Introduction: IgA pemphigus is a rare, distinct variant of Pemphigus characterized by vesiculopustular eruptions mediated by IgA autoantibodies targeting keratinocyte cell surface antigens, desmocollins 1-3 and sometimes desmogleins 1 and 3. Its classical features have been described in literature but atypical cases have also been documented. This report presents such case posing a diagnostic dilemma. Case Report: A 35-year-old female presented with a 16-year history of intermittent eruptions of multiple hyperpigmented, annular and circinate, desquamating plaques and coalescing flaccid pustules on erythematous bases on the scalp, neck, trunk, and extremities. Histopathologic examination revealed subcorneal pustular dermatitis, and direct immunofluorescence was positive for granular intercellular IgG and IgA deposits in the epidermis. Antinuclear antibody test was negative and C3 level was normal. Antibody tests against desmogleins 1 and 3 were both negative. Topical potent corticosteroid therapy resulted in complete resolution of all lesions in three weeks. Conclusion Diagnostic dilemmas arise when laboratory results do not correlate with clinical findings. Findings of IgA autoantibodies in patients with pemphigus-like skin eruptions led to the diagnosis of subcorneal pustular dermatosis type of IgA pemphigus. Dapsone is the treatment of choice although topical potent corticosteroid alone may provide complete remission in some cases, avoiding the potential adverse effects of systemic therapy
Skin Diseases, Vesiculobullous

Introduction: Impetigo herpetiformis is a rare pustular disorder that affects pregnant women. It is also otherwise termed as “pustular psoriasis of pregnancy”, owing to the fact that the pustules are sterile and are not associated with a viral etiology. The classic lesions are erythematous patches or plaques with margins studded with subcorneal pustules spreading centrifugally. A cardinal feature of this disorder is the rapid resolution of lesions after delivery. Case Summary: This is a case report of a 33-year-old female, gravida 3, para 2 (2-0-0-2) at 36 weeks age of gestation who presented with one week history of multiple well defined irregularly shaped erythematous annular patches and plaques with marginal pustules on the trunk and extremities. The lesions began on the trunk spreading centrifugally, sparing the face, palms, soles and mucus membrane. Biopsy showed scale crust in the stratum corneum, the epidermis showed acanthosis, spongiosis and neutrophilic microabscesses with focal vacuolar alteration and small sub-epidermal nests on the basal cell layer. The dermis revealed dilated blood vessels and mild superficial perivascular lymphocytic infiltrates. Patient was given Prednisone and Cetirizine with noted resolution of lesions. Conclusion: Recurrences of impetigo herpetiformis in subsequent pregnancies are common with earlier onset in gestation and are characteristically more severe. The complications are placental insufficiency, stillbirth or neonatal death. Early detection is of utmost importance. Management must be multidisciplinary involving a dermatologist, obstetrician and pediatrician.
Pregnancy ; Female ; Impetigo ; Psoriasis ; Skin Diseases, Vesiculobullous ; Exanthema

Allergy and Immunology ; Ambulatory Care ; Autoimmune Diseases/therapy* ; COVID-19 ; Communicable Disease Control ; Connective Tissue Diseases/therapy* ; Delivery of Health Care/methods* ; Dermatology ; Humans ; Immunosuppressive Agents/therapeutic use* ; Patient Selection ; SARS-CoV-2 ; Singapore ; Skin Diseases, Vesiculobullous/therapy* ; Telemedicine/methods* ; Tertiary Care Centers ; Vasculitis/therapy*

Antibodies, Monoclonal, Humanized/therapeutic use* ; Humans ; Psoriasis/drug therapy* ; Skin Diseases, Vesiculobullous

Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the half-and-half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.
Adolescent ; Antibodies ; Biopsy ; Blister ; Colchicine ; Epidermis ; Female ; Fluorescent Antibody Technique, Direct ; Follow-Up Studies ; Humans ; Immunoglobulin A ; Immunoglobulins ; Neutrophils ; Pemphigus ; Skin Diseases ; Skin Diseases, Vesiculobullous ; Steroids

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson's disease, is a rare pustular eruption that occurs mainly in middle-aged women and rarely during childhood. Clinically, the pustules are distributed on the trunk and proximal region of the limbs, and cultures of the pustules consistently do not reveal bacterial growth. Histopathology shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. SPD is a rare condition, especially in children, and only one case has been reported in Korea. Here, we report a case of 4-year-old girl as an educational case for the diagnosis and treatment of pediatric SPD.
Child ; Child, Preschool ; Diagnosis ; Extremities ; Female ; Humans ; Korea ; Neutrophils ; Skin Diseases, Vesiculobullous

