ABSTRACT
Common variable immunodeficiency (CVID) is one of the primary immunodeficiency disorders, which is characterized by hypogammaglobulinemia, defect in antibody synthesis and recurrent sinorespiratory bacterial infections. Granuloma is an uncommon skin manifestation in primary immunodeficiency disorders. Here we present a 5-year-old girl who had asymptomatic erythematous plaques on the face and legs. She had a past medical history of recurrent sinorespiratory and gastrointestical bacterial infections since 2 years old. The histopathologic findings of the skin lesions showed sarcoid-like granulomas with focal necrobiosis. No specific organism was found with evaluation. Cells forming granulomas were strongly positive for CD3 or CD68. CD4/CD8 ratio was estimated less than 1. Laboratory examination showed low hemoglobin, low B cell and normal T cell number, low IgA and IgG levels.
Subject(s)
Agammaglobulinemia , Bacterial Infections , Cell Count , Common Variable Immunodeficiency , Granuloma , Hemoglobins , Immunoglobulin A , Immunoglobulin G , Leg , Necrobiotic Disorders , Child, Preschool , Skin , Skin ManifestationsABSTRACT
Neutrophilic dermatosis of the dorsal hands (NDDH) is a localized, pustular variant of acute febrile neutrophilic dermatosis (Sweet syndrome). The lesions of NDDH clinically resemble those of Sweet syndrome (SS), but they differ from classic SS according to their locations (NDDH is predominantly restricted to the dorsal hands) and the smaller number of accompanying systemic symptoms. The histology of the NDDH lesion shows a dense dermal neutrophilic infiltration. The lesions rapidly resolve after systemic corticosteroid and/or dapsone therapy. We herein report on a case of neutrophilic dermatosis of the dorsal hands in a 34-year-old woman. The patient also had skin changes with erythematous plaque on the right lips.
Subject(s)
Adult , Female , Humans , Dapsone , Hand , Lip , Neutrophils , Skin , Skin Diseases , Sweet SyndromeABSTRACT
BACKGROUND: Extensive alopecia areata (EAA) is resistant to multiple individual treatment modalities and has poor prognosis for cosmetically adequate regrowth. Anthralin is a widely used topical anti-psoriatic drug that may have an immunomodulating effect on AA as is does in psoriasis. But, there has only been small number of clinical trials of anthralin in the treatment of AA. OBJECTIVE: The purposes of the study were to evaluate the efficacy, prognostic factor, side effects and recurrence rate of topical anthralin therapy in treatment-resistant EAA. METHODS: A total of 16 cases of EAA (>50% scalp hair loss) who had failed in previous treatments were subjected in this study. Anthralin in 0.5% concentrations was applied to alopectic lesions for 1 hour daily over 4 weeks, gradually increasing anthralin concentration until low-grade erythema and pruritus develops. Treatment was withdrawn after complete response or if there were no signs of improvement at 6 months. Responders were followed up for 6 months after discontinuation of therapy. RESULTS: The overall response rate was 62.5%, complete response (>90% regrowth or cosmetically acceptable appearance) was obtained in 25% of cases and, good response (50~99% regrowth) in 39.5% of cases. In this study, among the investigated prognostic factors, there were no statistically significant factors (p<0.05, Fisher exact test). The most frequent side effects were therapeutically induced mild pruritus (93.8%), erythema (93.8%) and scale (56.3%). Other side-effects were transient folliculitis (31.3%) and regional lymph adenopathy (12.5%). Relapse was observed in 60% of responders after 6 month of follow up. CONCLUSION: Topical anthralin for treatment-resistant EAA is an effective therapy with tolerable side effects. Therefore, we propose the topical anthralin as a reasonable therapeutic option for treatment-resistant EAA.
Subject(s)
Alopecia , Alopecia Areata , Anthralin , Erythema , Folliculitis , Hair , Prognosis , Pruritus , Psoriasis , Recurrence , ScalpABSTRACT
Scrub typhus (tsutusgamushi disease) is an acute febrile illness caused by Orientia tsutsugamushi, which is endemic in the Asia-Pacific area. Scrub typhus in pregnant woman is uncommon and some reports have suggested that this disease may be associated with increased fetal loss. An effective and safe treatment regime for scrub typhus on pregnancy has not been established and the clinical outcome on the pregnant woman and fetus is unclear. We report a case of scrub typhus during pregnancy treated with azithromycin, which showed no recurrence and no fetal and neonatal complications.
