Acantholytic and Pagetoid Variant of Bowen's Disease with Microinvasion on the Scalp of a Young Female Patient: A Case Report.

Bowen's disease (BD) is a non-melanoma skin cancer with several histological subtypes. Herein we describe a case of a 35-year-old woman with a 4-cm diameter crusted plaque on the parietal scalp region. She had the lesion for 2 years. It had previously been histologically diagnosed as pemphigus vulgaris and only treated with a topical cream. The lesion progressively became thicker and larger. A new biopsy showed atypical cell proliferation through the whole thickness of the epidermis and follicular epithelium, with dermal microinvasion along with acantholysis and clear cell formation. The patient underwent total lesion excision (1 cm margin) with the diagnosis of both acantholytic and pagetoid subtypes of BD and dermal microinvasion. We describe a rare case of a young female patient with both subtypes of BD present in one lesion on an area not exposed to the sunlight. The lesion was initially misdiagnosed as pemphigus vulgaris.

C4d immunohistochemical stain of formalin-fixed paraffin-embedded tissue as a sensitive method in the diagnosis of bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is the most common type of subepidermal bullous disease. Direct immunofluorescence (DIF) is the gold standard for diagnosis, but it requires a fresh tissue specimen. This study was conducted to evaluate the accuracy of using immunohistochemical (IHC) analysis for the detection of C4d in paraffin-embedded tissue of BP cases. METHODS: A retrospective study was performed on 30 BP cases (confirmed by Hematoxylin and eosin [H&E] and DIF) and 30 controls. IHC studies were performed on formalin-fixed paraffin-embedded (FFPE) tissues. RESULTS: Of 30 BP patients (11 male and 19 female), 27 (90%) were C4d positive and three patients (10%) were C4d negative. The C4d deposits were seen as linear brown stain along the dermoepidermal junction (18 cases), around basal keratinocytes (eight cases), or Civatte-like (three cases). Two cases revealed more than one pattern in IHC staining. Of 30 control specimens, 26 (86%) cases were C4d negative and four (13.4%) were positive for granular deposits of C4d which were diagnosed as dermatitis herpetiformis. CONCLUSION: C4d IHC study is a valuable diagnostic tool with sensitivity of 90% and specificity of 86.7%, and is especially useful in cases in which frozen specimens are not available or FFPE specimens are the only available material for analysis.

Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission.

BACKGROUND: Pemphigus vulgaris (PV) is characterized by multiple relapses, occurring especially in patients on minimal therapy or off therapy. OBJECTIVE: To identify immunologic predictors (anti-desmoglein [Dsg] 1 and 3 antibodies; direct immunofluorescence [DIF]) for relapse in PV patients. METHODS: Eighty-nine patients in complete clinical remission for at least 6 months and receiving less than or equal to 10 mg prednisolone daily and no immunosuppressive drugs were evaluated using DIF (n=89) and Dsg ELISA (n=46). They were followed until relapse or for at least 18 months. RESULTS: DIF was positive in 44 of 89 patients (49.5%); anti-Dsg 3 antibodies were detected in 18 of 46 patients (39.1%) and anti-Dsg 1 antibodies were detected in 4 of 46 patients (8.7%). Relapse occurred in 38 patients (42.7%). Mean relapse-free time was significantly shorter in anti-Dsg 3-positive patients compared to anti-Dsg 3- negative patients (P = .015) and in DIF-positive patients compared to DIF-negative patients (P = .047), but not in anti-Dsg 1- positive patients compared to anti-Dsg 1-negative patients (P = .501). Sensitivity and predictive values of neither of these tests were high. LIMITATIONS: Small number of anti-Dsg 1-positive patients and use of conventional ELISA. CONCLUSION: Positive anti-Dsg 3 ELISA and, to a lesser degree, positive DIF are predictors of relapse in PV patients in clinical remission. Decision on discontinuing treatment should be based on the results of these tests as well as on clinical findings.

Longitudinal melanonychia in an Iranian population: a study of 96 patients.

BACKGROUND: Longitudinal melanonychia (LM) can be a challenging sign since it may be caused by a wide variety of benign and malignant conditions. Cutaneous melanoma is the most important cause of LM. Objective: We performed this study to examine different aspects of LM in Iran, where cutaneous melanoma is rare. METHODS: In this cross-sectional study, we reviewed medical records and pathology reports of a total of 96 patients presenting with LM. These patients had been visited and undergone nail biopsy in Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Demographic, clinical, and pathological data were recorded. RESULTS: The most common diagnosis was junctional nevi in 28 patients (29.2%) followed by melanoma in 19 patients (19.8%). Patients had a mean age of 42.4 years (± 19.4). The mean ages in the groups with junctional nevi and melanoma were 33.3 (± 19.5) and 51.9 (± 17.8), respectively; their difference was statistically significant (P value = 0.001). Hutchinson's sign was present in 10 patients, 9 of which had melanoma. Also, melanoma was only observed in patients presenting with a solitary nail lesion. Nails mostly affected by melanoma were middle fingers of the hands (7 patients) and thumbs (6 patients). Out of 18 patients with nail dystrophy, 13 (72.2%) were diagnosed with melanoma. LIMITATIONS: Only patients who have undergone biopsy were studied. CONCLUSION: Melanoma is an important cause of LM in Iranian patients and should especially be suspected in older patients who present with a solitary nail lesion on their middle finger or thumb. Other findings that direct us toward melanoma are presence of Hutchinson's sign and nail dystrophy.

Modulation of hepatitis C virus core DNA vaccine immune responses by co-immunization with CC-chemokine ligand 20 (CCL20) gene as immunoadjuvant.

