Search

Oral acute graft-versus-host disease (aGVHD) is a significant sequelae of allogeneic hematopoietic stem cell transplantation (HSCT). Presently, transplant physicians have to diagnose GVHD based on clinical judgment by interpreting available clinical and relevant laboratory findings. As such, characterization of diagnostic and distinctive clinical signs and symptoms of GVHD is essential for diagnosis and grading. The oral features of aGVHD have been reported infrequently and remain ill defined, unlike in oral chronic GVHD. The report describes an atypical and painful presentation of oral aGVHD in a 15-year-old boy, 16 days after haploidentical HSCT, who presented with swollen lips, herpetiform ulcerations, and erythematous fungiform papilla.

Background Only a few studies characterized cutaneous non-tuberculous Mycobacterium (NTM) infections in this region of the world. Objective The aim of this study was to describe the epidemiological, clinical and histological findings of cutaneous NTM infections in Lebanon. Patients/Methods Retrospective study of 17 patients (19 histological specimens) diagnosed with cutaneous NTM infections and confirmed by culture-based partial sequencing of the 16S rRNA gene at the American University of Beirut Medical Center between 2005 and 2008. Results Of 17 cases, 14 were caused by Mycobacterium marinum. All patients were immunocompetent except for one. Clinically, the most common presentation was multiple sporotrichoid lesions over an extremity (8/17). Many patients had peculiar presentations including bruise-like patches, herpetiform lesions, annular ulcerated plaques, symmetrical nodules over the buttocks and locally disseminated lesions with surrounding pale halo. Almost all patients cleared their infection on either minocycline or clarithromycin monotherapies. Histologically, a dermal small vessel proliferation with mixed inflammation (granulation tissue-like changes) was identified in 58% of specimens. The most common type of granulomatous inflammation was the suppurative (47%) followed by the tuberculoid (30%), sarcoidal (11%), and palisading (5%) types. Lichenoid granulomatous dermatitis was noted in 42% of cases. Special staining highlighted mycobacteria in only two specimens. Conclusions The incidence of cutaneous NTM infections is high in our area. Many patients had peculiar clinical presentations. Our study is the second to report the common presence of granulation tissue-like changes as a good histological indicator of cutaneous NTM infections. Minocycline and clarithromycin remain the drugs of choice in our area.

A case with Epidermolysis bullosa simplex Dowling-Meara is presented. It concerns a 20 days breast-fed girl with severe blistering and erosions on the skin formed at birth after minor mechanical trauma. There are no other affected relatives. A medical examination showed normal somatic and visceral status. There were multiple herpetiform grouped bullous and erosive lesions on the face, trunk, upper and lower limbs, palms and soles. The mucous membranes were not affected. There was also a marked nail dystrophy on the fingers and toenails. The electron microscopic examination revealed cytolysis of the basal keratinocytes with clumping of the perinuclear tonofilaments in the lesional skin. Because of the clinical features and the results of the electron microscopic examination of the skin the case here reported should be considered as an Epidermolysis bullosa simplex Dowling-Meara. The child is followed up to the age of three. The course of the disease was benign with a decreased formation of blisters and erosions. The marked nail dystrophy was persistent. There was palmar and plantar hyperkeratosis. The child’s growth, neural and psychic development wsas normal. The early diagnosis of the bullous epidermolysis is helpful in the prognostic assessment of the disease and in the medical and genetic advice for the parents.

A 25-year-old man, known to have chronic myeloid leukaemia for four years, acutely developed a fever of 39.5 degrees and severe pain in the shoulder and hip joints. There was no evidence of joint disease. Treatment with indomethacin briefly improved the symptoms, but within 72 hours the patient developed a fulminant illness with high fever and clinical as well as biochemical signs of a severe consumption coagulopathy. Herpetiform efflorescences appeared over the head and trunk shortly before death. At autopsy there was histological and immunohistochemical evidence of a varicella infection with hepatitis, oesophageal involvement and severe internal organ bleedings. In immune-compromised patients with atypical prodromal symptoms a varicella infection must be considered so that causal treatment with acyclovir and hyperimmune-globulin can be begun in time.

A 14-month-old heifer with a 17-day history of unresponsive bloody diarrhea was necropsied. There were focal, pink-red erosions of the nares and hard palate; ulcers and fissures of the tongue; and multiple ulcerative lesions of the alimentary canal. Interdigital skin of both rear limbs was ulcerated and bleeding; and the margins of the vulva contained punctiform red ulcers. The gross lesions were consistent with mucosal disease. Histopathology and laboratory testing ruled out rinderpest, foot-and-mouth disease, and vesicular stomatitis, and identified bovine virus diarrhea virus to be the cause of this disease. Lesions of the vulva similar to those seen in some stages of infectious pustular vulvovaginitis were negative for bovine herpesvirus-1 and tested positive for bovine viral diarrhea virus antigen by immunohistochemistry.

