a primary scarring alopecia characterized by frontotemporoparietal hairline recession with associated clinical findings crucial for diagnosis and which may be linked to severity and prognosis. Objective: To evaluate clinical-trichoscopic-histological features of a series of FFA cases comparing with published literature. Methods: Clinical, trichoscopic and histological features of FFA cases diagnosed at a single Dermatology Center from 2009 to 2016 were retrospectively reviewed in order to seek for possible correlations. Severity was classified by the largest measure of hairline recession. Results: One-hundred and eight patients were enrolled including 105 females and 3 males with a mean age of 57.8 years. A mild-moderate frontal hairline recession prevailed (53.7%). Premenopausal females represented 26.8% of our sample. Eyebrow loss was depicted in 89.8% being the first noticed sign in 40.7%. Facial papules were observed in 57.4% of patients more commonly found in premenopausal compared with postmenopausal females (P=0.023); they often exhibited histological sebaceous gland hypertrophy. Independent factors for severe FFA include visible frontal veins (OR 4.15; P=0.001), trichoscopic perifollicular erythema and scaling (OR 3.91; P=0.021) and ivory white patches (OR 3.68; P=0.008). Conclusions: Our results support an emergent burden of FFA with an increasing reported prevalence within premenopausal females. Facial papules described as part of the clinical spectrum of FFA were frequently found and especially common among premenopausal females. Trichoscopy represents an important aid in the diagnosis and may herald FFA severity. Treatment is still elusive although antiandrogens, usually combined with topical anti-inflammatory therapies, display a moderate effect.
Bullous lichen planus is a rare variant of lichen planus. It is characterized by vesicles or bullae, which usually develop in the context of pre-existing LP lesions. It is often misdiagnosed and should be differentiated from other subepidermal bullous diseases especially lichen planus pemphigoides. The diagnosis is based on clinical suspicion and is confirmed by histopathology and immunofluoresence. The clinical features of bullous lichen planus include typical lichen planus lesions, accompanied by the formation of bullae on the affected or perilesional skin. This is evident on histology, with alteration of the dermo-epidermal junction and intrabasal bullae as a consequence of extensive inflammation. The histologic features in conjunction with the negative immunofluoresence indicate that bullous lichen planus is a form of "hyper-reactive lichen planus" rather than a distinct entity. There is no standard treatment of bullous lichen planus. Topical and systemic corticosteroids, dapsone and acitretin have been described as effective choices.
Background: Oral lichen planus is a chronic inflammatory disease of unknown etiology.It is characterized by notable heterogeneity in clinical presentation and behavior. Main observations:We describe 3 patients with lichenoid lesions of the upper lip and upper anterior gingiva.These cases probably represent a clinical subtype of lichenoid lesions associated with microbial antigenicity and are characterized by persistency.All three patients had a long history of previous ineffective treatments and all three showed an excellent response to a combination of clarithromycin (2 x 250 mg/day) and prednisolone (15 mg/day). Conclusion:The combination of clarithromycin and prednisolone may be considered as an alternative intervention in patients with lichenoid lesions of the upper lip and upper anterior gingiva.
Introduction: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant genodermatosis characterized by cutaneous fibrofolliculomas and/or trichodiscomas, lung cysts, spontaneous pneumothorax and renal tumors.However, its clinical expression is highly variable.This syndrome is caused by germline mutations in the folliculin gene (FLCN) on chromosome 17p11.2.Main observations: Two men, 60 and 39-year-old, presented with a several year history of asymptomatic whitish papules scattered over the face and neck.Skin biopsies revealed fibrofolliculomas.The clinical diagnosis of BHDS was corroborated by identification of new heterozygotic mutations in FLCN gene, in exon 6 (C.573_574delinsT) and in exon 9 (c.1015C>T), respectively.Computed tomography scan of the thorax and abdomen showed pulmonary cysts with no suspicious kidneys lesions, and, in the case of the second patient, a mass in left adrenal gland.Laparoscopic left adrenalectomy was performed and histopathological examination was compatible with a malignant perivascular epithelioid cell tumor. Conclusions:The presence of multiple fibrofolliculomas should raise the suspicion of BHDS.Patients with this syndrome, regardless of the detected mutation, should be carefully monitored to ensure that potentially serious disease-related conditions can be detected early.(
Background Apremilast is a new immunomodulatory drug, a small molecule inhibitor of PDE4, which down-regulates the expression of multiple pro-inflammatory cytokines, such as tumor necrosis factor alpha, interleukin 17, interleukin 23. Main observations We describe a case of a 54-year-old man with erythroderma in the course of psoriasis (PASI=49), with contraindications to other psoriasis therapies, in whom total clearance of skin lesions was achieved by day 20 after therapy with apremilast at a dose of 30 mg bid (ΔPASI = 100). The patient had a history of prior use of cyclosporine, methotrexate and adalimumab. His comorbidities included obesity, fatty liver and hypercholesterolemia. Conclusion In this case of erythroderma in the course of psoriasis apremilast led to total clearance of all cutaneous lesions.
