Kuniki Sakamoto

Cutaneous Bronchogenic Cyst

A 22-year-old man had a small asymptomatic papule on his left neck that had been present since early childhood. There was no history of trauma or infection to the affected site prior to onset. On close examination, a !-mm papule was present on his left neck (Fig. !). General physical examination revealed no other abnormalities. This skin lesion was excised under local anesthesia. At operation, small amounts of clear mucoid fluid were expressed from the cyst by lateral compression, however, there was no evidence of extension or connections to other structures or to deep tissues. The postoperative course was uneventful. Microscopic examination revealed a cyst lined by ciliated pseudostratified columnar epithelium intermingled with goblet cells in the upper-to-mid dermis (Fig. 2). The cytoplasm of these goblet cells stained positively with diastase resistant PAS and alcian blue (at pH 2.5) (Fig. 3). In the connective tissues surrounding the cyst, groups of seromucous glands were observed, but, smooth muscles, cartilages, and thyroid tissues could not be identified. Based on these histologic features, a diagnosis of cutaneous bronchogenic cyst of the neck was made.

Lichen planus pemphigoides‐like lesions induced by cinnarizine

A 72‐year‐old woman developed a lichen planus pemphigoides‐like eruption following the administration of cinnarizine. The eruption recurred on challenge with the drug. Direct immunofluorescence studies of the lesions demonstrated deposition of IgG, IgM and C3 on colloid bodies and fibrin at the epidermal basement membrane zone. Circulating IgG antibasement membrane zone antibodies were detected at high titres, with no complement‐ fixing activities. To our knowledge, this is the first report of immunologically defined lichen planus pemphigoides induced by a drug.

Anaphylactoid purpura and familial IgA nephropathy

I gA nephropathy has become recognized worldwide as a fairly common form of glomerulonephritis. The diagnosis is established by the predominance of IgA in the mesangial immune-complex deposits in the glomeruli, usually accompanied by C3 and sometimes b IgG and IgM. Since the original description in 1968 [l , 3 it has been generally assumed that IgA nephropathy develops randomly in persons. However, increased, awareness of instances of biopsy-proven IgA nephropathy in two or more members of the same family [Z-4] has strengthened the hypothesis that a hereditary factor is important in some patients. A significant development within the past decade has been the immunologic studies of these cases and the discovery of similar changes in patients with anaphylactoid purpura; namely, the presence of IgA in the mesangium of renal biopsy specimens [5-71. This similarity prompted Baart de la Faille-Kuyper et al [B] and Meadow and Scott [9] to suggest that isolated primary IgA nephropathy may be a monosymptomatic form of anaphylactoid purpura. The difference between these two entities is primarily based on the vascular involvement of the skin, joints, and gastrointestinal tract present only in, patients with anaphylactoid purpura. An argument for a relationship between the two diseases is their occasional, although rare, occurrence in the same family [g-11]. We herein report on a family with IgA nephropathy, one member of which had recurrent purpuric rashes accompanied by severe involvement of the kidneys and eventual death ,from cerebral hemorrhage after development of renal failure. This is the first description of anaphylactoid purpura associated with familial IgA nephropathy in an adult.

Systemic sclerosis-like lesions during long-term penicillamine therapy for Wilson's disease

Systemic sclerosis‐like lesions developed in a 14‐year‐old boy with Wilsons disease who had been treated with D‐penicillamine for 11 years. Clinical and laboratory manifestations included proximal scleroderma, pulmonary restrictive defects, positive antinuclear antibodies, and the deposition of C3 at the dermal‐epidermal junction of the lesional skin. This is the first case reported in which long‐term administration of penicillamine was followed by the development of systemic sclerosis‐like lesions.

Pemphigus Foliaceus Associated with Acanthosis Nigricans-like Lesions and Hepatocellular Carcinoma

A 54-year-old man was referred to our department for evaluation of crusting and blistering skin lesions. Five months previously the skin lesions had begun with mild pruritic, erythematous, and eroded eruption in the axillae, subsequently spreading to the scalp, face, trunk, and extremities. Three months later, these lesions developed into hyperpigmented and hyperkeratotic plaques. Physical examination revealed small flaccid bullae and scaly, crusted, hyperpigmented, verrucous skin lesions varying in size from a few millimeters to a few centimeters on the scalp, face, trunk, and extremities. These skin lesions were prominent in the axillae and groin (Fig. 1). The Nikolsky sign was positive; however, there was no involvement in the mucous membranes. The results of the following laboratory studies were negative or within normal limits: complete blood cell count, urinalysis, total serum proteins, serum electrolytes, serum protein electrophoresis, serum immunoglobulin and complement levels, LE test, DNA test, a-fetoprotein and HBs Ag. Chest x-ray and ECG were normal. Liver function tests disclosed chronic hepatitis. A biopsy specimen obtained from an erythematous, vesicular lesion showed superficial acantholysis with cleft formation at or near the granular layer (Fig. 2). A biopsy specimen obtained from a verrucous lesion on the right axilla showed basket-weave hyperkeratosis, parakeratosis, papillomatosis, and slight acanthosis (Fig. 3). A moderate mononuclear cell infiltrate was present in the papillary and upper reticular dermis. These histologic features resembled those of acanthosis nigricans. Direct immunofluorescence microscopy of an erythematous vesicular lesion showed intercellular depositions of IgG and C3 throughout the epidermis; however, a verrucous lesion showed intercellular deposition of C3. Indirect immunofluorescence studies of the serum using normal human skin as substrates demonstrated circulating intercellular antibodies at a titer of 1:20. The diagnosis of pemphigus foliaceus was confirmed. Treatment was initiated with 5.0 mg of oral betamethasone per day. This produced suppression of new lesions, and apparent clinical remission was observed after 1 week.