Subject(s)
Female , Humans , Pregnancy , Azithromycin , Fetus , Orientia tsutsugamushi , Pregnant Women , Recurrence , Scrub TyphusABSTRACT
We report a 58-year-old woman with an unusual clinical presentation of macular amyloidosis who had erythematous depressed patches without rippled pattern. The lesion was localized involving only the face: both lateral sides of the forehead. A skin biopsy revealed amyloid deposits on the papillay dermis. An immunofluorescence study demonstrated positivity for IgA in the amyloid deposits. The skin lesion of this patient could easily be confused with chronic cutaneous lupus erythematousus and the immunofluorescence finding suggests the possibility of specific immunoglobulin absorption of amyloid deposits.
Subject(s)
Female , Humans , Middle Aged , Absorption , Amyloid , Amyloidosis , Biopsy , Dermis , Fluorescent Antibody Technique , Forehead , Immunoglobulin A , Immunoglobulins , Plaque, Amyloid , SkinABSTRACT
Rheumatoid neutrophilic dermatitis (RND) is a rare distinctive complication of rheumatoid arthritis (RA). Clinically, RND is characterized by slightly tender erythematous patches and papules, often associated with vesicles or pustules, which are symmetrically distributed on the extensor surfaces of the extremities. Histopathological findings revealed a dermal neutrophilic infiltration with leukocytoclasia without vasculitis. We report a case of rheumatoid neutrophilic dermatitis associated with seronegative RA.
Subject(s)
Arthritis , Arthritis, Rheumatoid , Dermatitis , Extremities , Neutrophils , VasculitisABSTRACT
Lichenoid drug eruption is a lichenoid skin eruption caused by certain drugs and compounds, and can be similar to lichen planus. A 51-year-old man who had taken antihypertensive drugs (enalapril, dilazep dihydrochloride) and cholesterol lowering drug (atorvastatin) for 4 years had received allopurinol for asymptomatic hyperuricemia for 4 months. He developed pruritic erythematous papules and plaques with silvery scales on the face, trunk and extremities after taking allopurinol for 3 months. He had no fever, no eosinophilia and no systemic abnormalities. Histopathologic findings showed hyperkeratosis, parakeratosis, hypergranulosis, band-like lymphohistiocytic infiltration and perivascular lymphohistiocytic infiltration in the dermis, which were compatible with lichenoid drug eruption. The skin rashes disappeared after he stopped taking allopurinol and was treated with steroids.
Subject(s)
Humans , Middle Aged , Allopurinol , Antihypertensive Agents , Cholesterol , Dermis , Dilazep , Drug Eruptions , Enalapril , Eosinophilia , Exanthema , Extremities , Fever , Hyperuricemia , Lichen Planus , Parakeratosis , Skin , Steroids , Weights and MeasuresABSTRACT
Metastatic cancer of the umbilicus is very rare and is called Sister Mary Joseph's nodule. Metastasis to the umbilicus usually presents as diffuse spreading of primary cancer or recurrence after treatment of primary cancer, carries very poor prognosis, and is generally inoperable. However, umbilical metastasis presents rarely as a first sign of primary cancer and can lead to its diagnosis. The diagnosis is usually made with the histologic examination and the most common primary cancer is an adenocarcinoma of the stomach. We report a case of umbilical metastasis as a first sign of gastric cancer which leads to its diagnosis. This report emphasizes the need for careful evaluation of any umbilical lesion.
Subject(s)
Humans , Adenocarcinoma , Neoplasm Metastasis , Prognosis , Recurrence , Siblings , Sister Mary Joseph's Nodule , Stomach , Stomach Neoplasms , UmbilicusABSTRACT
A digital mucous cyst is a benign, non-inflammatory disease frequently observed on the dorsal aspect of the fingers and toes. Multiple punctures with extraction of the cyst contents and intralesional steroid injections are the suggested treatments. However, this approach has shown a high failure and recurrence rate. Cryotherapy has had a better success rate but it is a more painful procedure. Surgical excision of the communicating tract between the cyst and joint has been reported to have the highest cure rate but it requires highly skilled surgery, and the surgery has the risk of severe complications including contractures of the joints and acquired nail deformity. We report three patients with digital mucous cysts treated by sclerotherapy without complications.
Subject(s)
Humans , Congenital Abnormalities , Contracture , Cryotherapy , Fingers , Joints , Nails , Punctures , Recurrence , Sclerotherapy , Sodium , Sodium Tetradecyl Sulfate , ToesABSTRACT
Cowden syndrome is a rare autosomal dominant disorder characterized by mucocutaneous alterations including multiple facial trichilemmomas, oral mucosal papillomatosis, and acral keratoses. Extracutaneous lesions include polyposis of the gastrointestinal tract, and other multiple hamartoma of the internal organs. A 39 year-old man presented with multiple verruca-like papules and lichenified patches on the face, one year ago. He had been treated for atopic dermatitis for more than 10 years. Although he had been treated in accordance with atopic dermatitis for 1 year, his skin lesions had not improved, so a skin biopsy was performed. The skin biopsy specimen revealed the typical finding of trichilemmoma. Colonoscopy diagnosed diffuse colorectal polyposis. The clinical and hisopathologic findings were consistent with Cowden syndrome. We, herein, report a case of Cowden syndrome with atopic dermatitis.
