Mild Perivascular Lymphocytic Infiltrate Research Articles

Diffuse hyperpigmentation with guttate hypopigmentation: a new pigmentary disorder.

Diffuse hyperpigmentation with guttate hypopigmentation (DHGH) is a new acquired pigmentary disorder. Only a few cases have previously been reported in the Chinese population, in Chinese. To summarise the clinical, dermoscopic, and histopathological findings of DHGH in the English literature, to improve the recognition and management of this condition. This was a retrospective study to summarise the clinical, dermoscopic, and pathological findings of nine cases of DHGH. All nine patients with DHGH were female. The age at onset varied from 6 to 24 years (median 17 years). Patients were generally in good health without systemic disease. The lesions were often generalised to the trunk and extremities without any discomfort. Typical lesions were characterised by multiple uniform hypopigmented spots, 2-5mm in diameter, irregularly distributed over diffuse hyperpigmentation. Dermoscopy revealed multiple blurred patchy areas of brownish pigmentation, sparse linear and dotted vessels, and perifollicular pigmentation on a white to bright white background, surrounded by brown hyperpigmentation. Histopathological findings included mild abnormal pigment of the epidermis, focal vacuolar degeneration of the basal cells, mild pigment incontinence and perivascular lymphocytic infiltration in the dermis. DHGH is a new entity with distinctive clinical manifestations that differ from those of other known pigmentary disorders. So far, DHGH has only been reported in the Chinese population. It may not be uncommon and has not received much attention due to the few reports. The aetiology and pathogenesis of DHGH are still unknown and require further investigation.

Classic Kaposi Sarcoma: An Unusual Presentation in an HIV-Negative Man

Kaposi sarcoma (KS) is a type of cancer that develops in blood vessels of the mouth, lymph nodes, and lungs. Our case report is about a 66-year-old man with purple patches and plaques, as well as nodules distributed in the legs and arms. Biopsy of the skin lesions showed angiomatous proliferation of small vessels with mild perivascular lymphocytic infiltration with plasma cells. We started chemotherapy for this disseminated form of Kaposi sarcoma.

A Connexin Gene (GJB3) Mutation in a Chinese Family With Erythrokeratodermia Variabilis, Ichthyosis and Nonsyndromic Hearing Loss: Case Report and Mutations Update.

Background: Gap junctions formed by connexins are channels on cytoplasm functioning in ion recycling and homeostasis. Some members of connexin family including connexin 31 are significant components in human skin and cochlea. In clinic, mutations of connexin 31 have been revealed as the cause of a rare hereditary skin disease called erythrokeratodermia variabilis (EKV) and non-syndromic hearing loss (NSHL). Objective: To determine the underlying genetic cause of EKV, ichthyosis and NSHL in three members of a Chinese pedigree and skin histologic characteristics of the EKV patient. Methods: By performing whole exome sequencing (WES), Sanger sequencing and skin biopsy, we demonstrate a Chinese pedigree carrying a mutation of GJB3 with three patients separately diagnosed with EKV, ichthyosis and NSHL. Results: The proband, a 6-year-old Chinese girl, presented with demarcated annular red-brown plaques and hyperkeratotic scaly patches on her trunk and limbs. Her mother has ichthyosis with hyperkeratosis and geographic tongue while her younger brother had NSHL since birth. Mutation analysis revealed all of them carried a heterozygous missense mutation c.293G>A of GJB3. Skin biopsy showed many grain cells with dyskeratosis in the granular layer. Acanthosis, papillomatosis, and a mild superficial perivascular lymphocytic infiltrate were observed. Conclusion: A mutation of GJB3 associated with EKV, ichthyosis and NSHL is reported in this case. The daughter with EKV and the son with NSHL in this Chinese family inherited the mutation from their mother with ichthyosis. The variation of clinical features may involve with genetic, epigenetic and environmental factors.