Antigen specificities of antibasement membrane zone antibodies: immunofluorescence and Western immunoblotting studies.

SummaryTo clarify the antigen specificities of autoantibodies in sera and blister fluids from patients diagnosed as bullous pemphigoid (BP) by routine histology and immunofluorescence (IF) methods, indirect IF studies using the salt split-skin technique were performed. In addition, to detect the BP antigen(s) in human epidermal extracts, Western immunoblotting analyses were carried out. Of 41 sera, 39 (95%) showed a linear pattern of fluorescence along the epidermal side of the separation. Two (5%) sera showed a linear pattern of fluorescence along the dermal side. Blister fluids produced IF staining patterns identical with those of serum samples. These fluorescence patterns were not related to the BP antigen expression of the skin used as substrates. In Western immunoblotting analyses, selected sera showing an epidermal pattern on separated skin primarily reacted with 240 kD, 220 kD, 180 kD, and 150 kD proteins extracted from normal human epidermis. Two sera showing a dermal pattern on separated skin revealed no specific bands. The protein bands recognized by blister fluids were indentical with those of serum samples. These results indicated that blister fluids are also available in immunological analysis, and that BP antibodies have more than one antigenic specificity. Moreover, it is suggested that differential diagnosis between BP and other bullous diseases may be more important than previously recognized, particularly in patients with epidermolysis bullosa acquisita (EBA).

Annular Elastolytic Giant Cell Granuloma: An Unusual Case with Lesions Arising in Non-sun-exposed Areas

A 70‐year‐old man with a 2‐year history of annular elastolytic giant cell granuloma associated with diabetes mellitus was reported. The lesions mainly developed in non‐sun‐exposed areas. Histologic examination revealed phagocytosis of elastic fibers by histiocytic cells. Immunoperoxidase staining for lysozyme disclosed positive reactivity within the cytoplasm of these histiocytic cells. Electron microscopic study also showed elastic fibers and numerous lipid‐like substances in the cytoplasm of these cells. These findings indicate high phagocytolytic activity by these infiltrating cells. In our case, actinic damage was not considered to be a primary causative factor, and a possible pathogenesis was also discussed.

KID Syndrome : congenital ichthyosiform dermatosis with keratitis and deafness : report of the first case in Japan

A 2‐year‐old male with KID syndrome was presented. Family history was negative for similar skin disease. The patient showed generalized ichthyosiform erythroderma, vascularizing keratitis, and neurosensory deafness. Additional clinical features included nail dystrophy, hypotrichosis, anhidrosis, and recurrent cutaneous bacterial and fungal infections. Histologic examination disclosed a basket‐weave pattern of hyperkeratosis and irregular papillomatous configuration of the epidermis. Electron microscopic examination showed an excessive amount of glycogen in the cytoplasm of erector pili muscle cells. This is the first case reported in Japan.

SKIN LESIONS ASSOCIATED WITH SJÖGREN'S SYNDROME AND ANTICYTOPLASMIC ANTIBODIES IN SLE PATIENTS

Among forty‐two systemic lupus erythematosus patients, three had antibodies against cytoplasmic antigens SSA and SSB. All three patients showed annular nonscarring erythema as a sign of their illness. The most predominant site of skin lesions was the face, but trunk and extremities were involved as well. All of the patients had clinically distinct Sjögrens syndrome following the onset of their skin lesions.

Synthesis of epidermolysis bullosa acquisita antigen by simian virus 40-transformed human keratinocytes

SummaryThe synthesis of epidermolysis bullosa acquisita (EBA) antigen in simian virus 40 (SV40)-transformed human epidermal keratinocytes was studied. Indirect immunofluorescent staining of SV40-transformed keratinocytes employing a serum sample from an EBA patient as a source of antibodies decorated EBA antigen as a perinuclear granular fluorescence. This staining pattern was similar to that of nontransformed epidermal keratinocytes grown in a low Ca2+ medium. In contrast, stratified primary cultures of keratinocytes stained after growth in a high Ca2+ medium showed only small amounts of the antigen localized in the substrate-attached basal cells. To demonstrate biosynthesis of the EBA antigen by SB40-transformed keratinocytes, cells were metabolically labeled with 14C-amino acids and the cell lysates were immunoprecipitated with EBA antiserum. Analysis of immunoprecipitates by sodium dodecylsulfate-polyacrylamide gel electrophoresis and fluorography revealed that the EBA serum precipitated a protein with an apparent molecular weight of 290 kD from extracts of these cells. These results indicate that SV40 induces the synthesis of the 290 kD EBA antigen. Expression of this antigen may be a general feature of nonstratifying, proliferating epidermal cells.