Juvenile subcorneal pustular dermatosis: a case report

Subcorneal pustular dermatosis is a chronic, relapsing, pustular dermatosis that usually develops in elderly women. It is rarely seen in childhood and adolescence. The hallmark of the disease is formation of a subcorneal pustule detected on histopathologic examination. Herein we present a 13-year-old girl diagnosed with subcorneal pustular dermatosis based on the typical clinical and histologic findings. Direct and indirect immunofluorescence and serum protein electrophoresis had negative results. The patient partially recovered after 5 weeks of treatment consisting of oral dapsone and a topical steroid ointment.     

异维A酸治疗角层下脓疱病一例

临床资料 患者,女,28岁。因全身皱褶部位出现红斑、水疱、脓疱4周,加重7d,于2010年7月就诊于我科。患者4周前无明显诱因颈部、胸部出现红斑,很快发展至双侧上肢近端、腋窝、腹股沟及腹部,并在红斑基础上出现水疱,且水疱很快变为脓疱,伴轻度瘙瘁,脓疱破溃后有轻度疼痛。     

Intercellular IgA dermatosis with clinical features of subcorneal pustular dermatosis

In a patient whose clinical features resembled subcorneal pustular dermatosis, IgA deposits in the intercellular space of the upper epidermis were found on direct immunofluorescence study. Furthermore, by indirect immunofluorescence, IgA autoantibodies against the same area of the epidermis were demonstrated in the patient's serum.     

A case of subcorneal pustular dermatosis treated with tacalcitol (1alpha,24-dihydroxyvitamin D3)

We report a case of subcorneal pustular dermatosis (SPD) treated with tacalcitol (1alpha,24-Dihydroxyvitamin D3) ointment. A 77-year-old male had had asymptomatic, superficial, grouped pustules in annular patterns with mild background erythema for six years which affected his axillae and bilateral groin. A diagnosis of SPD was made. Topical vitamin D3 (tacalcitol) was applied to the lesion of SPD and its clinical effectiveness was assessed. Within one month, symptoms in the lesion treated with tacalcitol ointment started subsiding, and eventually only erythema remained. After three months, the region treated with tacalcitol ointment showed no relapse. We conclude that tacalcitol ointment is effective for SPD in some case.     

Pentoxyfilline as a treatment for subcorneal pustular dermatosis

Subcorneal pustular dermatosis (SPD) is a rare pustular neutrophilic dermatosis in which groups of sterile pustules appear in the superficial (subcorneal) skin. This chronic condition can be associated with significant morbidity and decreased quality of life. Dapsone is the first‐line therapy for SPD, but some patients fail to respond or cannot tolerate it. In these instances, patients may be treated with second‐line therapies such as phototherapy, topical corticosteroids, or systemic agents including glucocorticoids, acitretin, immunosuppressive, or biologic medications. These therapies may not always be efficacious and can be associated with intolerable adverse effects. Here, we report a case of a patient who sustained long‐term remission and no side effects with the novel use of pentoxifylline, a tumor necrosis factor‐alpha inhibitor, as monotherapy. Pentoxifylline should be considered as a possible therapy in patients with SPD intolerant to dapsone.     

Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma

We report a 57-year-old woman with a 12-year history of ulcerative pyoderma gangrenosum (PG). Five years after the onset of PG, she developed subcorneal pustular dermatosis (SPD) and biclonal IgA and IgG gammopathy. She developed PG at two bone-marrow biopsy sites, showing pathergy. Finally, she developed multiple myeloma. Although PG and SPD may occur without associated underlying malignancy, these patients should be followed up for any prospective malignancy because of the association between these disorders.     

Colchicine in the treatment of subcorneal pustular dermatosis

A male patient with subcorneal pustular dermatosis was initially treated with dapsone with excellent response. Since he developed hypersensitivity to dapsone, corticosteroid was given instead of dapsone. But steroid also was withdrawn since he developed hypertension and diabetes. There was relapse of skin lesions which were treated with oral colchicine, which has profound inhibitory effects on neutrophils. There was excellent therapeutic response to colchicine and the drug well-tolerated.     

IgA pemphigus of the subcorneal pustular dermatosis type. Successful therapy with a combination of dapsone and acitretin

IgA pemphigus of the subcorneal pustular dermatosis type is a rare autoimmune blistering disease in the pemphigus spectrum. Patients are clinically characterized by extensive erythemas that primarily affect intertriginous areas. The erythematous macules are covered with numerous vesicles and pustules with occasional hypopyon formation. Histopathology shows subcorneal acantholysis with clefting and numerous neutrophils within the blister as well as in the edematous papillary dermis. IgA autoantibodies bind in vivo to keratinocytes within the upper half of the epidermis. Desmocollin 1, the autoantigen of this disease, is a member of desmosomal cadherins and is only expressed on more differentiated keratinocytes. The demonstration of circulating autoantibodies against desmocollin 1 in routine diagnosis is challenging and requires indirect immunofluorescence staining of desmocollin 1 transfected COS7 cells. We report a patient with a severe course of the disease who only responded to combined therapy with dapsone and acitretin.     

IgA pemphigus: a case report and its characteristic clinical features compared with subcorneal pustular dermatosis

We report a case of IgA pemphigus. A 35-year-old woman had vesiculopustular eruptions on her scalp, trunk, and extremities. Histologic examination showed a subcorneal pustule containing numerous neutrophils without acantholysis. Direct immunofluorescence revealed IgA deposits in the intercellular space throughout the epidermis, more intense in superficial layers and less intense in lower layers. The titer of circulating IgA antibodies was 1:160 in normal human skin as a substrate. Skin lesions responded to dapsone. The IgA autoantibody from this patient did not react with desmogleins or desmocollins when immunoblotting, enzyme-linked immunosorbent assay, or complement DNA transfection was used. We consider the characteristic clinical features that favor the diagnosis of IgA pemphigus rather than classic subcorneal pustular dermatosis as follows: (1) the lesions involve the scalp and/or face, and (2) the distribution of the lesions is more widespread.