Behcet's disease (BD) is a multisystem disease, in which recurrent aphthous stomatitis (RAS) is a universal finding. We studied the expression of RAS in patients with BD, and the correlation between major or minor RAS and systemic expression and severity of the disease. Thirty-five patients with BD were studied, of whom 13 (37%) had major, 21 (60%) had minor and one (3%) had herpetiform RAS. The frequency of major RAS was significantly higher compared with a control group of patients with idiopathic RAS (37% vs 9%, P < 0.05). The BD patients with major RAS had significantly more relapses of oral ulceration in a year, higher numbers of oral ulcers per relapse, and longer duration of aphthous episodes, compared with patients with minor RAS. Oral ulcers also appeared at a significantly younger age in patients with major than with minor RAS. However, the systemic expression of the disease, as well as the disease severity score, were similar in patients with major and minor RAS. The results of this study indicate that major RAS is common in patients with BD, and is associated with a more severe, repeated and prolonged oral disease. Nevertheless, the presence of major RAS in BD does not predict a more severe systemic illness.

We describe a highly aggressive squamous cell carcinoma that presents in a dermatome pattern shortly following shingles and review the literature on herpetiform lesions.

Herpes zoster is a secondary reactivation of primary contagious varicella-zoster virus in the dorsal root ganglia. While thoracic zona is common, cervical dermatomal zona is a rare segmental complication of herpes zoster and can be easily misdiagnosed as other diseases. This article describes a patient with initial neuralgia without dermatomal lesions that was treated as ulnar nerve entrapment syndrome until manifestation of herpetiform cutaneous lesions appeared. It is important that clinicians should be aware of the possibility of zoster infection when evaluating the onset of neuralgia in a dermatomal distribution in the upper limb, especially without rash.

Background and objective: Recurrent aphthous ulceration is the most common oral mucosal disease known. It presents as three types: minor (most prevalent), major and herpetiform. However, there are no well established effective and reliable treatments of this condition. Alchemilla vulgaris (Lady’s Mantle) has traditionally been used in oral hygiene and was recently shown to accelerate wound healing when used in combination with glycerine. The objective of this study was to determine whether this combination is effective in the treatment of the most prevalent form of aphthous ulcers. Methods: An open-label study was conducted in 48 otherwise healthy male and female patients aged 4–44 years to determine the putative healing properties and tolerability of a standard 3% extract of A. vulgaris in glycerine (Aphtarine®) on common minor oral ulcers. Patients with major or herpetiform ulcers were excluded from the study. Results: Topical application three times daily of Aphtarine® gel to minor mouth ulcers relieved discomfort and produced complete healing in the majority of patients (60.4%) within 2 days and in 75% within 3 days, compared with 10.4% and 33.3%, respectively, without treatment and 15% and 40%, respectively, with commonly available treatments. Most patients appreciated the product’s ease of application, taste and texture. Aphtarine® was well tolerated locally and most patients rated the product good to excellent overall. Conclusion: Aphtarine® is a safe, well tolerated and highly effective promising new treatment for healing common mouth ulcers.

The clinical manifestations of syphilis are variable in appearance and have been described for centuries. The disease has been arbitrarily divided mainly into three stages. Uncommon presentations of syphilis in adults include (a) primary syphilis—atypical forms of chancre vary in size, shape, morphology, and color. Small ulcus durum is single or multiple, grouped, or herpetiform. Giant necrotic and phagedenic chancres are resolved with scar formation. In intratriginous areas, ulcus durum is rhagadiform, linear, brocket type,Q or bilateral. (b) Secondary syphilids include macular (roseolas, leukomelanoderma), papular (small miliar or lichenoid, or with large size—lenticular or nummular), papulosquamous, syphilis cornee, psoriasiform, annular en cockade, nodular, condylomata lata, malignant syphilis, and others; there are also mucosal lesions, loss of the hairs, and alteration of the nails. (c) Tertiary syphilis occurs decades after infection in three main forms: gummatous, cardiovascular, and neurosyphilis (asymptomatic, meningeal, meningovascular, and parenchyma- tous—such as general paresis or tabes dorsalis). Early recognition of the clinical manifestations of syphilis is important for the start of treatment, recovery of patients, and the prevention of the spread of disease.

Summary The immunodeficiency state in HIV infected patients has been the cause of severe episodes of Recurrent Aphthous Stomatitis (RAS). Aim : Our study aims to establish correlation between the manifestations of RAS and the immunosuppression state caused by HIV infection, through counting of CD4+ cells, CD8+ cells, CD4+:CD8+ cells ratio and viral load. Study design: series study. Material and Method : Ninety-four HIV infected patients (25 women and 69 men) with RAS were evaluated in the ENT Department of the University of Sao Paulo-Medical School from January 1998 to December 2003. The age ranged between 19 and 63 years (mean = 35.3 years). The patients were divided in two groups: AIDS group and HIV infected group. Results : The patients with AIDS and HIV infection presented, respectively, eight ulcers and two ulcers by outbreaks. Similarly, patients with major RAS presented smaller counting of cells CD8+, CD4+ and CD4+/CD8+ cells, and higher mean value of viral load than the patients with herpetiform and minor RAS. Between patients with minor and herpetiform RAS there were no statistical differences. Conclusions : The emergence of the lesions, mainly in major RAS, is directly related to the immunological state of the HIV infected patient. These patients frequently present nutritional deficits and worsening in life style. Thus, diagnosis and treatment of RAS is a challenge that should not be neglected.

