Dercum's Disease: A Rare Disease of Painful Fatty Lumps.

Dercum's disease is a rare and poorly understood condition characterized by painful subcutaneous adipose tissue growth that can occur anywhere beneath the skin surface. We present the case of a 27-year-old man with no significant medical history who had been experiencing painful subcutaneous nodules for two years. Skin biopsy revealed the proliferation of mature adipocytes that were surrounded by fibrous septa. There are currently no treatments approved by the US Food and Drug Administration for Dercum's disease, and the effectiveness of treatments that have been attempted is variable.

Intravascular Papillary Endothelial Hyperplasia Involving the Hand: A Case Report of a Rare Entity.

Masson's tumor, also named intravascular papillary endothelial hyperplasia (IPEH), is a rare, benign vascular tumor. Evaluation by clinical features can be confused with other soft tissue tumors. Therefore, the diagnosis should be confirmed by histopathological examination. The patient reported here is 67 years old and came to us with a small painful lesion over the left thumb of about two months duration. Histopathological examination was consistent with Masson's tumor (IPEH) following excisional biopsy, with good functional outcomes. To the best of our knowledge, this is the first report of this entity from Kuwait. Dermatologists and surgeons should know about this rare entity and its unusual presentation, to be able to distinguish it from similar presenting serious conditions, especially angiosarcoma. Through this report, we purport to facilitate recognition of this condition apart from some other conditions it may mimic.

The combined effect of CO2 laser, topical diclofenac 3%, and imiquimod 5% in treating high-risk basal cell carcinoma.

BACKGROUND: Some basal cell carcinoma (BCC) patients are considered as a high risk regarding the site, size, histopathological variant, or recurrence. High-risk BCC is a challenging therapeutic problem due to the trial to balance between complete surgical excision from one side and tissue preservation from the other side. AIM: To evaluate the efficacy of combining ablative CO2  laser, imiquimod 5%, and diclofenac 3% as a therapeutic regimen in high-risk and inoperable BCC. PATIENTS/METHODS: The study was conducted on 14 patients that were assessed clinically and pathologically then categorized regarding the site, size, histopathology, and fitness for surgery as high-risk inoperable BCC. They received an ablative session of CO2  laser, followed by application of diclofenac sodium 3% gel once daily for 5 days and imiquimod 5% cream for another 2 days. RESULTS: The study included 11 males and 3 females. Nine lesions were located on the scalp, 4 on the face, and one lesion on the trunk. All lesions were of large size >5 cm in diameter. Histopathology showed 4 patterns: nodular type in 8 patients, infiltrating type in 3 patients, metatypical type in 2 patients, and micronodular type in one patient. At the end of the treatment period, 9 patients showed significant (moderate to marked) improvement while 5 patients showed weak (poor to mild) response. Significant improvement was more observed in nodular type. Relapse was more observed during the 5th to 6th months with 2 patients showed no relapse. CONCLUSION: This combined regimen is a good alternative therapeutic modality in high-risk inoperable BCC especially the nodular pathologic pattern.

Superficial Morphea: Clinicopathological Characteristics and a Novel Therapeutic Outcome to Excimer Light Therapy.

INTRODUCTION: Superficial morphea (SM) is an uncommon entity that was described in the literature without definitive correlation to localized scleroderma (LS) or other atrophoderma diseases. AIM: To demonstrate the clinicopathological features of SM and evaluate the efficacy of different therapeutic modalities in its management. PATIENTS AND METHODS: A total of 28 patients with SM were studied during the period from 2010 to 2015. Clinicopathological features and therapeutic outcomes were recorded and analyzed. RESULTS: Clinically, SM was predominant in females (71.4%) with an average onset at 33 years of age and an average duration of 15 months. It was commonly presented as asymptomatic, darkly pigmented, and multiple and slightly indurated patches. The lesions were mostly ill-defined, large-sized, and located more on the trunk. Histologically, thickening of collagen fibers was observed either localized to the papillary dermis only (38.9%) or extended into the upper reticular dermis (61.1%). Elastic fibers were generally diminished in the upper reticular dermis while the number of fibroblasts and basal melanin pigmentation were increased in the majority of cases (92.9% and 96.4%, respectively). The most commonly associated diseases were diabetes mellitus (50%) and hepatitis C virus (HCV) infection (42.8%), and their incidence was significantly higher than that in patients with LS. Excimer light showed promising effective results in the treatment of most cases (78.9%) while the response to other modalities such as topical corticosteroid alone or in combination with tacrolimus or treatment with UVA1 alone was less effective (7.1%, 23.1%, and 5%, respectively). CONCLUSION: Our results proposed that SM is a distinctive clinicopathological variant and not a stage in the spectrum of LS. The novel response of SM to excimer light and not for UVA1 therapy also suggests the different therapeutic outcome of SM from LS. Although SM has a significant association with DM and HCV infection, they seem not to affect the course of the disease.

