Defining urticarial dermatitis: a subset of dermal hypersensitivity reaction pattern

BACKGROUND: Urticarial dermatitis may represent a useful term for a subset of a reaction pattern designated most commonly as dermal hypersensitivity by pathologists. The term is not commonly used, and requires definition to determine whether it is clinically relevant. OBJECTIVES: To define urticarial dermatitis and distinguish it from other urticarial reaction patterns and to review the frequency with which dermatologists can recognize clinical settings that match the biopsy findings of urticarial dermatitis. DESIGN: Retrospective analysis of clinical and/or histological diagnosis of urticarial dermatitis, applying strict histological criteria in a center using urticarial dermatitis as a diagnostic term in 190 archived reports. SETTING: Tertiary referral dermatopathology service reporting for dermatological practices in Sydney, Australia. MAIN OUTCOME MEASURES: The correlation between clinical and histological diagnoses of urticarial dermatitis and alternate diagnoses was analyzed. The frequency of positive immunofluorescence findings for bullous pemphigoid was determined in a subset of patients with urticarial dermatitis in whom this test was ordered to exclude prodromal bullous pemphigoid. RESULTS: Urticarial dermatitis was the histological diagnosis in at least 1 biopsy result in 148 patients, and matched the provisional clinical diagnosis in 49 (33.1%) patients. Urticarial dermatitis was the only diagnosis provided in 21 patients. The main alternate clinical diagnoses provided were early bullous pemphigoid or dermatitis herpetiformis (47 patients [31.8%]), dermatitis (39 patients [26.4%]), drug reaction (35 patients [23.6%]), urticarial vasculitis (24 patients [16.2%]), and urticaria (12 patients [8.1%]). In 91 patients with a clinical diagnosis of urticarial dermatitis, the histological diagnosis in at least 1 biopsy result was matched in 49 patients (53.8%); other histological diagnoses included dermatitis (21 patients [23.1%]), papular urticaria (12 patients [13.2%]), drug reaction (6 patients [6.6%]), and urticaria (3 patients [3.3%]). Review of 38 direct immunofluorescent results for prodromal bullous pemphigoid and a biopsy finding of urticarial dermatitis revealed only 3 positive results (7.9%). CONCLUSIONS: Urticarial dermatitis seems to be a useful histological and clinical term for a subset of the dermal hypersensitivity reaction pattern. Although the clinical presentation is not restricted to a specific entity, eczema and drug reactions seem to be the most frequent clinical associations; and in a subset of patients, urticarial dermatitis remains as a recognizable reaction pattern. Urticarial dermatitis without eosinophilic spongiosis is not a reliable indicator for bullous pemphigoid, because the findings of immunofluorescence are often negative.     

Rosacea: a clinicopathological approach

BACKGROUND: There are few reports of the histological changes in rosacea, and little attempt has been made to correlate such changes with clinical findings. In the present study, we describe in detail the histopathological features of rosacea in a large number of patients and simultaneously investigate the aetiopathogenesis of the disease based on the comparative assessment of epidemiological, clinical and histological findings. METHODS: The study included 73 patients with rosacea. A skin biopsy with a 4-mm punch was performed in each case. All biopsy specimens included subcutaneous tissue. In 10 randomly selected patients, facial biopsy specimens were obtained from both involved and uninvolved (non-lesional) skin. Demodex mite presence was estimated semi-quantitatively under light microscopy. Patients with self-reported gastro-intestinal symptoms were submitted to upper gastro-intestinal endoscopy, and a rapid urease test was performed. Serological antibodies, IgG and IgA, against Helicobacter pylori were also detected. RESULTS: The patients had a broad clinical spectrum of lesions. No specific histological features associated with either erythematous-telangiectatic or papulopustular clinical forms were noticed. Histological examination showed that there is no histological pattern unique to rosacea. Three different types of granulomas were observed: small palisaded ones around altered collagen and other granulomas of elastolytic and non-specific epithelioid type, all coexisting in 5 cases. The deep dermis and subcutis were frequently involved. Comparative study in 10 rosacea patients between lesional and non-lesional skin biopsies revealed almost the same histological changes to the latter biopsies, to a lesser degree though. CONCLUSION: Rosacea seems to be a reaction pattern to which a variety of pathogenetic routes may lead.     

Pemphigus foliaceus and oral lichen planus in a patient with systemic lupus erythematosus and thymoma

A 49-year-old woman with systemic lupus erythematosus, and a history of thymoma and chronic white plaques of the oral mucosa, developed a blistering eruption which was diagnosed as pemphigus foliaceus based on clinical, histological and direct immunofluorescence findings. However, the white plaques had both the clinical and histological features of lichen planus, as well as intercellular antibodies suggestive of pemphigus. Such conditions in combination have been documented previously, but this is believed to be the first report of the coexistence of all four in one individual. The common mechanism underlying these four conditions may well be an immunological disturbance.     