Histopathologic Patterns in Dermatophyte Infections

Background: Dermatophyte infections (DI) mimic a wide range of inflammatory diseases clinically and hyphae often play hide and seek on microscopy. Pathologists encounter biopsies with no clinical suspicion. In such instances, we need to recognize clues that point to the diagnosis. Aim: This study aims to describe the histopathologic patterns in DI. Materials and Methods: A total of 20 cases of DI were reviewed for various epidermal and dermal patterns. Results: Altered pattern of cornification was seen in all 20 cases (100%), the commonest being parakeratosis (75%), often in small mounds (55%), followed by compact orthokeratosis (50%). Neutrophils (65%) and crusting (55%) were seen, with sandwich sign noted in 65% cases. The reaction pattern most commonly seen was spongiosis (50%). Pustules were rare (20%). Mild superficial perivascular lymphocytic infiltrate (45%) was the commonest dermal pattern. Neutrophils (45%) and eosinophils (25%) were less frequent. Fungal hyphae were identified either within the zone of compact orthokeratosis (55%) or at the junction between two different patterns of cornification (45%). The limitations of this study are relatively small number of cases studied and lack of correlation with fungal culture, as they were not done in 90% of patients due to lack of clinical suspicion. Conclusion: Histopathologic patterns in DI are most often subtle, in the form of small mounds of parakeratosis and mild superficial perivascular dermal infiltrate, which overlap with several other skin diseases. Neutrophils and sandwich sign are not seen in almost half the cases. A careful search for fungi together with periodic acid–Schiff stain is mandatory whenever altered cornification is seen. Investigations such as potassium hydroxide preparation and cultures are not done unless there is clinical suspicion, which is absent in majority of the cases. Histopathology offers the only opportunity to establish a diagnosis in this easily curable condition.

Bilateral Chilblain-like Lesions of the Toes Characterized by Microvascular Remodeling in Adolescents During the COVID-19 Pandemic

Chilblain-like lesions have been one of the most frequently described cutaneous manifestations during the COVID-19 pandemic. Their etiopathogenesis, including the role of SARS-CoV-2, remains elusive. To examine the association of chilblain-like lesions with SARS-CoV-2 infection. This prospective case series enrolled 17 adolescents who presented with chilblain-like lesions from April 1 to June 30, 2020, at a tertiary referral academic hospital in Italy. Macroscopic (clinical and dermoscopic) and microscopic (histopathologic) analysis contributed to a thorough understanding of the lesions. Nasopharyngeal swab, serologic testing, and in situ hybridization of the skin biopsy specimens were performed to test for SARS-CoV-2 infection. Laboratory tests explored signs of systemic inflammation or thrombophilia. Structural changes in peripheral microcirculation were investigated by capillaroscopy. Of the 17 adolescents (9 [52.9%] male; median [interquartile range] age, 13.2 [12.5-14.3] years) enrolled during the first wave of the COVID-19 pandemic, 16 (94.1%) had bilaterally localized distal erythematous or cyanotic lesions. A triad of red dots (16 [100%]), white rosettes (11 [68.8%]), and white streaks (10 [62.5%]) characterized the dermoscopic picture. Histologic analysis revealed a remodeling of the dermal blood vessels with a lobular arrangement, wall thickening, and a mild perivascular lymphocytic infiltrate. SARS-CoV-2 infection was excluded by molecular and serologic testing. In situ hybridization did not highlight the viral genome in the lesions. This study delineated the clinical, histologic, and laboratory features of chilblain-like lesions that emerged during the COVID-19 pandemic, and its findings do not support their association with SARS-CoV-2 infection. The lesions occurred in otherwise healthy adolescents, had a long but benign course to self-resolution, and were characterized by a microvascular remodeling with perivascular lymphocytic infiltrate but no other signs of vasculitis. These results suggest that chilblain-like lesions do not imply a concomitant SARS-CoV-2 infection. Ongoing studies will help clarify the etiopathogenic mechanisms.

Frontal fibrosing alopecia: a clinicopathological study of 22 cases from Shiraz, Southern Iran

Background: Since its recognition, frontal fibrosing alopecia (FFA) has increasingly been studied globally in terms of its diverse aspects. Having found no studies describing this condition in Southern Iran, we examined the different clinicopathological features of patients with FFA who referred to Faghihi Hospital, Southern Iran, between 2013 and 2018. Methods: We searched the archives of the Pathology Department of Faghihi Hospital for the cases with a diagnosis of FFA. Due to its similar histopathologic features with lichen planopilaris, the final diagnosis was made using clinical correlations. Results: Twenty-two patients were enrolled; all were female. Fifteen (68.2%) presented with the disease before menopause. Besides frontal and/or temporal hairline involvement in all the patients, eyebrow alopecia, eyelash loss, body hair loss, and facial papules were present in 81.8%, 27.3%, 50%, and 68.2% of them, respectively. Dermoscopic findings included follicular opening loss (100%), honeycombing of the scalp (81.8%), multiple white dots (77.3%), perifollicular erythema (63.6%), and perifollicular scales (59.1%). The histopathologic examination revealed follicular dropout (95.4%), perifollicular lymphocytic infiltration in the infundibulum and isthmus of the follicles (81.8%), perifollicular fibroplasia (77.3%), intact interfollicular epidermis (59.1%), mild perivascular lymphocytic infiltration in the upper dermis (54.5%), and apoptotic keratinocytes in the infundibulum and isthmus (50%). The most common comorbidity was hypothyroidism (40.9%). Conclusions: Thediagnosis of FFA should be considered in both premenopausal and postmenopausal women. Eyebrow alopecia, eyelash loss, body hair involvement, and facial papules are helpful clues in the diagnosis. The coexistence of hypothyroidism with FFA suggests immunological involvement in the pathogenesis.

A localized non-healing ulcer: an unusual clinical presentation of pemphigus vulgaris

<p>Pemphigus vulgaris is an autoimmune blistering disorder commonly involving oral mucosa and skin<strong> </strong>with characteristic acantholysis that results in formation of fluid filled blisters and painful erosions. Herein we report a case distinguished by its unusual clinical presentation.<strong> </strong>A twenty-five year old man, presented to our outpatient department of dermatology, complaining of ulcer on forehead in the last one year. Examination revealed single ulcer covered with crust on right side of forehead. Clinically lupus vulgaris, pyoderma gangrenosum, recurrent herpes simplex, leishmaniasis and pemphigus vulgaris were kept as differential diagnosis. Tzanck smear revealed multiple acantholytic cells and mild neutrophilic inflammatory infiltrate. Histological examination of biopsy sample showed suprabasal clefting, acantholytic cells and mild perivascular dermal lymphocytic infiltrate. Features were suggestive of pemphigus vulgaris. Significant improvement occurred after three months of treatment with oral prednisolone, azathioprine and topical steroid. Although oral and skin involve­ment is common, in the form of flaccid blisters and erosions, but non healing ulcer is unusual. This may represent a period of limited activity during this chronic disease. There is paucity of data in literature regarding this. So, this<strong> </strong>case report highlights a, perhaps unique, clinical presentation of this autoimmune disease.<strong></strong></p>

The spectrum of fibroelastolytic papulosis: a retrospective case series.

Introduction: There is an overlap between clinical and pathological features of the entities known as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). Although the term fibroelastolytic papulosis has been used to encompass both entities as spectrum variants of a one and only disease, many authors still differentiate the two.Methods: Cases of fibroelastolytic papulosis confirmed by histopathologic examination were retrospectively reviewed within a time frame of 2.5 years.Results: Five cases of fibroelastolytic papulosis were identified. All patients were females aged between 63 and 78 years, presenting with an asymptomatic eruption of isolated or coalescing white to yellow papules on the neck. Two of the patients also had involvement of other anatomical areas. In all cases histology demonstrated a significantly decreased or absent papillary dermal elastic plexus, a mild superficial perivascular lymphocytic infiltrate and sparse melanophages in the papillary dermis, without interface changes. A decrease in upper reticular dermal elastic fibers was noted in four cases and thickening of dermal collagen bundles in three cases. Dilated superficial dermal vessels were present in 3 cases and mild epidermal atrophy in one case.Conclusion: We find the differentiation of PXE-PDE and WFPN to be somewhat theoretical, often confusing, and based on non-significant subtle histological and clinical differences. In our opinion, we should not continue to use these terms, but favor the common term of fibroelastolytic papulosis.

Are anti-ganglioside antibodies associated with proventricular dilatation disease in birds?