Plasmid DNA vaccination is a promising vaccine platform for prevention and treatment of infectious disease. Enhancement of the DNA vaccine potency by co-inoculation of immunoadjuvant has been shown to be an effective strategy. Modulation of dendritic cells and T-cells locomotion and trafficking to prime an immune response is mediated by distinct chemokines. The recent study was designed to elucidate the adjuvant activity of plasmid expressing CC-chemokine ligand 20 (pCCL20) in co-inoculation with hepatitis C virus (HCV) core DNA vaccine immunization. pCCL20 was constructed and evaluated for its functional expression. Sub-cutaneous inoculation of pCCL20 with HCV core DNA vaccine was performed via electroporation in BALB/c mice on day 0 and 14 and a HCV core protein booster was applied on day 28. On week after final immunization, both humoral and cell-mediated immune responses were assessed by indirect ELISA for core specific antibodies, lymphocyte proliferation, cytokine ELISA/ELISpot and cytotoxic Grenzyme B (GrzB) release assays. Mice were co-immunized with pCCL20 developed higher levels of core specific IFN-γ/IL-4 ratio and IL-2 release, IFN-γ producing cells, lymphocyte proliferation and cytotoxic Grenzyme B release in both draining lymph nodes and spleen cells of immunized mice. The core-specific serum total IgG and IgG2a/IgG1 ratio were significantly higher when the pCCL20 was co-inoculated. These results suggest the potential of CCL20 chemokine as vaccine adjuvant to enhance Th1 mediated cellular and humoral immune responses in HCV core DNA immunization.

Porokeratotic eccrine ostial and dermal duct nevus.

PEODDN is a rare benign cutaneous disorder that clinically resembles comedo nevus but favors the palms and soles, where pilosebaceous follicles are absent. Widespread involvement along Blaschko's lines can also occur. It is a disorder of keratinization involving the intraepidermal eccrine duct (acrosyringium) and is characterized by eccrine hamartoma and cornoid lamellation in pathology. The patient is a 29-year-old man with an 8-year history of pruritic skin lesions on his right lateral ankle. In the pathologic examination, multiple small epidermal invagination with overlying parakeratotic cornoid lamellation, loss of granular layer, and few dyskeratotic cells at the base of epidermal invagination are revealed. After clinic-pathologic correlation, the diagnosis of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) was made. Late-onset and rare clinical presentation as pruritic lesion are the characteristic features that make this patient an extraordinary presentation of PEODDN.

Pachyonychia congenita-associated alopecia.

A 5-year-old female, known case of pachyonychia congenita, presented with diffuse hair loss; remaining hairs were easily plucked kinky hairs. Hair samples from patient were investigated using a light microscope. The hairs of the patients were mainly anagen hairs and unlike normal plucked anagen hairs, showed keratinization and cornification of their hair bulbs. No specific hair shaft abnormality was found.

The expression of CXCR3 and CD30 in mycosis fungoides.

BACKGROUND: The clinical progression of mycosis fungoides (MF) often correlates with microscopic large cell transformation (LCT). It is reported that CD30 expression in the LCT of MF is associated with an absence of CXCR3 expression. This study investigates a large number of patients diagnosed with MF to determine the correlation between expression of CXCR3 and CD30 in additional sections. METHODS: The study included archival skin specimens from 101 patients with MF. We analyzed these specimens by immunohistochemistry for expressions of CXCR3 and CD30. RESULTS: The biopsy specimens showed microscopic features of low grade MF (LG-MF) in 80 cases and transformed MF (T-MF) in 21 cases. Tumor lymphocytes expressed CXCR3 in 61 out of 80 cases (76.3%) of LG-MF and in 10 out of 21 cases (47.6%) of T-MF.  CD30 positivity (CD30+) was seen in 16 of 80 cases (20%) of LG-MF and 12 of 21 cases (57.1%) of T-MF. The tumor cells in 8 of the 12 CD30+ T-MF cases showed scattered expression of CXCR3. CONCLUSION: CXCR3 expression was associated with epidermotropic T cell tumors but was greatly absent in dermal ones. Scattered or diffuse CD30 expression in T-MF was not associated with an absence of CXCR3 expression.

Description of some dermatoscopic features of acral pigmented lesions in Iranian patients: a preliminary study.

Proper differentiation between acral malignant melanoma and benign pigmented lesions like melanocytic nevi is of great value. To avoid unnecessary biopsies, dermatoscopy has been introduced as a non-invasive modality and has improved the clinical diagnostic accuracy in recent decades. We aimed to describe dermoscopic patterns of acral pigmented lesions of patients in the clinic of dermatology in Razi Hospital, Tehran, Iran. This study was conducted as a descriptional study among a total of 62 pigmented lesions located on volar skin of palms and soles. After initial clinical evaluation, lesions were examined entirely by dermoscopy. All the patterns within a lesion were described, and lesions suspicious of malignancy (clinically or dermatoscopically) were selected for histopathological evaluation. Of our 62 lesions, three lesions were not melanocytic. According to our final clinicopathological diagnosis, 47 lesions were benign melanocytic nevi and 12 lesions were malignant melanoma. Parallel furrow pattern was the most frequent among our benign lesions (51.1%) followed by lattice-like pattern (23.4%) and acral reticular pattern (21.3%). Diffuse multi-component pattern, parallel ridge pattern and abrupt edge were respectively most common patterns among malignant melanomas. Acral benign melanocytic nevi and malignant melanomas respectively have well distinctive characteristics in dermatoscopy among our patients.

Lethal systemic Degos disease with prominent cardio-pulmonary involvement.

Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs.