Objective To analyze locations of acantholysis in pemphigus vulgaris (PV) and pemphigus foliaceus (PF),so as to explain why acantholysis in pemphigus occurs in different locations of the epidermis.Methods Clinical data,histopathological and immunopathological findings,and pemphigus antibody level values were collected from 43 patients with PV and 28 with PF,and retrospectively analyzed.Results Of the 43 patients with PV,35 showed acantholysis in the upper basal layer,8 in the middle-to-upper epidermis.Of the 28 patients with PF,25 showed acantholysis in the granular layer and the upper prickle cell layer,3 in the middle-to-lower epidermis.Patients with PF showed significantly higher levels of anti-Desmoglein 1 (Dsg1) antibody compared with patients with PV (P =0.047).However,there were no significant differences in the levels of anti-Dsg1 and anti-Dsg3 antibodies between PV patients who had acantholysis in the middle-to-upper epidermis and those in the upper basal layer.Conclusion Histopathological examinations of PV and PF lesions show that acantholysis can occur in the middle-to-upper epidermis,as well as in the middle-to-lower epidermis,and locations of acantholysis may be associated with levels of anti-Dsg1 and anti-Dsg3 antibodies.

A 51-year-old man presented with multiple, disseminated dark erythematous maculopapules and nodules over the body surface for more than 1 year. Initially, the patient presented with dark erythematous macules on the trunk without discomfort. Then, lesions gradually spread over the whole body surface with the development of tenderness. Physical examination revealed multiple disseminated dark erythematous, well-demarcated maculopapules, infiltrative plaques and subcutaneous nodules on the face, neck, trunk, upper and lower limbs. Some lesions were tender on palpation. An enlarged cherry-like lymph node was detected on the right inguina. Bone marrow inspiration showed that lymphocytes amounted to 32.5%, and naive lymphocytes accounted for 10%. These lymphocytes varied in size with irregular shape, moderate amount of basophilic cytoplasm, irregular nuclei and granular chromatin. Histopathological examination revealed diffuse infiltrate of numerous medium-sized atypical blastic cells with irregular nuclei in superficial dermis and subcutaneous fat tissue. The blastic cells showed sparse fine-granular chromatin, obscure nucleoli and obvious karyokinesis. Immunophenotype examination showed that tumor cells were strongly positive for CD4, CD56 and CD43, weakly positive for CD68 and terminal deoxynucleotidyl transferase, but negative for L26, CD3, CD38, granzyme B and myeloperoxidase. The diagnosis of BPDCN is confirmed based on typical clinical features, histopathology and immunohistology findings.

A case of ulerythema ophryogenes (UO) is reported.A 12-year-old boy presented with erythema and follicular papules on the eyebrows and cheeks for 7 years.The lesions started as follicular papules surrounded by erythema,then spread symmetrically to the cheeks and forehead followed by the loss of eyebrows.There was no complaint of pruritus.Physical examination showed pinhead- to grain-sized,smooth,slightly indurated follicular hyperkeratotic papules surrounded by erythematous halo on the eyebrows,forehead and cheeks.Both eyebrows were nearly completely lost.Histological analysis of lesions from eyebrows revealed dilated follicular infundibulum with orthokeratotic plugs,sparse perivascular and perifollicular lymphohistiocytic infiltrate,widened and sclerotic collagen fibers in the dermis.According to the clinical manifestations and histopathological findings,the patient was diagnosed with ulerythema ophryogenes,and given oral vitamin A 2.5 million unit once a day,vitamin E 100 mg once a day,topical vitamine E cream twice a day,0.025％tretinoin ointment once at night.Two weeks later,the lesions improved.

A 48-year-old man presented with a 4-day history of fever and 10-year history of papulovesicles on the face, neck, trunk and limbs which had been aggravated 10 days prior to the presentation.Skin biopsy showed a dermal infiltration of numerous small- to medium-sized atypical lymphocytes, which was mainly located around blood vessels or appendages, with the involvement of subcutaneous fat tissue and destruction of blood vessels. The infiltrating atypical cells stained positive for CD45RO, CD8, CD56, T-cell intracellular antigen-1, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs (EBER), but negative for CD20, CD79a, CD3, CD4 or CD30. Cytoplasmic CD3ε was also observed in these cells. Laboratory examinations on admission revealed a progressive decrease in peripheral erythrocytes, white cells and platelets, persistent increase in serum aminotransferase and bilirubin, and decline in serum fibrinogen and hypertriglyceridemia. The B-mode ultrasound of the abdomen showed hepatosplenomegaly. Based on the above findings,the diagnosis was made as extranodal nasal type NK/T-cell lymphoma of skin complicated by hemophagocytic syndrome.

OBJECTIVE：To investiga te the role of clinical pharmacists in drug therapy for patients with severe pemphigus. METHODS：Clinical pharmacists participated in drug therapy for a patient with severe pemphigus. Clinical pharmacist adjusted the doctor’s medication plan in time according to the symptoms and adverse reactions of the patients. In view of several new blisters on the hand palms and back that may be caused by hormone reduction ，the clinical pharmacist suggested that the dosage of Prednisone acetate tablets should be adjusted to 70 mg before administration ，once a day. For poor infection control ，it was recommended to adjust the dose of minocycline to 100 mg，bid；for hypokalemia ，the clinical pharmacist advised the patients to take potassium orally，and added it into juice ，milk or honey for taking ；in order to prevent osteoporosis caused by glucocorticoid ，the clinical pharmacist suggested that patients supplement Calcium carbonate and vitamin D 3 chewable tablets and Alendronate sodium and vitamin D 3 tablets 70 mg at the same time ，once a week. For Candida albicans in patient ’s oral mucosa ，according to the results of drug sensitivity test ，the clinical pharmacists suggested that Itraconazole capsules should be adjusted to Fluconazole capsules 50 mg，once a day. At the same time ，pharmacist paid close attention to the adverse reactions after the infusion of Rituximab injection and pharmaceutical care was provided ，such as ADR monitoring ，discharge medication education. RESULTS ：The doctors took all the advice of clinical pharmacists. The patient recovered and was discharged 47 days later. CONCLUSIONS ：During drug therapy for the patient with severe pemphigus. Clinical pharmacists can help doctors improve the treatment plan so as to ensure the effectiveness and safety of patients ’medication.