The identification of Parrot bornaviruses (PaBV) in psittacine birds with proventricular dilatation disease (PDD) has not been sufficient to explain the pathogenesis of this fatal disease, since not all infected birds develop clinical signs. Although the most accepted theory indicates that PaBV directly triggers an inflammatory response in this disease, another hypothesis suggests the disease is triggered by autoantibodies targeting neuronal gangliosides, and PDD might therefore resemble Guillain-Barré Syndrome (GBS) in its pathogenesis. Experimental inoculation of pure gangliosides and brain-derived ganglioside extracts were used in two different immunization studies. The first study was performed on 17 healthy chickens (Gallus gallus domesticus): 11 chickens were inoculated with a brain ganglioside extract in Freund’s complete adjuvant (FCA) and six chickens inoculated with phosphate-buffered saline. A second study was performed five healthy quaker parrots (Myiopsitta monachus) that were divided into three groups: Two quaker parrots received purified gangliosides in FCA, two received a crude brain extract in FCA, and one control quaker parrot received FCA alone. One chicken developed difficult in walking. Histologically, only a mild perivascular and perineural lymphocytic infiltrate in the proventriculus. Two quaker parrots (one from each treatment group) had mild lymphoplasmacytic encephalitis and myelitis. However, none of the quaker parrots developed myenteric ganglioneuritis, suggesting that autoantibodies against gangliosides in birds are not associated with a condition resembling PDD.

A study on histopathological changes in lesions of vitiligo in Karnataka population

<p class="abstract"><strong>Background:</strong> <span lang="EN-IN">Vitiligo is considered to be symptom less and its presentation is boundless varying from isolated focal lesion to bizarre generalized lesions. The present study was undertaken to study the histopathological changes in lesions of vitiligo in south Indian population.</span></p><p class="abstract"><strong>Methods:</strong> 150 patients with mild to moderate vitiligo features attending the outpatient department of Dermatology, Venereology and Leprology at Chitageri General Hospital and Bapuji Hospital attached to J.J.M Medical College, Davengere were utilized to study the histopathological features in vitiligo and its association with other diseases.<strong></strong></p><p class="abstract"><strong>Results:</strong> <span lang="EN-IN">Destruction of melanocytes at dermo-epidermal junction was noted. We have observed presence of melanocytes in the basal layer of the epidermis on left side of the lesion, whereas decreased melanocytes in the basal layer of the epidermis on the right side of the same lesion in the present study. Dermis with mild perivascular lymphocytic infiltration and acanthosis along with mono nuclear cell infiltration in the upper dermis was observed. </span></p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-IN">The present study gives better knowledge to the clinicians about the lesions of vitiligo and its pathogenesis.</span></p>

Coexistence of porokeratosis ptychotropica with porokeratosis of Mibelli in a Chinese man.

Coexistence of porokeratosis ptychotropica with porokeratosis of Mibelli in a Chinese man.

Prurigo Pigmentosa

Prurigo pigmentosa (PP) is a rare inflammatory dermatosis originally reported in Japan. Since then, most reports have originated from Asia, and to a lesser extent from Europe. Although the pathogenesis remains unclear, it is now established that PP is linked to ketoacidotic states. Four patients diagnosed with PP were identified from the dermatopathology database at the American University of Beirut Medical Center between January 2009 and December 2013. Clinicopathologic findings in the 4 patients were similar to those previously reported in the literature. The patients were all female with a mean age of 23.5 years. They all presented with itchy erythematous reticulated papulovesicles/plaques leaving variable reticulated brownish patches. Two patients had, in addition, annular lesions arranged en cocarde and pustules, respectively. In 3 patients, the rash was associated with fasting or dieting. The rash had a predilection to the trunk and proximal part of the upper extremities. One patient had intergluteal area involvement. Two biopsy specimens revealed psoriasiform hyperplasia and neutrophilic exocytosis mimicking psoriasis or an impetiginized spongiotic dermatitis. One biopsy specimen exhibited a mild superficial perivascular lymphocytic infiltrate with ballooning and reticular degeneration, a picture mimicking a viral exanthema. Another biopsy specimen exhibited a picture similar to chronic spongiotic dermatitis. Although mostly described in Japan, PP has been described much less frequently in the Middle East region likely due to mis/underdiagnosis. Therefore, increased awareness is necessary especially because fasting is a common religious practice among Arab countries. Further investigations are necessary to better understand the etiopathogenesis of this rare entity.