Background Necrotizing vasculitides are basically characterized by vessel wall neutrophil infiltration and necrosis and they can occur as a primary process or secondary to an underlying disease. Although Henoch-Schönlein purpura (HSp) is the more frequent primary vasculitis in childhood, sometimes it has to be distinguished from other secondary vasculitides induced by infections, drugs, vaccines, or immune-mediated disorders. Main observations We report a case of a 14-year-old girl with cutaneous necrotizing vasculitis, appearing in the course of acute Epstein-Barr virus infection. Physical examination revealed highly aching erythematous-purple lesions with reticular edges localized on the back of feet. Pain was non-responsive to ibuprofen and required administration of tapentadol and pregabalin. The patient was also heterozygous for factor V Leiden that might have contributed to the development of cutaneous painful lesions. Conclusions To our knowledge this is the first documented pediatric case of necrotizing vasculitis associated with acute EBV infection in a girl heterozygous for factor V Leiden. In this patient the severity of skin manifestations might have been influenced by the concomitant factor V Leiden, which gave rise to hypercoagulability and occlusive vasculopathy with markedly severe pain, a symptom rather infrequent in other childhood vasculitides.
BACKGROUND:Facial hypermelanosis is a significant cause of cosmetic disfigurement, social embarrassment and psychological morbidity affecting quality of life.OBJECTIVE:To study clinicoepidemlogic patterns of facial hypermelanoses among men.MATERIAL AND METHODS:Medical records of all adult males presenting with facial hypermelanoses were analyzed for this retrospective cross sectional study for demographic details, duration, cosmetic usage, sun exposure, drug intake, infections, systemic or cutaneous diseases, and family history of hypermelanotic dermatosis. Laboratory investigations and skin biopsy were performed when deemed necessary.RESULTS:These were 300 Indian men aged 18 to 74 (mean 37.35) years with 121 (40.3%) individuals aged 31-50 years. Various patterns of melasma in 230 (76.7%) patients were the major cause of facial hypermelanosis. Periorbital hypermelanosis was observed in 32 (10.7%), freckles and lentigens in 26 (8.7%), acanthosis nigricans in 12 (4%) and lichen planus pigmentosus in 10 (3.3%), pigmented cosmetic contact dermatitis in 7, and nevus of Ota in 6 persons. The 71 (30.8%) patients with melasma had a history of frequent sun exposure, 9 (3.9%) patients had systemic comorbidities. Family history of periorbital melanosis was present in 7 (21.8%), personal or family history of atopy in 5 (15.6%) patients. Acanthosis nigricans was associated with obesity in 9 (75%) of patients and with diabetes mellitus in 4 (33.3%) cases.CONCLUSIONS:Melasma, periorbital hypermelanosis, acanthosis nigricans and lichen planus pigmentosus remain the predominant causes for facial hypermelanosis in men.
Postradiation sarcomas are rare and highly malignant tumors which may appear as a consequence of radiotherapy. They may originate on bone or soft tissues.We report the case of a patient who developed a malignant fibrous histiocytoma 35 years after radiotherapy for a melanoma on her right leg.