Clinicopathologic, immunophenotyping and cytogenetic analysis of Sweet syndrome in Egyptian patients with acute myeloid leukemia.

BACKGROUND: Sweet syndrome (SS) is an uncommon dermatologic disorder that could be associated with hematologic malignancies. OBJECTIVE: To describe the clinicopathologic, immunophenotyping and cytogenetic characteristics of SS in Egyptian patients with acute myeloid leukemia (AML). METHODS: The study was conducted during the period from April 2011 to March 2015. For each patient, a clinical evaluation and histological assessment of cutaneous lesions were recorded. Diagnostic investigations, immunophenotyping and cytogenetic features of leukemia were analyzed. Therapeutic monitoring and follow up of both diseases were registered. RESULTS: The study included 13 patients (7 males and 6 females) with a mean age of 44.4±17.49years. Fever was recorded in 10 cases and most of the lesions (61.5%) appeared during the post remission period. Clinically, the lesions were more frequently located on the extremities (61.5%), presented as solitary lesion (53.8%) and mostly tender (69.2%). Atypical presentations were observed in 5 cases and included ulcerative lesion, indurated mass and a gangrenous mass. Histological assessment revealed two patterns of inflammatory reactions described as classic (dermal) form (38.5%) and deep (subcutaneous) form (61.5%). Laboratory investigations showed leukocytosis in 61.5%, neutropenia in 38.5%, anaemia in 92.3%, and thrombocytopenia in 84.6%. Bone marrow aspiration and biopsy showed suppressed trilineage hematopoesis in 84.6% and blast cell count >50% in 69.2%. The common subtypes of AML included M2 and M4 (23.1% for each). Cytogenetic studies revealed genetic abnormalities in 69.2% of cases. Most of the cases (76.9%) showed a poor response to oral prednisolone but responded well to alternative therapies, including dapsone, colchicine and cyclosporine. CONCLUSION: Sweet syndrome associated with AML may show atypical clinical forms that have an aggressive course and is mostly associated with subcutaneous involvement. Although chemotherapy of AML may play a significant role in the development of SS, the exact mechanism remains unclear. The disease is considered a steroid refractory and genetic abnormalities may have a role in altering the classic nature of the disease.

Role of Helicobacter pylori in common rosacea subtypes: a genotypic comparative study of Egyptian patients.

Helicobacter pylori was incriminated as an etiological factor of rosacea. However, there is still controversy about this association. We conducted a comparative study in order to assess the role of H. pylori in rosacea patients who had dyspeptic symptoms. The study included 68 patients and 54 controls. Screening for H. pylori was performed and positive cases were referred for gastric endoscopy. The inflammatory response and bacterial density were evaluated in gastric biopsy. H. pylori vacA alleles, cagA and iceA genotypes were assessed by polymerase chain reaction. We found that 49 rosacea (72%) and 25 controls (46.3%) were infected with H. pylori. Thirty-one rosacea cases were papulopustular (PPR) while 18 were erythematotelangiectatic (ETR). Gastric ulceration was higher in PPR cases (38.7%) than ETR (11.1%) and controls (12%). A significant inflammatory reaction was observed more in PPR cases (74.2%) compared with 44.4% in ETR (P = 0.04) and 44% in controls (P = 0.02). Analysis of H. pylori genotypes revealed that vacA s1m1 was more identified in PPR cases (54.8%) compared with 22.2% in ETR (P = 0.03) and 16% in controls (P = 0.003). There was a significant elevation of cagA/vacA s1m1 positivity in PPR cases. After the eradication regimen of H. pylori, a significant improvement (P < 0.05) was observed in 15 out of 27 PPR cases (55.6%) compared with three out of 17 ETR (17.6%). We concluded that H. pylori has a significant role in rosacea patients who had dyspeptic symptoms. The PPR type is more influenced by H. pylori and this is regarded as being because of certain virulent strains that increase the inflammatory response in gastric mucosa and also in cutaneous lesions.

Ulcerative granular cell tumor: a clinicopathological and immunohistochemical study.

Granular cell tumor (GCT) is uncommonly presented with cutaneous ulcer. We examined the clinicopathological and immunohistochemical features of this ulcerative form in fourteen cases that may raise the awareness of this variant. The study included 11 males and 3 females with a mean age 31.5 ± 7.42 years. All cases were presented with large solitary ulcer with indurated base, elevated border, skin colored margin, and necrotic floor. Twelve lesions were located on the extremities and two lesions on the genital region. Histologically, the lesions showed dermal infiltrate composed of large polygonal cells with granular cytoplasm and characteristic infiltration of the dermal muscles in all cases. Immunostaining showed positive reaction for S100 (14/14), NSE (14/14), CD68 (5/14), and Vimentin (7/14) while HMB45, CK, EMA, and Desmin were negative. We hope that this paper increases the awareness of ulcerative GCT and consider it in the differential diagnosis of ulcerative lesions.