Acquired idiopathic anhidrosis: A diagnosis often missed

Acquired idiopathic anhidrosis (AIA) is an uncommon entity characterized by anhidrosis in the absence of any neurologic or sweat gland abnormalities. The aim of this study was to characterize the clinical profile in a cohort of patients diagnosed with AIA at a tertiary dermatologic center. We retrospectively evaluated cases seen during a 10-year period. Inclusion criteria included all cases of generalized or partial anhidrosis with no obvious causes, confirmed by provocative starch-iodine sweat test. Ectodermal dysplasias, poral dysfunction from chronic dermatoses, autonomic dysfunction, and drug-induced causes were excluded. Fifteen Chinese patients were diagnosed with AIA, mostly healthy young men with no significant medical or drug history, with variable extent of body surface area involvement. Serum immunoglobulin E, a complete blood cell count, thyroid function test, and antinuclear antibody levels were unremarkable. Anhidrotic areas revealed normal eccrine appendages with mild perivascular and perieccrine lymphocytic infiltrate. There were no neurologic abnormalities. This was a retrospective study. Our study shows that AIA seems to be a heterogeneous group with no major dysfunction other than anhidrosis. Proper recognition and evaluation is paramount, especially for at-risk populations, so that appropriate measures on the prevention of heat injuries can be instituted.

Updated diagnosis criteria for confluent and reticulated papillomatosis: a case report.

Dear Editor: Confluent and reticulated papillomatosis (CRP), a rare skin disorder of unknown etiology, is characterized by hyperkeratotic papules confluent in the central area and reticulated hyperpigmented patch in the peripheral area1. Since Gougerot and Carteud first identified CRP in 1927, this disease has been diagnosed by using clinical courses, histological findings, and treatment results. However, other diseases, such as fungal infection or other pigmentary skin diseases, were frequently misdiagnosed as CRP because of the lack of diagnostic criteria for CRP. Therefore, Davis et al.1 proposed the diagnostic criteria for CRP on the basis of a study on 39 patients, as follows: (i) clinical findings of scaly brown macules and patches, with at least some appearing reticulated and papillomatous; (ii) involvement of the upper trunk and neck; (iii) negative fungal staining of scales; (iv) no response to antifungal treatment; and (v) excellent response to minocycline. However, a few cases of CRP sparing the upper trunk have also shown the typical histopathological findings, clinical findings, and treatment responses of CRP. A 42-year-old Korean woman presented to our clinic with a 4-year history of asymptomatic, brownish, and reticulated skin lesions in her antecubital and axillary regions (Fig. 1A). She reported that her skin lesions had worsened in May and improved slightly in October without any specific treatment. We performed a potassium hydroxide test to rule out fungal infection. Because the test result was negative at the antecubital fossa but positive at the axilla, we began treatment with oral itraconazole and topical flutrimazole cream for 2 weeks. Despite the 2 weeks of treatment, her skin lesions persisted. We then performed an axillary skin biopsy. Histopathological evaluation revealed papillomatosis and hyperkeratosis of the epidermis, and downward growth of the epidermis between the rete ridges (Fig. 2). Periodic acid Schiff staining did not reveal any fungal organism. On the basis of these findings, we diagnosed her condition as CRP limited to the flexural areas. After 2 months of doxycycline treatment (100 mg twice a day), her skin lesions completely disappeared (Fig. 1B). Fig. 1 (A) Hyperpigmented scaly macules with a reticular pattern on the axilla and antecubital fossa. (B) Complete resolution of the skin lesions after 2 months of doxycycline treatment. Fig. 2 Histopathological findings of the axillary lesion showing an orthokeratotic epidermis with acanthosis and papillomatosis plus mild perivascular lymphocytic infiltrate in the superficial dermis (H&E, ×200). Our patient exhibited only limited lesions at the antecubital fossae and axilla, which is inconsistent with the previous diagnostic criteria for CRP1. Several cases of CRP had involved various flexural areas alone, including the axilla2,3, popliteal fossa2, or antecubital regions4. These lesions fulfilled other diagnostic criteria for CRP. Therefore the criteria related to the primary affected sites should be revised. In addition, the criteria for fungal staining and response to antifungal treatment should be modified. Occasionally, CRP and fungal infection coexist5, as in this patient, however an important diagnostic consideration is that CRP lesions cannot be improved with antifungal treatment even in the presence of a positive fungal examination. Finally, minocycline3,4 has been known as the treatment of choice for CRP; however, other antibiotics have also been efficacious3. The exact reason for the effect of antibiotics is unclear, but many dermatologists speculate that their anti-inflammatory effect may be more predominant than their anti-bacterial effect1. As mentioned above, the previous diagnostic criteria for CRP had several limitations in cases of unusually involved location, co-infection with fungus, and use of antibiotics other than minocycline. Therefore, we propose a modified diagnostic criteria, as follows: (i) clinical findings includeing scaly brown macules and patches, with at least some appearing reticulated and papillomatous; (ii) involvement of the upper trunk, neck, or flexural areas; (iii) negative fungal staining of scales or no response to antifungal treatment; and (iv) excellent response to antibiotics.