Mutations in genes keratin 5 (KRT5) and 14 (KRT14) encoding the basal type keratin intermediate filaments have been identified in epidermolysis bullosa simplex (EBS) families and are likely to cause skin fragility. Three novel keratin 14 mutations in cases from the Hungarian Epidermolysis Bullosa Centre are reported. In a 7-year-old boy with Dowling-Meara type EBS (DM-EBS), who had severe skin symptoms with extended herpetiform blisters, a novel amino acid substitution N123K in keratin 14 had been detected. A 26-year-old woman with mild DM-EBS with prominent palmoplantar hyperkeratosis and without active blister formation had a novel R125G mutation in keratin 14. In a 6-year-old girl, with Weber-Cockayne type EBS (WC-EBS) with palmoplantar blisters and moderate mental retardation, a novel V133L substitution was detected. Her pedigree showed autosomal dominant mode of inheritance; in the two other families, only the index patients were affected. The N123K and R125G mutations causing DM-EBS phenotypes are located within the helix initiation motif of the rod domain, whereas the very close V133L mutation underlying the WC-EBS phenotype is outside of this region. These novel amino acid substitutions provide further information for genotype–phenotype correlation in KRT14 mutations, and demonstrate the first molecular genetic data in EBS patients from Hungary.

Recurrent aphthous stomatitis (RAS) is the most common oral mucosal disorder found in men and women of all ages, races, and geographic regions. There are three forms of the lesions (minor, major, and herpetiform), with major aphthous ulcers causing significant pain and potential for scarring. In HIV-infected individuals, these ulcers occur more frequently, last longer, and produce more painful symptoms than in immunocompetent persons. In addition, they may be associated with similar ulcerations involving the esophagus, rectum, anus, and genitals. The diagnosis of HIV-induced RAS requires a careful history of the condition, and a thorough extra- and intra-oral examination. Oral mucosal biopsies are required for non-healing ulcers in order to exclude the possibility of deep fungal infections, viral infections, and neoplasms. The cause of the ulcers in HIV-positive persons has not been elucidated--local diseases, genetic, immunologic, and infectious factors all probably play a role. The goals of current treatments are to promote ulcer healing, to reduce ulcer duration and pain while maintaining nutritional intake, and to prevent or diminish the frequency of recurrence. Initial therapy for infrequent RAS recurrences includes over-the-counter topical protective and analgesic products. Initial therapy for frequent RAS outbreaks requires topical anesthetics, binding agents, and corticosteroids. Major RAS and non-healing minor or herpetiform RAS may require intralesional corticosteroids and systemic prednisone. Second-line immunomodulators for frequent and non-healing ulcers includes thalidomide and other immunomodulators.

Irritant contact dermatitis is a common cause of chronic vulvitis in patients wearing diapers and incontinence garments. In most cases the diagnosis is obvious; however, atypical presentations may mimic more serious dermatoses. We present a 4-year-old girl who presented at birth with cloacal atresia corrected surgically and resulting in chronic incontinence requiring full-time diapers. She presented with crops of herpetiform vesicles and bullae on a base that ranged from normal skin to severe erythema and oedema. A histological examination revealed a well-demarcated lesion showing a thickened epidermis with hyperkeratosis and parakeratosis, acanthosis and an abrupt transition to pallor of the upper half. Focal full thickness epidermis necrosis and small areas of spongiosis, acantholysis and apoptotic keratinocytes were seen. Immunofluorescence was negative. The lesions improved with the treatment of secondary infection and minimal topical therapy with an emollient only. This case represents an unusual vesicular variant of pseudoverrucous papules and nodules, which has been reported only once previously.

Introduction Presuming that psoriasis is an autoimmune skin disease, the aim of this study was to establish its association with other autoimmune skin diseases. The material was obtained at the Dermatovenereological Clinic Clinical Center Novi Sad. Material and methods This 10-year retrospective study (1990-1999) included 1743 psoriasis patients. The control group consisted of 7492 nonpsoriatic dermatological patients. Results Association of psoriasis with other dermatological diseases of autoimmune nature has been established in 13 (0.74 %) patients. The most frequent association was with lichen ruber planus in five patients, with alopecia areata and vitiligo in three patients, and in one with bullous pemphigoid and herpetiform dermatitis. Using Fisher's test no significant association was established. Discussion and conclusion According to literature data association of psoriasis with other autoimmune diseases is well known, but rare, which is in accordance with our results. The question arises whether this association is the matter of poor coexistence or the matter of genetic mutations. However, once established, these associations can further highlight the autoimmune nature of psoriasis. The research of autoimmunity would lead us to epithelial cells in thymus, and their badly learnt cognitive function about what is own, and what is not.