Atypical pemphigus: discordance between clinicopathological findings and the antigenic profile in four cases

BACKGROUND: The diagnosis of pemphigus vulgaris and pemphigus foliaceus is usually based on clinical, histological, and immunofluorescence (IF) findings. In recent years, the antigenic profile of both diseases has been further defined by immunobiochemical techniques (ELISA, immunoblot, and immunoprecipitation). METHODS: A retrospective study of 40 pemphigus patients was performed to determine the clinical, histological, and antigenic profile in patients with pemphigus followed at our Department. Charts review, clinical data, histological and IF findings, and antigenic analysis by ELISA were performed in all patients. RESULTS: In most patients, there was a perfect correlation between the clinical and histological findings and their antigenic profile. In four patients (10%), clinicopathological features and antigenic findings were discordant. CONCLUSIONS: The antigenic profiles in pemphigus do not always correlate with the clinical diagnosis. Therefore, clinical and histological features should be considered as the mainstay for the diagnosis of pemphigus.     

Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease

Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox-Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease.     

Poikiloderma of Civatte: a histopathological and ultrastructural study

BACKGROUND: Poikiloderma of the face and neck (Civatte) has not been studied in depth for decades, especially as far as the histopathology is concerned. MATERIAL AND METHODS: We studied 50 consecutive patients with poikiloderma of Civatte (PC). Their evaluation included: history, physical examination, lesional skin biopsy and histological examination of sections stained with hematoxylin-eosin, PAS, Fontana-Masson, acid orcein Giemsa for elastic fibers and toluidine blue for mast cells. In 10 randomly selected subjects, a second skin biopsy was performed and specimens were examined under the electron microscope. RESULTS: There were 34 females (68%) and 16 males. The mean age at diagnosis was 47.8 years for females and 61.7 years for males. Histological examination revealed an atrophic (62%), flattened (84%) epidermis with hyperkeratosis (92%) and occasional follicular plugging (34%). In some cases, mild hydropic degeneration of the basal cell layer was evident (46%). Melanin was irregularly distributed in the lower epidermis (94%), and melanophages were often present in the dermis (92%). The most prominent and constant feature (100%) was solar elastosis of the papillary dermis. The blood vessels were almost invariably dilated (96%) with a mild perivascular lymphohistiocytic infiltrate (78%), sometimes with plasmacytes (56%). At the ultrastructural level, the epidermis showed only minor changes. The dermoepidermal junction was intact. The most constant findings were swelling and disruption of the collagen fibers as well as focal degeneration of the collagen bundles. Occasionally, several vacuolar spaces were found just under the basal lamina. Melanin-laden macrophages scattered in the dermis were also detected. CONCLUSIONS: PC shows distinct histological and ultrastructural features, supporting the theory that it represents a separate entity. The histology of PC is characteristic but not pathognomonic. On this basis, the differential diagnosis from Riehl's melanosis, poikiloderma atrophicans et vasculare and other acquired poikilodermas can be made. Ultrastructural findings were consistent with the histological findings. Changes of the dermal connective tissue (solar elastosis) predominated, providing morphological evidence for the role of ultraviolet radiation in the pathogenesis of PC.     

Secondary syphilis: a clinico-pathological review

The histological appearances found in biopsies from fifty-seven patients with secondary syphilis have been correlated with the clinical morphology of the eruptions. Considerable variation of histological pattern was encountered, and the frequency with which some of the classically described changes were found to be absent or inconspicuous is stressed. Of particular interest were the findings that, in nearly one-quarter of the biopsies, plasma cell infiltration was either absent or very sparse, and that vascular damage was seen in less than half. Where present, the vessel changes were almost entirely confined to swelling of the endothelial cells. Proliferation of the endothelial cells was most uncommon. The epidermis was very frequently involved in the inflammatory process. Exocytosis, spongiosis, parakeratosis, and acanthosis were the most frequent changes. No consistent histological difference between papular and papulo-squamous lesions could be found but macular lesions demonstrated more superficial and less intense dermal infiltration as well as less severe epidermal involvement. In late secondary lesions, the infiltrate became granulomatous, but in other respects the duration of the exanthem could not be correlated with the pathology. The differential diagnosis from pityriasis lichenoides and other inflammatory dermatoses is discussed and the value of histopathology in the diagnosis of secondary syphilis is emphasized.     