Subject(s)
Adult , Humans , Biopsy , Colonoscopy , Dermatitis, Atopic , Gastrointestinal Tract , Hamartoma , Hamartoma Syndrome, Multiple , Keratosis , Papilloma , SkinABSTRACT
An umbilical omphalomesenteric duct polyp arises from remnants of the omphalomesenteric duct, which connects the midgut to the Yolk sac of the embryo and is closed completely at 7 weeks. Persistence of the omphalomesenteric duct shows various clinical manifestations of embryologic anomalies such as umbilical enteric fistula, Meckel's diverticulum or vitelline cysts. In particular, the umbilical omphalomesenteric duct polyp should be clinically discerned from persistent granulation tissue or pyogenic granuloma and attention should be given to external openings with discharge, which can be suggestive of underlying anomalies. We herein report an case of an umbilical omphalomesenteric duct polyp in a 10-year-old male who had no underlying anomalies.
Subject(s)
Child , Humans , Male , Embryonic Structures , Fistula , Granulation Tissue , Granuloma, Pyogenic , Meckel Diverticulum , Polyps , Vitelline Duct , Vitellins , Yolk SacABSTRACT
Acrosyringeal nevus is a rare condition, which includes proliferation of acrosyringeal keratinocytes, and the proliferation extends from the undersurface of the epidermis down into the dermis as thin anastomosing cords. It is, however, controversial whether acrosyringeal nevus is the same lesion as eccrine syringofibroadenoma, because of several clinicopathological differences. We report a case of benign tumor, consisting of eccrine structures, which occurred in a 60-year-old man. The histopathological features of the excisional biopsy were consistent with acrosyringeal nevus.
Subject(s)
Humans , Middle Aged , Biopsy , Dermis , Epidermis , Keratinocytes , NevusABSTRACT
Dermatomyositis is a rare disease of unknown origin that combines an inflammatory myopathy with characteristic cutaneous findings. Scalp disease has rarely been considered as a manifestation of dermatomyositis and is often misdiagnosed as being scalp paosiasis or seborrheic dermatitis. We report a case of 27-year-old woman who presented with brownish alopecic patches on the scalp, and chronic proximal muscle weakness.
Subject(s)
Adult , Female , Humans , Cicatrix , Dermatitis, Seborrheic , Dermatomyositis , Muscle Weakness , Myositis , Rare Diseases , ScalpABSTRACT
Pilomatricoma is a common, benign, cutaneous neoplasm, differentiating towards elements of the hair matrix and shaft. It typically presents as a deep-seated, solitary, firm nodule with overlying normal epidermis. However, pilomatricoma with bullous appearance is very rare. We report two cases of pilomatricoma with bullous appearance. The first case was an 11-year-old male who presented with 6 month history of a soft, erythematous, translucent polypoid mass on the left upper arm. The other case was a 20-year-old female who presented with 12 month history of a soft, slightly-brownish, peduncluated mass with indurated nodules on the left upper arm. In both cases, the histopathologic findings revealed multiple islands of shadow cells, with small foci of basophilic cells at the periphery of the deep dermis. There was dilation of lymphatic vessels in the overlying dermis, which explained the clinical bullous appearance.
Subject(s)
Child , Female , Humans , Male , Young Adult , Arm , Basophils , Dermis , Epidermis , Hair , Islands , Lymphatic Vessels , PilomatrixomaABSTRACT
Pilomatricoma is a common, benign, cutaneous neoplasm, differentiating towards elements of the hair matrix and shaft. It typically presents as a deep-seated, solitary, firm nodule with overlying normal epidermis. However, pilomatricoma with bullous appearance is very rare. We report two cases of pilomatricoma with bullous appearance. The first case was an 11-year-old male who presented with 6 month history of a soft, erythematous, translucent polypoid mass on the left upper arm. The other case was a 20-year-old female who presented with 12 month history of a soft, slightly-brownish, peduncluated mass with indurated nodules on the left upper arm. In both cases, the histopathologic findings revealed multiple islands of shadow cells, with small foci of basophilic cells at the periphery of the deep dermis. There was dilation of lymphatic vessels in the overlying dermis, which explained the clinical bullous appearance.