A 46 year old woman who had been diagnosed with breast cancer two years previously noticed an asymptomatic rash on her left anterior …

Oral aphthous ulcers typically present as painful, sharply circumscribed fibrin-covered mucosal defects with a hyperemic border. Chronic recurrent oral aphthous ulcers occur in three different clinical morphological variants and with two different time courses. Small ulcers of the minor-type (Mikulicz) are less than 1 cm in diameter (usually 2–5 mm) and heal spontaneously in 4–14 days. They account for 80–90% of all recurrent oral aphthous ulcers (1, e1). Scarring occurs in around 8% of cases (1, e2) (Figure 1a). Large ulcers of the major-type (Sutton ulcers) are usually 1–3 cm in diameter, deeply indurated and can last for 10 days to 6 weeks or occasionally even longer (1, e3) (Figure 1b). They account for around 10% of recurrent benign oral ulcers. About 64% of Sutton ulcers heal with scarring. Herpetiform aphthous ulcers are very small (1–2 mm) grouped lesions (1, e4) (Figure 1c). They account for around 5% of recurrent oral aphthous ulcers, are extremely painful and persist for 7–10 days. As many as a 100 ulcers can be present; they may coalesce into larger erosive plagues and about 32% heal with scarring. The three morphologic variants can occasionally appear simultaneously (2). Figure 1 (a) Minor-type oral aphthous ulcers, (b) major-type oral aphthous ulcer, (c) herpetiform oral aphthous ulcers Another classification is based on the time course. The simple chronic recurrent oral aphthous ulcers present with a limited number of small, quickly healing, minimally painful ulcers limited to the oral mucosa and recurring with 3–6 episodes annually. In complex aphthosis, there are a few or many slowly healing intensely painful ulcers on the oral and perhaps genital mucosa (3). The latter may also be perigenital, affecting the scrotum, vulva, anus, perineum and inguinal region. Complex aphthosis features frequently appearing ulcers with either short lesion-free periods or even repeatedly recurrent ulcers, severe pain and even systemic effects such as interference with eating and the resultant problems of inadequate nutrition (3).

A 43-year-old woman presented a 2-year history of tender blisters and erythematous and crusted excoriations on the face, trunk and proximal extremities. During her first examination in 1995, erosions and yellow crusts on her face, thorax, upper torso and proximal limbs were present, without mucosal involvement. Laboratory profile confirmed the diagnosis of pemphigus foliaceus, as follows: histopathological analysis exhibited subcorneal abscess with mild spongiosis and scanty acantholytic cells; neutrophilic and linfohystiocitic perivascular infiltrate was present in the superficial dermis; direct immunofluorescence showed intercellular IgG deposits within the entire epidermis; indirect immunofluorescence revealed circulating IgG antibodies (titer=1:1,280) and positive ELISA index (99, recombinant desmoglein 1). Treatment with systemic corticosteroid (prednisone 1 mg/kg/day) was initiated. Clinical remission was achieved after 2 months, followed by gradual corticosteroid tapering. The patient presented reactivation when receiving prednisone 10 mg/day, with vesicles with a herpetiform arrangement on erythematous plaques on her legs, with intense pruritus (Fig. 13.1).

WOS: 000215206500041 PubMed ID: 25276457 Ramsay Hunt syndrome (RHS) is characterized by facial paralysis, inner ear dysfunction, periauricular pain, and herpetiform vesicles. The reported incidence in children is 2.7/100,000. The pathogenesis involves the reactivation of latent varicella zoster virus (VZV) in the geniculate ganglion of the facial nerve. The recovery rate is better in children than in adults. This paper discusses a 12-year-old girl with a rare case of peripheral facial paralysis caused by RHS and reviews the literature.

Recurrent aphthous ulcers (RAU) are the most common oral ulcerative disease, affecting 10% to 20% of the population. There are 3 clinical subtypes-minor, major, and herpetiform. Minor aphthous ulcers are the most common subtype, representing 80% to 90% of all recurrent aphthous ulcers. Clinically, RAU present as extremely painful, shallow ulcerations with an erythematous halo on unattached oral mucosa. The primary differential diagnosis is oral herpes simplex. The etiology of RAU is unknown. Topical corticosteroids are the mainstay of therapy.

36歳女性。5年前より全身にそう痒性皮疹および舌の潰瘍が出現した。天疱瘡の診断のもと最大量40mg/日のプレドニゾロン(プレドニン®)内服によって皮疹はコントロールされ,2年前よりステロイド離脱し,小康状態であったが,妊娠4ヵ月頃より皮疹が再燃した。皮疹の臨床像はジューリング疱疹状皮膚炎に類似し,組織学的には基底細胞層直上部に棘融解細胞を伴う表皮内水疱と好酸球性海綿状態を認めた。蛍光抗体直接法では表皮細胞間にIgGとC3の沈着を認めたことから疱疹状天疱瘡と診断した。ヒト表皮抽出物を用いた免疫プロット法では,患者血清は130kDaおよびそれより低分子量の蛋白と反応を示し,またELISA法(Dsg 1, 3ELISA Kit, MBL社)による抗デスモグレインDsg 3抗体および抗Dsg 1抗体のindex値はそれぞれ200, 64であった。プレドニゾロン40 mg/日の内服により皮疹は著明に軽快するとともに血清抗表皮細胞間抗体価も低下した。妊娠36週で出産したが新生児には皮疹,粘膜疹を認めず母体の皮疹の増悪も認められなかった。以後順調にプレドニゾロンの減量が可能で,10ヵ月後再び離脱した。

A 59-year-old man with Duhring's disease (herpetiform dermatitis) developed undulating fever and pulmonary infiltrates soon after sulfapyridine treatment was started. When the drug was discontinued, a positive provocation test unequivocally established the diagnosis of hypersensitivity pneumonia. There was also a cross-reaction with sulfonyldianiline (dapsone). This case illustrates the need of considering a hypersensitivity reaction as the cause of fever and (or) pulmonary infiltration during sulphonamide administration.

