Introduction: Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune blistering disease which presents in the skin and mucous membranes. The decrease in anchoring fibrils in the basement membrane zone causes separation of the epidermis from the dermis, resulting in its blistering presentation. The treatment plan will depend on the severity of the disease. The first-line treatment for mild EBA includes topical corticosteroids and immunomodulators such as dapsone and colchicine; while severe cases of EBA may be given intravenous immunoglobulins, systemic steroids, and immunosuppressants such as azathioprine and cyclophosphamide. Case Report: This is a case of a 50-year-old Filipino male who presented with a 2-year history of vesicles and tense bullae which evolved into papules, plaques and erosions with scarring and milia formation on the scalp and trauma-prone areas of the trunk and extremities. Clinical examination revealed multiple, well-defined, irregularly shaped erythematous papules and plaques with crusts, scales, erosions, pearl-like milia and scarring on the chest, back, upper, and lower extremities. The oral mucosa was moist with some ulcers on the tongue. Histopathologic examination using Hematoxylin and Eosin (H&E) stain revealed the absence of the epidermis with retention of dermal papillae suggestive of subepidermal clefting. Further examination with direct immunofluorescence (DIF) revealed monoclonal immunoglobulin (IgG) deposits demonstrating an intense linear fluorescent band at the dermoepidermal junction, consistent with Epidermolysis Bullosa Acquisita. Overall, the combined administration of prednisone, azathioprine, and colchicine resulted only in transient and incomplete resolution of lesions in this case of EBA. Conclusion The management of EBA is mostly supportive with the goal of minimizing complications. Combination treatments using steroids, colchicine, and azathioprine have been reported with various results. Its management remains challenging as most cases are refractory to treatment.
Epidermolysis Bullosa Acquisita ; bullous disease ; azathioprine ; colchicine ; prednisone

We report herein a case of bullous pemphigoid(BP) associated with Graves' disease(GD) The patient was 43-year-old female who had suffered frorn GD for 8 years. She had annular, erythematous atrophic plaques with a margin of small vesicles on the frontal and temporal areas, Histopathologic findings of the skin lesion showed subepiderrnal bulla, and numerous eosinophilic infiltrate in the bulla cavity and dermis. Direct immunofluorescent study of perilesional skin revealed linear IgG deposition at the dermo epidermal junction. We think our case shows a higher than chance association of BP and GD because both are basecl on autoimmune mechanism.
Adult ; Dermis ; Eosinophils ; Female ; Graves Disease* ; Humans ; Immunoglobulin G ; Pemphigoid, Bullous* ; Skin

Onychatrophy is the processes in which the nail has initially formed satisfactorily and then shows total or partial regression. The causes of onychatrophy with pterygium include lichen planus, acrosclerosis, Stevens-Johnson syndrome, and cicatrical pemphigoid, and those without pterygium include severe paronychia, Stevens-Johnson syndrome, epidermolysis bullosa, and drugs. Bleomycin is an antitumor, antibacterial, and antiviral agent used in many dermatologic diseases such as warts, vascular anomalies, hemangioma, and cutaneous malignancies. Cutaneous adverse effects of bleomycin strongly depend on the route of administration and dosage. Bleomycin intralesional injection for periungual warts could result in permanent Raynaud phenomenon, nail dystrophy, and nail loss. We report five cases of onychatrophy following bleomycin intralesional injections for periungual warts. We remind that if bleomycin intralesional injection near the nail matrix is inevitable in recalcitrant periungual warts, the performer must manipulate precisely to avoid adverse effects.
Bleomycin ; Epidermolysis Bullosa ; Hemangioma ; Injections, Intralesional ; Lichen Planus ; Nails ; Paronychia ; Pemphigoid, Bullous ; Pterygium ; Raynaud Disease ; Stevens-Johnson Syndrome ; Warts