Background: Aquagenic keratoderma is a dermatosis characterized by transient whitish and transluscent hyperwrinkling after water exposure.The aim of the current report was to present a sporadic and familial cases of aquagenic keratoderma.Observation: Sporadic Case: A 38-year-old female patient presented with eruption in the right hand after exposure to water.The patient was placed on systemic acitretin therapy with the diagnosis of idiopathic acquired aquagenic keratoderma.No recurrence occurred during a 6-month follow-up period.Familial Cases: A 55-year-old male patient, who was engaged in fishery, presented to the outpatient clinics of the department of dermatology due to whitish vesicles in the palms of both hands.It was realized that the father, sister, and brother of the patient had similar complaints.The cases were thought to have familial aquagenic keratoderma; however acitretin therapy could not be initiated due to elevated alanine aminotransferase and triglyceride levels.Topical application of salicylic acid 10% and 10% urea containing lotions was effective but did not prevent recurrence.Conclusion: Systemic acitretin may be an effective agent in the treatment of aquagenic keratoderma, and topical application of 10% salicylic acid and 10% urea-containing lotion did not prevent recurrence.(
BACKGROUND:Pyoderma gangrenosum is a rare, ulcerative, destructive, non-infectious dermatologic disease and it is one clinical entity within the spectrum of neutrophilic dermatoses. Visceral involvement, manifesting as sterile neutrophilic infiltrates in sites other than skin and, is infrequent. Splenic involvement is very rare.MAIN OBSERVATIONS:We present a case of a 58-year-old woman with pyoderma gangrenosum with spleen involvement and review all reports of similar cases.We have found nine reported cases, our case being the tenth.CONCLUSION:Our review showed that spleen involvement in the course of pyoderma gangrenosum can occur at any age. It is slightly more frequent in men. An underlying or associated neutrophilic disorder is present in almost half of the patients. Skin manifestations were usually present before splenic involvement. In most cases the disese responds well to glucocorticosteroids.
Background: Epidermolysis bullosa simplex associated with muscular dystrophy is a genetic skin disease caused by plectin deficiency.A case of a 19-year-old Czech patient affected with this disease and a review all previously published clinical cases are presented.Main observations: In our patient, skin signs of the disease developed after birth.Bilateral ptosis at the age of 8 years was considered as the first specific symptom of muscular dystrophy.Since then, severe scoliosis, urological and psychiatric complication have quickly developed.The signs of plectin deficiency were found by histopathological studies, electron microscopy and antigen mapping of the skin and muscular samples.Two autosomal recessive mutations in the plectin gene leading to premature termination codon were disclosed by mutation analysis.By review of all published clinical cases, 49 patients with this disease were found.54 different mutations in the plectin gene were published, p.(Arg2319*) in exon 31 being the most frequently found.Median age of muscular dystrophy development was 9.5 years.Hoarseness and respiratory complications were the most often complications beside skin involvement.Conclusion: Epidermolysis bullosa simplex with muscular dystrophy was diagnosed based on clinical, histopathological (skin and muscle biopsy) and mutation analysis of the plectin gene.Overview of the genetic and clinical characteristic of this disease could be presented by review of all previously published clinical cases.(
BACKGROUND:Erythroderma is an uncommon and severe dermatological manifestation of a variety of diseases. It is commonly challenging to find the underlying cause.OBJECTIVE:The aim of this study was to analyze the causes of the disease in patients with erythroderma.PATIENTS AND METHODS:Data including the clinical symptoms, laboratory examinations, histopathology and follow-up information were collected from patients with erythroderma admitted to our department between 2000 and 2010.RESULTS:One-hundred and three patients diagnosed with erythroderma were identified during this period (11.9% of all hospitalized patients; hospital incidence = 9.4 cases/year). The mean age of onset was 54.4 years (range: 17-89 years) with a male:female ratio of 1.5:1. The most frequent cause of erythroderma was exacerbation of preexisting dermatoses (65.0%), including psoriasis (44.7%) and eczema (16.5%). Drugs (18.4%) and cutaneous T-cell lymphomas (11.7%) induced most of the remaining cases. No cause could be identified in four cases (3.9%). Apart from erythema and scaling, that were present in all patients, clinical findings were dominated by pruritus (97.1%), followed by edema (56.3%), fever (54.4%), palmoplantar keratoderma (50.5%), nail changes (42.7%), liver or spleen enlargement (41.7%) and lymphadenopathy (40.8%).CONCLUSIONS:Although numerous clinical features and laboratory values were abnormal, most findings were non-specific. The skin biopsy yielded a positive clinical correlation in most cases. Our study had a high percentage of erythroderma secondary to preexisting skin disease and a relatively low percentage of idiopathic erythroderma.