A 46 year old patient presented with a since 4 weeks progressive lumbar pain, which radiate to the left ventral thigh down to the knee, since 2 weeks additionally sensory changes in this location. Apart from Crohn’s disease there are no further illnesses known in the patient’s history. The clinical-neurological results show numbness and paresthesia in the left ventral thigh, no paresis and normal deep tendon reflexes. No signs of herpetiform skin disorders. The MRI scan of the lumbar spine showed an hour glass forming gadolinium enhancing mass with intra- and extradural parts between the 2nd and 3rd lumbar vertebrae on the left side ( Fig. 1 , Fig. 2 ), interpretated most likely as a schwannoma. Laboratory chemistry results were without adverse findings. CRP, blood sedimentation rate, ACE, HIV serology, VZV serology and serology of borreliosis were all negative. The liquor findings were also normal, no intrathecal immunglobuline synthesis, no oligoclonal bands. In the following 2 months the symptoms came into complete remission spontaneously. The follow-up- MRI scan also showed surprisingly also a complete remission of the enhancing mass. Discussion The case report describes a typical radicular syndrome, the correlation in the MRI was an hour glass forming enhancing mass, primarily interpretated as a schwannoma. Through the complete clinical remission and remission in MRI, a neoplasma could be ruled out. Possible differential diagnoses are a radiculitis or an atypical intervertrebral disc prolapse. Gadolinum uptake in the MRI and the lack of evidence of the enhancing mass in the MRI at follow-up are arguments against an intervertebral disc prolapse, the normal liquor findings against a radiculitis. That’s why an exact assignment of the symptoms was therefore impossible and can be discussed.

Summary We report a patient with herpetiform pemphigus(HP)who showed reactivity only with pemphigus vulgaris(PV)antigen but not with pemphigus foliaceus(PF)antigen. Direct and indirect immuno- fluorescence revealed keratinocyte cell surface staining in the lower layers of the epidermis, where desmoglein 3 (Dsg3) is expressed. Immunoblot analysis, using ethylenediamine tetra-acetic acid- separated human epidermal extracts, revealed that the patient's serum recognized only a 130-kDa polypeptide which co-migrated with Dsg3. By antigen-specific immunoadsorption studies, using desmoglein 1 (Dsg1) and Dsg3 recombinant protein produced by baculovirus expression system, immunoreactivity of the patient's serum was completely adsorbed by Dsg3 alone, but not by Dsg1. These results indicate that this HP patient produced only anti-Dsg3 autoantibodies and no other autoantibodies against components of the keratinocyte cell surface. HP could be a variant of PV.in addition to PF, with unique clinical and histological features.

We present the case of a 63-year-old woman with herpetiform pemphigus and disseminated esophageal carcinoma which began to manifest themselves simultaneously. Some cases of herpetiform pemphigus associated with neoplasms have previously been reported, but these have primarily been bronchopulmonary. The association of any type of pemphigus with esophageal cancer is very infrequent. We should be alert to the infrequent varieties of pemphigus and atypical bullous diseases, because they sometimes do not meet the diagnostic criteria of paraneoplastic pemphigus, but are the first manifestation of an underlying neoplasm.

A 48-year-old man was referred from Baylor College of Dentistry for evaluation of a 2-month history of large, flaccid, superficial bullae/erosions in the mouth, including the tongue, with subsequent development of blisters on the skin. Examination revealed shallow erosions on his oral mucosa (Figure ​(Figure11) as well as discrete clusters of herpetiform lesions on his lower back (Figure ​(Figure22). He was in good general health otherwise, with no new medications and no family history of similar lesions. A skin biopsy was performed (Figure ​(Figure33). Figure 1 Desquamative gingivitis and superficial erosions on the oral mucosa. Figure 2 Small pruritic herpetiform bullae on the trunk. Figure 3 Skin biopsy demonstrating “tombstoning” of basal keratinocytes and an intraepidermal bulla. Hematoxylin-eosin stain. What is the diagnosis? What treatment options exist? DIAGNOSIS: Pemphigus vulgaris.

Recurrent apthous stomatitis (RAS) is one of the most common oral inflammatory diseases characterised by painful recurrent ulcerations of the orofacial region. The ulcers occur in three clinical forms: minor, major and herpetiform. Several therapies have been advocated to manage these lesions such as topical corticosteroids (triamcinolone acetonide, hydrocortisone acetate and clobetasol propionate), chlorhexidine mouth rinses, tetracycline oral rinses, thalidomide, fluocinonide, colchicines and the immune boosting agent levamosile, vitamin therapy and topical interferon α-2a. Laser therapy is used as an alternative method in treatment of RAS. In this paper one patient with RAS was treated using a 940 nm diode laser for symptomatic relief of pain and burning sensation and healing of ulcer.