嗜酸细胞性脂膜炎1例

报告1例嗜酸细胞性脂膜炎,患者男,33岁,因双下肢皮损伴肿胀2个月,症状加重伴发热10余天就诊,皮损组织病理检查示皮下脂肪层有弥漫性嗜酸粒细胞为主的浸润,根据临床表现及组织病理所见,诊断为嗜酸细胞性脂膜炎,经抗生素和糖皮质激素治疗后皮损痊愈。     

Pemphigus vegetans presenting as acrodermatitis continua suppurativa

The authors present a 51-year-old patient with clinical signs of acrodermatitis continua suppurativa of the toes. Since histological findings failed to unambiguously support the diagnosis of acrodermatitis continua suppurativa, immunohistology was performed. The results showed intercellular epidermal fluorescence. The subsequent clinical picture (vegetating formation), the repeated histological examination (suprabasal cleft and eosinophilia), as well as the data of immunohistological findings supported the diagnosis of pemphigus vegetans Hallopeau. The patient was treated successfully with a combination of cyclosporine-A and low-dose steroid therapy. The case is of special interest, because there is only a single publication about phalangeal and nail involvement in Hallopeau's type pemphigus vegetans in the dermatological literature according to our knowledge.     

Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature

Acrodermatitis enteropathica (AE) is a rare disorder associated with poor absorption of zinc. A variety of clinical and histological findings have been reported in the literature, described mainly in isolated case reports. Because of the varied nature of these cases, the histological features of AE are described often as non-specific. We describe lesions of AE in two patients who presented with vesiculobullous and erosive skin lesions, both showing intra-epidermal, inflammatory vesiculation with surrounding eosinophilic epidermis and necrotic keratinocytes. The lack of clinical suspicion of AE led to their misdiagnosis. We present these two patients to further characterize the bullous variant of AE, and we review the previously reported clinical and histopathological findings.     

Naproxen-induced pseudolymphoma syndrome: a case report

Drug-induced pseudolymphoma syndrome is a rare form of adverse cutaneous drug reaction. Its clinical and histological presentation may mimic mycosis fungoides, which sometimes leads to misdiagnosis and unnecessary treatments. We describe the case of a 65-year-old man with a generalized pruritic and confluent maculopapular eruption. His history of skin lesions was concordant with medication and resolved after discontinuation. However, microscopic examination showed characteristic features of mycosis fungoides, including Pautrier-like microabscesses and cerebriform nuclei of atypical lymphocytes. The immunohistochemical study showed CD4/CD8 infiltrate ratio to be 1 in the epidermis. The histopathological result of a second skin biopsy was compatible with drug eruption. This experience demonstrates that combining clinical history, histological and immunohistochemical findings is crucial for a definite diagnosis of PLS.     

副肿瘤性天疱疮1例

报告1例副肿瘤性天疱疮。患者男,50岁。反复口腔黏膜糜烂,全身有皮损、瘙痒,伴淋巴结增大1个月余。依据病史、组织病理和免疫病理改变,确诊为副肿瘤性天疱疮伴慢性淋巴细胞白血病。     

Wolf''s isotopic response: a case of zosteriform lichen planus on the site of healed herpes zoster

Based on histological findings, the patient had lichen planus. The clinical picture suggests Wolf's isotopic response on the site of healed herpes zoster. These conditions taken into account lead to the diagnosis of zosteriform lichen planus. Lichenoid papules in a dermatomal arrangement are extremely rare. The clinical, dermoscopic and histological features of this case are described.     

Comparison of basal cell carcinoma subtypes observed in preoperative biopsy and Mohs micrographic surgery

BackgroundThe treatment of basal cell carcinoma depends on its histological subtype. Therefore, a biopsy should be performed before definitive treatment. However, as the biopsy is only a sample of the tumor, it does not always shows every histological subtype present in the neoplasm. Few studies have compared the histological findings of biopsies with the findings of Mohs micrographic surgery. By evaluating the totality of the peripheral margins, in addition to sampling large tumor areas, this technique provides a more representative amount of tissue than preoperative biopsy.Objectivesa) Determine the agreement between the histological subtype of basal cell carcinoma from punch biopsy and the findings of Mohs surgery; b) To assess, among the discordant cases, the prevalence of non-aggressive tumors in the preoperative biopsy that were reclassified as aggressive by Mohs surgery.MethodsRetrospective analysis of 79 cases of basal cell carcinomas submitted to punch biopsy and subsequent Mohs surgery.ResultsThe agreement between the classification of the subtypes in the biopsy and in Mohs surgery was 40.5%. Punch biopsy was able to predict the most aggressive basal cell carcinoma growth pattern in 83% of cases.Study limitationsRetrospective nature, sample size, and biopsies performed by different professionals.ConclusionsThe agreement between the histopathological subtypes of basal cell carcinoma as seen in preoperative biopsy and Mohs surgery was low. However, preoperative biopsy presented good accuracy (83%) in detecting aggressive histopathological subtypes.     