Introduction: Sneddon-Wilkinson disease (SWD) is a rare, recurrent neutrophilic dermatosis presenting as sterile pustules, with a predilection for flexural and intertriginous areas. Case summary: A 49-year-old Filipino female presented with a three-year history of recurrent pustules and papules on the flexural areas of trunk and extremities. Skin punch biopsy was done and histopathology was consistent with subcorneal pustular dermatosis/SWD. She was started on Dapsone but after two weeks of intake, the patient developed generalized erythematous desquamating plaques on the trunk and extremities, with palmoplantar involvement. The patient did not have fever, jaundice, lymphadenopathy, and abdominal tenderness. Laboratory investigation such as complete blood count and liver function tests were normal. The final diagnosis was SWD with hypersensitivity to Dapsone. Dapsone was immediately discontinued and she was shifted to oral colchicine. After six weeks of oral colchicine therapy, the lesions have completely resolved. Patient was in remission for six months thereafter. Conclusion SWD is rare and the drug of choice is dapsone. In instances where dapsone is not suitable, oral colchicine can be an ideal alternative treatment.
Skin Diseases, Vesiculobullous ; Dapsone ; Colchicine

BACKGROUND: Generalized pustular psoriasis (GPP) and subcorneal pustular dermatosis (SPD) are clinically and histopathologically difficult to distinguish. There have been no comparative studies examining these two diseases in Korea. OBJECTIVE: To investigate the clinical and histopathological characteristics of GPP and SPD. METHODS: We evaluated the clinical features, laboratory, and histopathological findings in 16 patients with generalized pustular eruption who had visited our hospital over the past 10 years and reviewed the literature. RESULTS: Ten GPP and six SPD patients were included in the study. The mean age at diagnosis was 44.4 years in the GPP group and 50 years in the SPD group. The number of patients with previous personal history of psoriasis vulgaris was 2 (20%) for GPP and 0 (0%) for SPD. The number of patients with history of recent exposure to medications was 1 (10%) and 0 (0%) in the GPP and SPD groups, respectively. Symptoms of fever, arthralgia, and mucosal involvement were reported in 10%, 20%, and 10% of GPP patients and 16.7%, 16.7%, and 0% of SPD patients, respectively. Leukocytosis, eosinophilia, elevated ESR/CRP, and elevated AST/ALT were reported in 25%, 0%, 25%, and 50% of GPP patients and in 20%, 0%, 40%, and 40% of SPD patients, respectively. On histological findings, in the GPP group, spongiosis, and psoriasiform changes including hyperkeratosis/parakeratosis, and rete ridge changes were more apparent than in the SPD group. The mean period of clinical improvement was 32.9 days with 40% recurrence in the GPP group and 38.3 days with 66.7% recurrence in the SPD group. CONCLUSION: Although GPP and SPD exhibit clinical and laboratory findings that are similar and difficult to differentiate, systematic analyses including clinical course, laboratory findings, and histopathological findings are helpful for an accurate differential diagnosis.
Arthralgia ; Diagnosis ; Diagnosis, Differential ; Eosinophilia ; Fever ; Humans ; Korea* ; Leukocytosis ; Psoriasis* ; Recurrence ; Skin Diseases, Vesiculobullous* ; Tertiary Care Centers*

Adverse reactions of subcutaneous low molecular weight heparin or unfractionated heparin could be complications by bleeding, heparin-induced thrombocytopenia, drug-induced liver injury, osteoporosis, and cutaneous reactions. Heparin-induced skin lesions vary from allergic reactions like erythema, urticaria, eczema to intradermal microvascular thrombosis associated with heparin-induced thrombocytopenia. There is a rare cutaneous complication, called bullous hemorrhagic dermatosis. We experienced this rare case of the cutaneous complication caused by enoxaparin. Several tense bullous hemorrhagic lesions occurred after 3 days of enoxaparin in a known bullous pemphigoid patient who had aortic valve replacement surgery with a mechanical prosthesis. The bullous hemorrhagic lesions were regressed after the discontinuation of enoxaparin but recurred after re-administration. The lesions were controlled by the administration of systemic corticosteroid and alternative anticoagulant. To date, less than 20 cases have been reported worldwide. This is the first case of bullous hemorrhagic dermatosis induced by enoxaparin, a low-molecular-weight heparin in Korea. This is also the first case of bullous hemorrhagic dermatosis in a known bullous pemphigoid patient.
Aortic Valve ; Drug-Induced Liver Injury ; Eczema ; Enoxaparin ; Erythema ; Hemorrhage ; Heparin ; Heparin, Low-Molecular-Weight ; Humans ; Hypersensitivity ; Korea ; Osteoporosis ; Pemphigoid, Bullous ; Prostheses and Implants ; Skin ; Skin Diseases ; Skin Diseases, Vesiculobullous ; Thrombocytopenia ; Thrombosis ; Transcutaneous Electric Nerve Stimulation ; Urticaria

Subcorneal pustular dermatosis is a pustular eruption that shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. It most commonly affects the trunk, inguinal region, and axilla of middle-aged women, and the pustules are distributed bilaterally. Cultures of the pustules consistently do not reveal bacterial growth. Subcorneal pustular dermatosis rarely happens, and no specific etiology and pathogenesis are known. Thus, we present this case to provide dermatologists with information on its adequate diagnosis and treatment and to inform them of the risk of subcorneal pustular dermatosis in kidney dialysis patients who use darbepoetin-α.
Axilla ; Diagnosis ; Dialysis ; Female ; Humans ; Kidney ; Neutrophils ; Renal Dialysis* ; Skin Diseases, Vesiculobullous*