scholarly journals Exfoliative dermatitis causing diagnostic dilemma: Sibling provides the missing link

2020 ◽  
Vol 2 ◽  
pp. 40-42
Author(s):  
Soumya Jagadeesan ◽  
Malini Eapen ◽  
Aditi Karunakaran ◽  
Vinitha Panicker ◽  
Gopikrishnan Anjaneyan ◽  
...  

A 49-year-old male presented with erythroderma of 3-month duration with a flexural onset. No mucosal lesions were present, and skin biopsy showed features of acantholysis and suprabasal clefting. Direct immunofluorescence test was negative in repeated examinations. The patient was presumptively managed as pemphigus foliaceus with immunomodulators though the level of split was suprabasal rather than subcorneal, and the patient responded well to the treatment. However, his elder sister presented to the department with clinical and histopathological features of classical Hailey–Hailey disease (HHD) which resembled the pathological features found in the index case. This prompted us to review the diagnosis and retrospectively diagnose the index case as HHD presenting with erythroderma.

Author(s):  
Silvia Regina C. Sartori Barravieira ◽  
Neuza Lima Dillon ◽  
Maria Tereza Rezkallah-Iwasso ◽  
Maria Terezinha Serrão Peraçoli ◽  
Paulo Roberto Curi ◽  
...  

A study was conducted on 16 patients with pemphigus foliaceus, ten of them with the localized form (group G1) and six with the disseminated form (group G2). These patients were submitted to full blood counts, quantitation of mononuclear cell subpopulations by monoclonal antibodies, study of blastic lymphocyte transformation, and quantitation of circulating antibodies by the indirect immunofluorescence test, in order to correlate their clinical signs and symptoms and laboratory data with their immunological profile, and to determine the relationship between circulating autoantibody titers and lesion intensity and course of lesions under treatment. Leucocytosis was observed especially in group G2. All patients showed decreased relative CD3+ and CD4+ values and a tendency to decreased relative values of the CD8+ subpopulation. Blastic lymphocyte transformation indices in the presence of phytohemagglutinin were higher in patients (group G1+G2) than in controls. The indirect immunofluorescence test was positive in 100% of G2 patients and in 80% of G1 patients. The median value for the titers was higher in group G2 than in group G1. Analysis of the results as a whole permits us to conclude that cell immunity was preserved and that there was a relationship between antibody titers detected by the direct immunofluorescence test and extent of skin lesions.


1996 ◽  
Vol 33 (3) ◽  
pp. 332-336 ◽  
Author(s):  
T. Iwasaki ◽  
M. Shimizu ◽  
H. Obata ◽  
M. Ogata ◽  
M. Nagata ◽  
...  

The effect of substrate on indirect immunofluorescence (IIF) tests for the detection of circulating autoantibodies was studied by examining sera from 14 canine pemphigus foliaceus patients, six sera with non-pemphigus dermatoses and ten normal dog sera against five different substrates from three species. These substrates included bovine esophagus, bovine nose, bovine tongue, monkey esophagus, and canine nose skin. Nine out of 14 (64.3%) sera from patients with canine pemphigus foliaceus showed intercellular space staining by indirect immunofluorescence using bovine esophagus as substrate. However, sera from nonpemphigus dermatoses and normal dog did not react with bovine esophagus. In other substrates, only bovine tongue showed 1/8 (12.5%) positive reaction at the intercellular space by sera from canine pemphigus foliaceus. Dog nose skin showed the intercellular space staining against ten of ten (100%) normal dog serum. Monkey esophagus showed the fluorescent deposit at the intercellular space in four of nine (44.4%) of pemphigus foliacues dog sera, however, four of ten (40%) of normal dog sera revealed nonspecific intercellular staining. These results indicate that the sensitivity and the specifity of IIF test in canine pemphigus foliaceus depend on the substrate. The best substrate for detecting circulating autoantibody in canine pemphigus foliaceus patients among five different substrates was bovine esophagus because of its sensitivy and high specifity. The diagnosis of canine pemphigus foliaceus should be made on the basis of a combination of clinical signs, histopathology, direct immunofluorescence, and the detection of circulating autoantibody.


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska

Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.


2017 ◽  
Vol 1 (1) ◽  
pp. 19
Author(s):  
ManjunathShenoy Mala ◽  
Vaishnavi Gopal ◽  
Malcolm Pinto

2020 ◽  
Vol 10 (7) ◽  
pp. 1570-1574
Author(s):  
Xiangyu Liu ◽  
Xiaogang Ren ◽  
Xiaoying Ma ◽  
Xi Sun ◽  
Yue Wu ◽  
...  

To explore the clinical features and endosonographic (EUS) features of gastric submucosal tumors, the connection of submucosal tumor features and pathological features are analyzed to increase the awareness of submucosal tumors. This study is based on 237 cases of gastric mucosal lesions from March 2015 to March 2019. Endoscopic ultrasonography was performed to record the size and origin of the lesions and to make the preliminary qualitative diagnosis of the lesions, where endoscopic resections have 186 cases. According to the actual lesion resection in endoscopic ultrasonography, the origin level of SMTs and the coincidence rate of endoscopic ultrasonography diagnoses are judged. Therefore, endoscopic ultrasonography has high localization, characterization, and differential diagnosis value for gastric mucosal lesions. Diagnosis of gastric mucosal lesions under endoscopic ultrasonography is helpful for the choice of endoscopic resection surgical method.


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