A 6-year-old girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmoplantar keratoderma, and improvement with age. An unusual feature was the presence of a striking mottled pigmentation involving the arms, trunk, and legs. Histology, immunofluorescence, and electron microscopy of a fresh lesion showed an intraepidermal split within the basal keratinocytes, focal hyperpigmentation of the basal cells without an inflammatory infiltrate, and tonofilament clumping. Our patient illustrates the clinical features of Dowling-Meara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling-Meara and EBS with mottled pigmentation is discussed.

Recurrent aphthous ulceration or recurrent aphthous stomatitis is the most common oral mucosal disease known to human beings. Despite much clinical and research attention, the causes remain poorly understood, the ulcers are not preventable, and treatment is symptomatic. The most common presentation is minor recurrent aphthous stomatitis: recurrent, round, clearly defined, small, painful ulcers that heal in 10 to 14 days without scarring. Major recurrent aphthous stomatitis lesions are larger (greater than 5 mm), can last for 6 weeks or longer, and frequently scar. The third variety of recurrent aphthous stomatitis is herpetiform ulcers, which present as multiple small clusters of pinpoint lesions that can coalesce to form large irregular ulcers and last 7 to 10 days. Diagnosis of all varieties is usually made after clinical examination. Many local and systemic factors have been associated with these conditions, and there is evidence that there may be a genetic and immunopathogenic basis for recurrent aphthous ulceration. Management of this condition depends on the clinical presentation and symptoms and includes analgesic, antimicrobial, and immunomodulatory drugs. As dental clinicians and researchers become better trained in oral medicine and stomatology, it is anticipated that the pathophysiology, prevention, and treatment of recurrent aphthous ulceration will improve in the future.

We report a case who was clinically and histopathologically diagnosed as herpetiform pemphigus (HP) and associated with autoimmune hemolytic anemia (AIHA). However, immunofluorescence studies demonstrated concurrent anti-cell-surface and anti-basement-membrane-zone antibodies in the patient's serum. Immunochemical studies showed that the patient's serum reacted with both the pemphigus foliaceus antigen and the two bullous pemphigoid antigens. Subsequently, the patient developed AIHA. Both anemia and skin lesions were successfully treated with oral prednisolone. We believe that this is the first case with HP in association with AIHA. The presence of autoantibodies against multiple antigens suggests an abnormal immunologic tolerance in the antibody production system in this patient.

A patient who developed atypical vesicular Sweet's syndrome (SS) resembling herpes simplex virus infection, during relapse of acute myeloid leukaemia is presented. The morphologic and histologic spectrum of SS is discussed, alerting dermatologists to atypical varients. The association with malignancy, particularly of myeloprliferative origin, is highlighted, as are features of SS that suggest the presence of an underlying malignancy.

Cutaneous sporotrichosis, also known as “Rose Gardener's disease,” caused by dimorphic fungus Sporothrix schenkii, is usually characterized by indolent nodular or nodulo-ulcerative lesions arranged in a linear pattern. We report bizarre nonlinear presentation of Sporotrichosis, in an immunocompetent adult occurring after a visit to Amazon rain forest, speculating infection with more virulent species of Sporothrix. The diagnosis was reached with the help of periodic acid-Schiff positive yeast cells and cigar shaped bodies seen in skin biopsy along with the therapeutic response to potassium iodide.

ejd.2012.1643 Auteur(s) : Yuta Kurashige1 kurasige@tokyo-med.ac.jp, Yoshihiko Mitsuhashi1, Masuyoshi Saito1, Shunpei Fukuda2, Takashi Hashimoto2, Ryoji Tsuboi1 1 Department of Dermatology, Tokyo Medical University, 6-7-1 Nishishinjuku Shinjuku-ku, Tokyo 160-0023, Japan 2 Department of Dermatology, Kurume University, Kurume, Japan Herpetiform pemphigus (HP) has been considered an unusual clinical variant of pemphigus. HP shows vesicles arranged on the periphery of annular erythemas in a manner similar [...]

Lesions that may present in a grouped way include diseases with grouped vesicles such as dyshidrosis, nummular dermatitis, herpes simplex, and herpes zoster; diseases with grouped vesicobullae such as dermatitis herpetiformis, herpes gestationis, subacute lupus erythematosus in the bullous variant, and pemphigus herpetiformis; and diseases with grouped pustules such as pustular psoriasis, dermatitis continua of Hallopeau, and impetigo herpetiformis.

56歳女性。糖尿病。初診の10ヵ月前より全身に激しいそう痒を伴う蕁麻疹様紅斑が出現, 次第に増悪してきた。初診時辺縁に小水疱を伴う環状の紅斑と色素沈着が全身にみられた。好酸球は10,500/mm3であった。組織学的には, 表皮内小水疱とeosinophilic spongiosisがみられ, 真皮上層には好酸球の浸潤が認められた。これらの部位には肥満細胞も混在していた。蛍光抗体直接法で表皮細胞間にIgGの沈着を認め, 間接法は80倍陽性であった。疱疹状天疱瘡と診断し, トラニラスト300mg/日, DDS75mg/日, 金チオリンゴ酸ナトリウム10mg/週投与にて皮疹は徐々に軽快したが, トラニラストの内服中止にて皮疹は急激に増悪した。トラニラストの内服を再開したところ, 皮疹は2週後には軽快し, その後3剤併用にて皮疹の再発をみていない。