BACKGROUND/OBJECTIVES:Systemic sclerosis is a connective tissue disease, which is characterized by fibrosis of the skin and internal organs, presence of specific antibodies and vascular involvement. Capillaroscopy is a useful method for the diagnosis and follow-up of patients with systemic sclerosis. Trichoscopy is a rapid, non-invasive technique, which has become a standard procedure in differential diagnosis of scalp and hair diseases. The aim of this study was to assess whether trichoscopy may be applied in imaging microvessels in patients with systemic sclerosis.METHODS:The study included 17 patients with systemic sclerosis, and 31 healthy patients. In every patient 10 trichoscopy images were taken with Fotofinder II.RESULTS:In patients with systemic sclerosis trichoscopy of the frontal scalp area revealed polymorphic microvessels in 64,7% of patients, spider vessels (76,4%), capillary loops (52,9%), arborising vessels (41,1%) and avascular areas (35,2%). In healthy individuals these features were observed in polymorphic microvessels 6,4% of patients, spider vessels 6,4%, capillary loops 100%, arborising vessels 16,1%, avascular areas 9,6%, respectively.CONCLUSIONS:In conclusion, the presence of polymorphic vessels in frontal area in trichoscopy is characteristic for systemic sclerosis.
Background: Acrodermatitis enteropathica (AE) is a rare autosomal recessive metabolic disorder. First described by Brandt in 1936 and was named by Danbolt. A mutation in the SLC39A4 gene on chromosome 8 q24.3 is responsible for this disorder, which encodes zinc transporter Zip4. The diagnosis is made by the clinical presentation and histopathology and laboratory tests. In this case, we reported a twin presented with a typical rash and low zinc level. To our knowledge, very few cases reported as a twin with typical acrodermatitis enteropathica presentation.Main observations: Four months old twins both females, first children of a non-consanguineous marriage. The twins were born at term, caesarian section, with no complications. Presented with erythema, scaling, crusting and oozing over perioral, perianal areas, hands and feet of 2-3 week duration. The lesions started around the same time for both children with a history of intermittent diarrhea, and hair loss. There were no nail changes or neurological deficit or myopathy. There was a history of recent weaning from breast milk and now both children on formula feeds, ragi, fruits. There was no other significant history of other medical problems in the patients or in their family. On examination, erythema, scaling, crusting and oozing over perioral, perianal areas, hands and feet was seen. Minimal diffuse alopecia was noted. Nails were normal. No other abnormalities were observed. Clinical diagnosis of acrodermatitis enteropathica was considered and confirmed by low zinc levels (repeated plasma zinc levels were below 0.6 mcg/ml). The twins were managed with zinc supplementation 1 mg/kg/day. A significant improvement was seen within two weeks.Conclusions: Early diagnosis of acrodermatitis enteropathica is essential for preventing complications. We report a rare case of typical clinical presentation of the disease developing simultaneously in twins.
BACKGROUNDBullous lichen planus (BLP) is a rare variant of lichen planus, characterized by the development of vesicular and bullous lesions, of skin, nails, hair and/or mucosa.MAIN OBSERVATIONSWe present a case of 63-year-old woman with BLP, unresponsive to previous therapies with topical corticosteroids, topical calcipotriol, antihistamines and oral cyclosporine (4 mg/kg/day for 4 months). She was already receiving treatment for arterial hypertension, hyperlipidemia, atrial fibrillation and uncontrolled diabetes mellitus. Acitretin was administered for 5 months with complete remission of BLP lesions and no major side effects.CONCLUSIONSThis is probably the first reported case of BLP treated with acitretin monotherapy. In this case acitretin was an efficacious and well-tolerated therapeutic option for BLP.
