A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor

Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlying neoplasms, both malignant and benign. The most constant clinical presentation of PNP is the presence of intractable stomatitis. Herein we present a 25-year-old male with a 3-month history of refractory stomatitis especially involving the lips and widespread vesiculobullous eruption on his trunk and extremities. The diagnosis of PNP was confirmed based on histological and serological results. Investigation for the underlying neoplasm revealed a retroperitoneal tumorous mass which was biopsied and diagnosed as the inflammatory myofibroblastic tumor (IMT). The tumor was surgically excised, and different treatment regimens were used to treat the mucocutaneous lesions. Skin lesions responded favorably to treatment, but oral stomatitis still persists which is the case in most PNP patients. This combination of PNP and IMT has rarely been reported in the literature. Treatment started with corticosteroid and rituximab then tumor excised

Clinicoepidemiological Features of 82 Cases of Bullous Pemphigoid in Tehran, Iran

Bullous Pemphigoid is a chronic immunobullous disease, characterized by subepidermal bulla on the skin and mucosa. The purpose of the present study was to investigate the clinicoepidemiological features of bullous pemphigoid in Iranian patients. In this retrospective descriptive study, we reviewed 82 bullous pemphigoid patients within 2014-2016. The mean age of the patients was 67.13 years (range between 25 and 97) including 32(39.1%) males and 50(60.9%) females. Mucosal involvement was positive in 33(40.2%) of the patients, and oral mucosa was most commonly involved. Head and neck area were involved in 43 (52.4%) of the patients. Clinical and epidemiological characteristics of bullous pemphigoid are different in different regions of the world

Psoriasiform mycosis fungoides: a rare form of the disease with review of the literature

Background: Mycosis fungoides (MF) typically presents as erythematous scaly patches or plaques that may progress to cutaneous tumors. Although MF may be presented like other dermatoses, initial presentation as psoriasiform plaques simulating psoriasis is rare. Differentiating MF from psoriasis is important because systemic therapies used for psoriasis can worsen MF. We describe a case of psoriasiform MF and we also review the clinicopathological features of similar cases in the literature.Case: A 46-year-old woman was referred to our clinic with a history of psoriasiform plaques for 13 years. She had multiple, generalized, indurated plaques with thick psoriasiform scales that were unresponsive to topical treatments. The histopathology showed marked psoriasiform epidermal hyperplasia with epidermotropic atypical lymphocytes compatible with MF. Immunohistochemical (IHC) staining showed that atypical lymphocytes were positive for CD3, CD4, CD8, and CD5. Of note, upper dermal and intraepidermal large atypical lymphocytes were CD30 positive. The review of similar psoriasiform MF cases revealed that they had all been treated as psoriasis for many years and finally diagnosed as MF especially after deterioration induced by immunosuppressive therapies.Conclusions: In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent

Psoriasiform mycosis fungoides: a rare form of the disease with review of the literature

Background: Mycosis fungoides (MF) typically presents as erythematous scaly patches or plaques that may progress to cutaneous tumors. Although MF may be presented like other dermatoses, initial presentation as psoriasiform plaques simulating psoriasis is rare. Differentiating MF from psoriasis is important because systemic therapies used for psoriasis can worsen MF. We describe a case of psoriasiform MF and we also review the clinicopathological features of similar cases in the literature.Case: A 46-year-old woman was referred to our clinic with a history of psoriasiform plaques for 13 years. She had multiple, generalized, indurated plaques with thick psoriasiform scales that were unresponsive to topical treatments. The histopathology showed marked psoriasiform epidermal hyperplasia with epidermotropic atypical lymphocytes compatible with MF. Immunohistochemical (IHC) staining showed that atypical lymphocytes were positive for CD3, CD4, CD8, and CD5. Of note, upper dermal and intraepidermal large atypical lymphocytes were CD30 positive. The review of similar psoriasiform MF cases revealed that they had all been treated as psoriasis for many years and finally diagnosed as MF especially after deterioration induced by immunosuppressive therapies.Conclusions: In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent

Segmental pigmentation disorder: A rare form of cutaneous dyspigmentation

Background: Segmental pigmentation disorder (SegPD) is a rare type of cutaneous dyspigmentation. This hereditary disorder, first described some 20 years ago, is characterized by hypo and hyperpigmented patches on the trunk, extremities and less likely on the face and neck. These lesions are considered as a type of checkerboard pattern. Case Presentation: Herein, we present a 26-year-old male who presented with hyperpigmented patches on his trunk, neck and upper extremities. Considering the clinical and histopathological findings, the diagnosis of SegPD was confirmed. Conclusion: SegPD is a somewhat neglected entity which should be considered in differential diagnosis of pigmentation disorders

Assessment of the therapeutic aspect of systemic non-biologic anti-psoriatic treatment modalities used in combination with methotrexate

Introduction: Psoriasis is a common skin disease affecting 1% to 3% of the population worldwide. Combination therapy with traditional systemic anti-psoriatic agents offers a promising method for managing severe or recalcitrant psoriasis. Aims and Objectives: Lack of documented data regarding the efficacy and safety of combination therapy with methotrexate and other anti-psoriatic drugs in Iranian patients prompted us to design a study to investigate this issue in an Iranian educational hospital. Materials and Methods: Records of psoriatic patients in Razi Hospital were reviewed retrospectively from October 2008 to March 2012. Forty-six patients who had received methotrexate in combination with other systemic anti-psoriatic agents were included in this study. Response to treatment was evaluated by PASI. Adverse reactions and the type of combination were recorded for all patients. Results: Methotrexate was most frequently administered in combination with acitretin (n = 21) followed by cyclosporine (n = 11), and NB-UVB and PUVA (n = 6). Combinations were effective in the majority of patients; 46% showed good response (21 patients), 26% showed moderate response (12 patients) and 28% (13 patients) showed poor response who therefore switched to another treatment. The treatments were generally tolerable. Thirty-two patients experienced adverse events although none was severe or required hospitalization. The relative frequencies of adverse reactions were different among groups of patients receiving various therapeutic regimens. Conclusions: Methotrexate-combination therapy can offer an effective therapeutic regimen. However, the patients should be monitored for potential adverse effects. More studies are needed to determine the long-term safety and efficacy of these combinations