The spectrum of histopathologic findings in cutaneous eruptions associated with influenza A (H1N1) infection

Influenza A (H1N1), like many other viral infections, has been associated with cutaneous eruptions. Differential diagnoses in a viral exanthem generally include spongiotic dermatitis, urticaria and drug reaction. The aim of this series was to retrospectively review three cases (five biopsies) involving patients with a clinical history of H1N1 and an accompanying rash, and to evaluate whether unique histopathologic and immunohistochemical features exist among these patients' cutaneous eruptions. Findings among all cases included a sparse superficial perivascular infiltrate, and interestingly, scattered interstitial and prominent intravascular neutrophils. Two cases demonstrated mild spongiosis and mild interface change. Immunohistochemistry in all cases revealed a CD4-predominant lymphocytic infiltrate of the dermis with a sparse intraepidermal population of admixed CD4 and CD8 positive lymphocytes. Many changes found in the cutaneous eruption associated with H1N1 are similar to those of other viral eruptions, including a mild perivascular lymphocytic infiltrate, mild spongiosis and mild interface change; however, sparse dermal and intravascular neutrophils and intraepidermal lymphocytes appear to be the features unique to these cases of H1N1-associated cutaneous eruptions. Such a distinction may prove diagnostically important in the clinical setting and useful in the surveillance of this historically pandemic virus.

Two cases of diabetic mastopathy: MR imaging and pathological correlation

Diabetic mastopathy is a rare benign condition associated with long-standing diabetes mellitus and presents with breast lumps. This report describes two cases in which diffusion-weighted images (DWI) on magnetic resonance imaging were quite different from each other. In case 1, there were hyperintense lesions on DWI, and surgically removed specimens revealed ductitis with marked lymphocytic infiltration. In case 2, no abnormal intensity was depicted on DWI, and biopsy specimens showed dense stromal fibrosis with mild perivascular lymphocytic infiltration that corresponded to previous reports. Although it is reported that diabetic mastopathy is composed of dense fibrous tissue with low cellularity that results in no hyperintense lesion on DWI, in cases with marked lymphocytic infiltration, strong hyperintensity can be seen on DWI mimicking malignant breast tumors.

Telangiectasia macularis multiplex acquisita: a new entity in Chinese populations and an analysis of associated factors

Telangiectasia macularis multiplex acquisita (TMMA) is a rarely documented entity with distinctive clinical manifestation. Most patients are middle-aged adults. It may be associated with other diseases such as hepatitis, diabetes, or cardiovascular diseases. To assess the clinical presentation of TMMA and its association to other systemic diseases, especially the correlation to liver disease, in Taiwan. We identified and retrospectively analyzed clinical criteria-matched and skin biopsy-proven patients from 2002 to 2010 at a single medical center. The clinical criteria for diagnosis of TMMA included: (i) crops of telangiectasia superimposed on erythematous macules symmetrically on bilateral upper arms, possibly extending to the forearms, shoulders, V-shaped area of the anterior chest, back or thighs; (ii) no mucosal or systemic involvement; (iii) not associated with autoimmune diseases, such as lupus erythematosus, dermatomyositis or systemic scleroderma that may induce cutaneous telangiectasia; and (iv) no ataxia or unsteady gait. Twenty-five patients were enrolled in this study. The male-to-female ratio was 19:6. The age of onset was variable, mostly between the third to sixth decades. The V-shaped area of the anterior chest was commonly involved (68%), in addition to the arms (100%). Viral hepatitis was diagnosed in 56.5% of the patients (13/23); 38.9% (7/18) and 50% (9/18) of the patients had diabetes and hypertension, respectively. In addition, 64.7% of the patients (11/17) had dyslipidemia. The pathological reports of most cases showed only mild perivascular lymphocytic infiltration with or without telangiectasia. TMMA should be assigned to a distinct entity with unique clinical manifestation. It has not been well recognized and mostly misdiagnosed as other diseases with telangiectasia. It is important to evaluate the complete liver function tests when TMMA is suspected.