The value of anal cytology and human papillomavirus typing in the detection of anal intraepithelial neoplasia: a review of cases from an anoscopy clinic

BACKGROUND: Previous studies have reached differing conclusions about the utility of anal cytology as a screening tool for anal intraepithelial neoplasia (AIN). There is a need also to establish whether HPV typing offers a useful adjunct to screening. METHODS: We analysed data from 99 consecutive homosexual/bisexual male patients (89 HIV-1 positive) who underwent high resolution anoscopy. Follow up visits for these patients were also included, giving a total of 160 anoscopic procedures. Comparison was made between results of anal cytology using the sampling method of Palefsky, and histological findings of biopsies taken from abnormal areas seen on high resolution anoscopic examination of the anal canal. Swabs taken concurrently with the cytology were analysed for the presence of human papillomavirus (HPV) DNA and compared with the cytological and histological findings. RESULTS: The sensitivity of the cytology was 83%, and the specificity 38% when compared with histology. At screening of 34 asymptomatic men, 83% had anal cytological dysplasia and 78% had AIN. There were no significant differences in the prevalence of hrHPV genotypes between different cytological or histological grades of abnormalities. CONCLUSION: Anal cytology by the Palefsky method is simple to undertake, has a sensitivity and specificity comparable with cervical cytology, and can therefore be used as the basis of a pilot screening project in centres with large cohorts of HIV positive homosexual men who have a high risk of developing anal carcinoma. HPV genotyping is not a useful adjunct to cytological screening.     

A poroid neoplasia arising close to a seborrheic keratosis and a trichoepithelioma.

A 69-year-old woman had a well-defined, slightly raised, brownish, keratotic plaque with an eccentric group of roughly circular, bluish, dome nodules on her right scapular area. Histological study revealed the presence of an eccrine poroma demonstrating features of eccrine poroma, hidroacanthoma simplex, and dermal duct tumor and arising in a lesion of seborrheic keratosis and a trichoepithelioma. In the eccrine poroma region, an area with malignant changes was evident. The findings in this case support the view of eccrine poroma as a spectrum of histological variants and the possibility of malignant transformation from a eccrine poroma towards porocarcinoma. The association with seborrheic keratosis is most probably coincidental, and the trichoepithelioma was probably due to induction of the tumoral stroma on adjacent follicular epithelia.     

Melanoma epidemic: a midsummer night’s dream?

Background  The reported incidence of melanoma has greatly increased and this has been attributed to ultraviolet exposure.
Objectives  We considered the possibility that the increase was an artefact caused by diagnostic drift.
Methods  We tested this by analysing the histological diagnosis, mortality and incidence of all lesions reported as melanomas in East Anglia between 1991 and 2004.
Results  There were 3971 melanomas in all, and their annual incidence increased from 9·39 to 13·91 cases per 100 000 per year during the period studied. This increased incidence was almost entirely due to minimal, stage 1 disease. There was no change in the combined incidence of the other stages of the disease, and the overall mortality only increased from 2·16 to 2·54 cases per 100 000 per year.
Conclusions  We therefore conclude that the large increase in reported incidence is likely to be due to diagnostic drift which classifies benign lesions as stage 1 melanoma. This conclusion could be confirmed by direct histological comparison of contemporary and past histological samples. The distribution of the lesions reported did not correspond to the sites of lesions caused by solar exposure. These findings should lead to a reconsideration of the treatment of 'early' lesions, a search for better diagnostic methods to distinguish them from truly malignant melanomas, re-evaluation of the role of ultraviolet radiation and recommendations for protection from it, as well as the need for a new direction in the search for the cause of melanoma.     

Extramammary Paget's disease with bowenoid histologic features accompanied by an ectopic lesion on the upper abdomen

We present a 79-year-old man who suffered from extramammary Paget's disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowen's disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Paget's cells adjacent to areas characteristic of Bowen's disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Paget's disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Paget's disease with bowenoid histologic features.     

Prurigo pigmentosa: a possible mechanism of action of sulfonamides

The patient described here had all the clinical features (itchy red papules and reticular pigmentation) as well as the histological findings (lichenoid tissue reactions) of prurigo pigmentosa, a little-known disorder of unknown etiology, and responded well to sulfamethoxazole treatment. In order to investigate the mode of action of this drug and thus the underlying pathogenesis of this dermatosis, the effect of sulfamethoxazole on the production of oxygen intermediates (OIs) was examined both in the cell-mediated system and in the xanthine-xanthine oxidase system. It was found that therapeutic doses of the drug significantly reduced the hydroxyl radical levels, one of the most potent oxidants that might cause tissue injury. These findings suggest the possibility that OIs produced by infiltrated cells are involved in the inflammatory process of prurigo pigmentosa and that sulfonamides exert their anti-inflammatory effects by affecting OI generation which results in protection against lichenoid tissue reaction.