We report a patient with herpetiform pemphigus who was negative for autoantibodies to desmoglein (Dsg)1 or 3. He had erythemas with vesicles lining the margins on the trunk and extremities. Histopathology revealed intraepidermal blister with prominent eosinophil infiltration. Direct and indirect immunofluorescence demonstrated the presence of depositing and circulating immunoglobulin (Ig)G autoantibodies, but no IgA antibodies, to keratinocyte cell surface. Nonetheless, neither anti-Dsg1 nor Dsg3 antibodies were detected by enzyme-linked immunosorbent assay. Immunoblotting using human epidermal extracts also showed no reactivity with known intraepidermal or epidermal-dermal junctional substances. Immunoelectronmicroscopy revealed the reactivity on the portion of keratinocyte cell surface but not on the desmosomes. This case suggests that non-desmoglein antigen on keratinocyte cell surface can be targeted in some patients with this unusual variant of pemphigus.

The clinicopathological features of 22 cases of the Dowling-Meara form of epidermolysis bullosa simplex (DM-EBS) (11 males, 11 females; aged 5 days-46 years) were reviewed using data collected over a 10-year period. All cases presented clinically within the first 5 days of life. Early blisters were often large (up to 5 cm in diameter), and were mostly acral and particularly periungual. Some cases presented with more widespread erosive skin changes, and two neonates with extensive skin involvement died as a result of overwhelming sepsis. After the neonatal period a different pattern of blistering occurred with more proximal haemorrhagic, herpetiform clusters of blisters. Central healing with recurrent blistering at the margins of these areas was frequently noted. Other physical signs included varying degrees of intra-oral blistering, nail shedding, nail dystrophy, minor scarring, palmo-plantar keratoderma, a lack of seasonal variation and improvement during later childhood. The underlying pathological mechanism in DM-EBS is basal cell cytolysis, or rarely acantholysis, in association with tonofilament (TF) clumping. TF clumping was found in lesional, perilesional and some non-lesional skin, suggesting that the tonofilament abnormality may be of primary aetiological significance in DM-EBS. TF clumping may be due to specific keratin abnormalities because the altered TF were found in a distribution similar to the known distribution of the basal cell keratins, K5 and K14. The level of blistering was invariably very low within the epidermal basal layer and often less than 0.5 microns above the basement membrane. We conclude that DM-EBS is a distinct, and probably under-recognized genodermatosis which tends to have a good prognosis. However, the disease can occasionally be severe, especially during the neonatal period, when it may be confused with junctional or severe recessive dystrophic EB. Electron microscopy is the best means for demonstrating the characteristics cytoskeletal disorder and confirming the diagnosis.

Recurrent aphthous ulcers in patients with HIV infection can cause significant morbidity, which makes successful diagnosis and treatment imperative. We have found that the diagnostic paradigm for recurrent aphthous ulcers in HIV-seronegative patients, which is based on the uclers' clinical appearance, location, absence of other ulcer-causing pathogens or pathogenic processes, and response to therapy, may be successfully applied to recurrent aphthous ulcers in HIV-infected patients. However, one must be alert for ulcers with uncommon causes as well as ulcers with common causes that have atypical clinical appearances that may mimic recurrent aphthous ulcers. The topical glucocorticoids, which are used to treat recurrent aphthous ulcers in HIV-seronegative patients, proved very effective in HIV-infected patients for treatment of herpetiform and minor ulcers and most major ulcers and were without notable side effects. A few severe cases of major recurrent aphthous ulcers required treatment with systemic prednisone, and some side effects were encountered.