Diseases associated with immunoglobulin A (IgA) antibody include linear IgA dermatosis, IgA nephropathy, Celiac disease, Henoch-Schonlein purpura, etc. Although usually idiopathic, IgA antibody is occasionally induced by drugs (e.g., vancomycin, carbamazepine, ceftriaxone, and cyclosporine), malignancies, infections, and other causes. So far, only a few cases of IgA bullous dermatosis coexisting with IgA nephropathy have been reported. A 64-year-old female receiving intravenous ceftriaxone and metronidazole for liver abscess had purpuric macules and papules on her extremities. One week later, she had generalized edema and skin rash with bullae and was diagnosed with concurrent linear IgA dermatosis and IgA nephropathy. After steroid treatment, the skin lesion subsided within two weeks, and kidney function slowly returned to normal. As both diseases occurred after a common possible cause, we predict their pathogeneses are associated.
Carbamazepine ; Ceftriaxone ; Celiac Disease ; Drug Eruptions ; Edema ; Exanthema ; Extremities ; Female ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Kidney ; Linear IgA Bullous Dermatosis ; Liver Abscess ; Metronidazole ; Middle Aged ; Purpura, Schoenlein-Henoch ; Skin ; Skin Diseases* ; Vancomycin

Atypical manifestations of scabies have been reported, including impetigo, folliculitis, tinea, psoriasis, contact dermatitis, urticaria, dermatitis herpetiformis, bullous pemphigoid and Darier's disease. Although vesicles are commonly found in children, they are extremely uncommon in adults. We report a case of bullous scabies in 57-year-old male who developed a bullous pemphigoid-like eruption associated with scabies. Direct immunofluorescence test showed linear deposition of IgM and C3 at the dermoepidermal junction. Treatment with topical crotamiton was successful for up to 12 month of follow-up.
Adult ; Child ; Darier Disease ; Dermatitis Herpetiformis ; Dermatitis, Contact ; Fluorescent Antibody Technique, Direct ; Folliculitis ; Follow-Up Studies ; Humans ; Immunoglobulin M ; Impetigo ; Male ; Middle Aged ; Pemphigoid, Bullous ; Psoriasis ; Scabies* ; Tinea ; Urticaria

INTRODUCTIONAnti-BP180 IgG titres were observed to parallel disease activity in case series of bullous pemphigoid (BP). This study aimed to examine whether anti-BP180 titres are an indicator of disease severity, clinical course and outcome in Asian patients with BP.

MATERIALS AND METHODSThis was a prospective observational study conducted between March 2005 and March 2008 in the Immunodermatology Clinic at the National Skin Centre, Singapore. Disease activity and anti-BP180 IgG titres were measured 4-weekly for 12 weeks and during disease flares and clinical remission. Associations between anti-BP180 titres and disease activity, disease flare, clinical remission and cumulative prednisolone dose were examined.

RESULTSThirty-four patients with newly diagnosed BP were recruited. Median follow-up duration was 3 years. Notable correlations between disease activity and anti-BP180 titres were at baseline (r = 0.51, P = 0.002), and disease flare (r = 0.85, P <0.001). Lower titres at Week 12 were associated with greater likelihood of clinical remission (P = 0.036). Post hoc, patients with anti-BP180 titres above 87.5 U/mL at time of diagnosis who reached remission within 2 years of diagnosis received significantly higher cumulative doses (mg/kg) of prednisolone (median, 72.8; range, 56.5 to 127.1) than those with titres <87.5 U/mL (median, 44.6; range, 32.5 to 80.8); P = 0.025).

CONCLUSIONAnti-BP180 titres may be a useful indicator of disease activity at time of diagnosis and at disease flare. Lower titres at Week 12 may predict greater likelihood of clinical remission. Titres above 87.5 U/mL at time of diagnosis may suggest the need for higher cumulative doses of prednisolone to achieve remission within 2 years.