Investigating the role of interferon-alpha in Sjögren’s Disease (101.36)

Abstract We have previously demonstrated that IL14a transgenic mice (IL14aTG) reproduce features of Sjögren’s disease (SD) seen in patients in the same relative time frame, including autoantibodies, early destruction of the salivary and lachrymal glands, infiltration of the salivary and lachrymal glands with lymphocytes, lymphocytic pneumonia, mild renal disease and eventually lymphoma (J.Immunol. 177: 5676, 2006 and Clin. Immunol. 130:304, 2009). We have more recently demonstrated that lymphotoxin is an important early mediator of salivary gland injury in IL14aTG mice and patients with SD (J.Immunol. 185: 6355, 2010). In our current studies we investigate the role of interferon-alpha (IFN-α) in SD in IL14aTG mice. We demonstrate that IL14aTG mice lacking the type I IFN receptor (IL14aTG.IFNa/bR-/-) have the same early injury to the submandibular glands as IL14aTG mice. However, IL14aTG.IFNa/bR-/- do not get the later parotid gland injury that is seen in IL14aTG mice. Both IL14aTG mice and IL14aTG.IFNa/bR-/- get lung injury, but IL14aTG mice get a lymphocytic pneumonia and IL14aTG.IFNa/bR-/- get a mild perivascular and peribronchial lymphocytic infiltration. Thus, IFN-α is not critical in early stages of SD but involved in disease manifestations that occur after original salivary gland injury. These data have important implications for the use of IFN-α inhibitors in the treatment of SD.

Skin involvement in Susac's syndrome

Susac's syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear, presumably resulting from an autoimmune endotheliopathy. We report the first case of SS with histologically proven skin involvement, in a 24-year-old male who presented a subacute encephalopathy, branch retinal artery occlusions and bilateral hearing loss, two weeks after the onset of a livedo racemosa of the flanks and feet. Skin biopsies revealed a thrombus in several dermal arterioles, endothelial cells swelling and a mild perivascular lymphocytic infiltrate, which correspond to the same histological findings as previously observed in brain but also in muscle biopsies of patients with SS. A complete recovery was achieved in 4 months with corticosteroids. Follow-up MRI showed centro-callosal “holes”. Skin involvement in SS has pathological plausibility since serum antibodies directly binding to central nervous system but also to generic endothelium cells have been reported. Our report supports that SS is a systemic disease that could affect other organs in addition to the brain, retina and inner ear. We suggest careful skin examination should be considered in patients with a suspicion of SS.

Lipedematous alopecia with mucinosis: report of the first case in Taiwan

Lipedematous alopecia is an uncommon disease that mainly affects African American and Egyptian women. This report is of an 18-year-old Taiwanese woman who presented with asymptomatic boggy and thickened scalp for 10 years. In the last 6 months, there was diffuse hair loss on the affected scalp without scarring. Histopathologically, there were increased thickness of subcutaneous fat layer, mild perivascular lymphocytic infiltration, and separated collagen bundles in the dermis. Alcian blue stain demonstrated mucin deposition in the dermis and subcutis, whereas magnetic resonance imaging showed thickened scalp with expanded subcutaneous fat layer. The clinical findings and imaging study established the diagnosis of lipedematous alopecia. The pathogenesis and disease etiology remain unclear. The coexistence of mucin is extremely rare and its significance should be further investigated.