Herpetiform pemphigus was first introduced by Jablonska in 1975, as a variant of pemphigus that combines the clinical features of dermatitis herpetiformis with the immunological features of pemphigus.1 Because of its rare and atypical clinical presentation, patients are often initially diagnosed as Dermatitis herpetiformis, Linear IgA bullous disease, Bullous pemphigoid or Pemphigus foliaceus. We report a case of herpetiform pemphigus in an elderly lady which was initially misdiagnosed as bullous pemphigoid, however histology and immunofluorescence helped in clinching the diagnosis. A 75-year-old woman presented with a one year history of an intensely pruritic eruption which began on her extremities and later expanded to involve the trunk with only partial response to topical corticosteroids and antihistamines. She was otherwise healthy with no accompanying constitutional symptoms and not on any other medications. Cutaneous examination revealed a symmetric polymorphous eruption of old and new lesions, comprising of erythematous urticarial papules and plaques studded with clear and cloudy vesicles (Fig. 1), ulcerated and crusted lesions arranged in herpetiform groups. Oral and genital mucosa were normal. Clinically a differential diagnosis of bullous pemphogoid was entertained, however a Tzanck smear from a clear vesicle showed abundant neutrophils, with only few eosinophils. Biopsy for histopathology showed mild epidermal hyperplasia with a subcorneal blister containing neutrophils and few acantholytic cells. Predominant areas of neutrophilic spongiosis with a superficial perivascular infiltrate of eosinophils and neutrophils were seen in the papillary dermis. Direct and indirect immunofluorescence demonstrated strong intercellular deposits of IgG and C3 in the upper epidermis. Based on the histology and immunofluorescence findings, the diagnosis was revised to herpetiform pemphigus and patient was started on oral steroids. Fig. 1 Grouped flaccid vesicles and bullae in a herpetiform distribution Pemphigus herpetiformis (PH) is recognized as a distinct subset of pemphigus by its pruritus, rarity of mucosal involvement, invariable presence of eosinophils on histology and its tendency to respond to sulfones. Various terms that have been used to describe it are acantholytic herpetiform dermatitis, pemphigus controlled by sulfapyridine, mixed bullous disease and eosinophilic spongiosis in pemphigus. Clinically patients present with severe intractable pruritus and erythematous urticarial papules and plaques with occasional vesicles in a herpetiform arrangement. Even though the clinical picture is reminiscent of dermatitis herpetiformis, the histological findings are variable and include eosinophilic spongiosis, subcorneal pustules with minimal or no apparent acantholyis.2 Though eosinophilic spongoisis is classically seen in pemphigus herpetiformis, neutrophilic spongiosis may also be encountered and should be recognized as an important diagnostic clue3 as was seen in our case. Definitive diagnosis is established by immunopathology which shows in vivo bound IgG deposits on the keratinocyte cell surface primarily in the upper epidermis. The auto-antibodies in most patients of PH target desmoglein 1 and because desmoglein 1 is predominantly located in the upper epidermis, there is preferential binding of IgG to the upper epidermis as was seen in our case. Why the auto-antibodies in PH and pemphigus vulgaris give rise to different clinical presentations could be explained by their preferential binding to different epitopes on the same antigen molecule.4 In our case, the presence of severe pruritus in an elderly female with blisters and absence of mucosal involvement made us think of bullous pemphigoid, however, to our surprise the histology and immunofluorescence turned out to be PH. Patient was started on oral steroids along with dapsone and is currently under follow-up.

Six days ago, the patient had an intense headache; 4 days ago, painful herpetiform lesions appeared on his face. What is the diagnosis?

This study was conducted to characterize the recurrent aphthous ulcers (RAU) found in association with human immunodeficiency virus (HIV) infection, to examine evidence for increased severity of the ulcers with HIV disease, and to determine whether increased severity is associated with abnormalities of peripheral blood lymphocyte subsets. Seventy-five HIV-seropositive patients with RAU were followed for up to 2 years, and lymphocyte subsets were analyzed in 42. Minor, herpetiform, and major ulcer types were seen, but unexpectedly, 66% of the patients had the usually uncommon herpetiform and major types. These types were temporally associated with symptomatic HIV disease. Patients with major RAU were significantly more immunosuppressed than those with minor or herpetiform RAU in that they had fewer CD4 and CD8 lymphocytes (p less than 0.05). The lesion of RAU is considered to represent a local breakdown in immunoregulation. The systemic immune imbalance seen with HIV disease may amplify the local defect and lead to more severe ulcers.

Recurrent Aphthous Stomatitis (RAS) is a common inflammatory condition of the oral mucosa. The aetiology of RAS remains unclear, yet there are several predisposing factors which could be involved in the onset of the lesion. The herpetiform type of RAS appeared to be similar to recurrent oral Herpes Simplex infection and also could be part of Behçet Syndrome. This case report discussed a patient suffering from a herpetiform type of RAS with its clinical appearance resembling recurrent oral Herpes Simplex infection and Behçet syndrome. Initial treatment was undertaken based on the empirical treatment, yet the respond was not satisfactory. Then, laboratory tests were undertaken, including complete blood count, the total population of T lymphocyte, B lymphocyte, T helper, T suppressor, NK cells, T helper/T suppressor ratio, C3, C4, IgG, IgA, and IgM. These tests showed that there were immune and hematinic deficiency condition. Nevertheless, the clinical appearance, laboratory findings and consultation did not support the diagnosis of recurrent oral Herpes Simplex infection and Behçet Syndrome, thus, enhancing the definite diagnosis of the herpetiform type of RAS with immune and hematinic deficiency as the underlying condition. Based on the definite diagnosis, treatment plan was then revised to target the underlying condition.

Herpetiform vesicles and erythema with erosion developed on the trunk of a 48-year-old Japanese man. Acantholysis was observed in the spinous layer of the lesional epidermis and direct immunofluorescence revealed cell surface deposition of immunoglobulin IgG and C3. Indirect immunofluorescence could not detect circulating anti-cell surface antibodies. Immunoblot analysis detected neither anti-desmoglein (Dsg)1 antibody nor anti-Dsg3 antibody. Enzyme-linked immunosorbent assay could detect IgG and IgA anti-Dsg 1 antibodies and IgA anti-Dsg3 antibody, in addition to a gray zone titer of IgG anti-Dsg3 antibody. Intraepidermal neutrophilic infiltration with neutrophilic microabscesses and intense dyskeratotic cells were histopathologically characteristic in this case. Skin lesions improved within 1 month and remission has continued for 9 years under oral administration of dapsone.

The classical lesion of primary syphilis is a solitary, indurated, painless chancre. Atypical presentations, such as herpetiform ulceration, are well recognized. However, there are few references in the medical literature to primary syphilis presenting as balanitis or balanoposthitis. We report two cases of primary syphilitic balanitis in homosexual men.