Adult ; Aged ; Aged, 80 and over ; Antibodies, Anti-Idiotypic ; blood ; Asian Continental Ancestry Group ; Autoantibodies ; blood ; Autoantigens ; blood ; Disease Progression ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Male ; Middle Aged ; Non-Fibrillar Collagens ; blood ; Outcome Assessment (Health Care) ; Pemphigoid, Bullous ; diagnosis ; ethnology ; immunology ; Predictive Value of Tests ; Prospective Studies ; Singapore

One hundred and four biopsy specimens of various dermatoses were stained with Periodic Acid-Schiff and examined. The lesions were grouped into five groups; A. twenty two cases of vesicob-ullous diseases; B. twenty one cases of maculopapulosquamous diseases; C. eighteen cases of chronic granulomatous infection; D. seventeen cases of precancerosis and benign and malignant neoplasms; and E. twenty six cases of miscellaneous skin diseases. Special attention was given to the morph-ological changes of the dermoepidermal junction and the basement membrane. The results were as follows. 1. Among 10 cases of erythema multiforme, partial fraying appeared in 4 cases, partial thicken-ing in 4 and hyperchromasia in 4. Marked convolution was noted in the thickened basement membrane. PAS-positive material was scattered in the edemateus papillary dermis in some cases. 2. All of 6 cases of pemphigus erythematosus and pemphigus vulgaris showed normal basement membrane. 3, In bullous pemphigoid, partial absence in the areas of bulla formation appeared in 2 cases am-ong 3 cases. PAS positive material was present within the bullae and in the upper dermis. 4. All of 3 cases of dermatitis herpetiformis showed partial absence or fraying, 5. Half of 10 cases of psoriasis showed normal basement membrane. Among the remainder, fraying and partial thickening appeared in 3 and 2 cases respectively. 6. Among 6 cases of lichen planus, almsot total absence appeared in 2 cases and fraying in 3 cases. PAS positive material appeared focally in the upper dermis in some cases. 7. Four of 5 cases of pityriasis rubra pilaris showed diffuse thickening and marked convolution ef the basement membrane. 8. Half of 4 cases of lupus vulgaris showed normal basement membrane. Fraying and partial thickening appeared in 2 and 2 cases respectively. 9. All but 1 among 5 cases of lepromatous leprosy showed poor staining and poor visualization of the basement membrane. 10. One case of tuberculoid leprosy showed partial fraying, partial thickening and hyperchromasia with marked convolution, among 5 cases. il. Among 4 cases of condyloma latum, only one showed partial fraying. 12. One of 3 cases of senile keratosis showed partial fraying of the basemement membrane. 13. One case of Bowens disease showed partial thickening and hyperchromasia among 3 cases. PAS-positive material was present focally in the upper dermis in some cases. 14. All of 6 cases of basal cell carcinoma, showed normal basement membrane. PAS-positive ma-terial were present focally in the uppermoet dermis in some vases. 15. All of 3 cases of squamous cell carcinoma showed total absence of the basement membrane. 16. All of the 2 cases of keratoacanthoma showed partial fraying. 17. Among 10 cases of chronic discoid lupus erythematosus, 9 cases showed partial thickening and hyperchromasia with marked convolution of the basement membrane. Two cases showed partial fraying in areas of marked liquefaction degeneration of the basal cells. PAS positive material appe-ared in the uppermost dermis in some cases. 18. All of 3 cases of fixed drug eruption, 2 cases of seborrheic keratosis and 6 cases of verrucae showed normal basement membrane. 19. One case of poikiloderma atrophicans vasculare showed partial fraying, among 2 cases. 20. All of 2 cases of chronic radiodermatitis showed diffuse thickening and hyperchramasia. The thickened basement membrane showed marked convolution focally.
Basement Membrane* ; Biopsy ; Bowen's Disease ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Dermatitis Herpetiformis ; Dermis ; Drug Eruptions ; Erythema Multiforme ; Keratoacanthoma ; Keratosis ; Keratosis, Seborrheic ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Lichen Planus ; Lupus Erythematosus, Discoid ; Lupus Vulgaris ; Membranes ; Pemphigoid, Bullous ; Pemphigus ; Pityriasis Rubra Pilaris ; Psoriasis ; Radiodermatitis ; Skin Diseases ; Warts