Isabel Betlloch

Acute juvenile pityriasis rubra pilaris: a superantigen mediated disease?

Juvenile acute pityriasis rubra pilaris (JAPRP) is a form of pityriasis rubra pilaris (PRP) with particular clinical characteristics and course. It is usually preceded by an infectious condition. We report a boy, initially thought to have Kawasaki disease, and subsequently diagnosed as having JAPRP, who made a satisfactory recovery. In this case, prior antibiotic treatment made it impossible to show the presence of any microorganism. However, the clinical characteristics of these patients and the similarities with other disorders involving the skin that are mediated by superantigens led us to think that these antigens may be involved in the development of this disease. Also, in view of the obvious differences between JAPRP and the other PRP, we suggest that, in the future this disorder may be considered a separate entity as a reactive exanthem.

Eruptive melanocytic nevi in human immunodeficiency virus infection.

A 31-year-old man was admitted to the hospital because he sutiered from chronic diarrhea. He was using intravenous drugs until 5 years before admission, and he had been well until 4 months before admission when he developed weakness, weight loss, diarrhea, night sweats, and low grade fever. He had also realized the sudden appearance of multiple pigmented skin lesions during the last month. There was neither family nor personal history of dysplastic nevi. On physical examination he was found to have enlarged lymph nodes on laterocervical and axillary regions and multiple pigmented macular and papular lesions ranging from 2 to 10 mm over the trunk and extremities (Fig. 1). There were no other findings. Results of laboratory studies were remarkable for pancytopenia, and an enzyme-linked immunosorbent assay positive for HIV antibodies was subsequently confirmed by Western Blot method. The CD4 count was 400 and the CD4:CD8 ratio was 0.45. Stool examination disclosed cryptosporidium. A skin biopsy showed a compound nevus without melanocytic atypia. Therapy with Zidovudine and Spiramycin was started. The patient became asymptomatic and his cutaneous lesions remained unchanged. After 9 months of follow-up he has not developed any other opportunistic infection and so far, no new nevi have appeared.

MORPHEA‐LIKE REACTION FROM VITAMIN K1

A 70-year-old woman with ehronic liver disease was treated for hypoprothrombinemia with intramuscular injections of 10 mg oleous vitamin Ki (phytomenadione) administered on alternate days for several weeks. Approximately 10 days after the first dose, she developed a pruritic, erythematous eruption at the injection sites that became later asymptomatic with two infiltrating plaques persisting on the buttocks. Eight months after the initial reaction, she was examined in our outpatient clinic for evaluation of the eruption. Physical examination showed two indurated, bright, atrophic, ivory-violaceous areas, 4 x 2 cm and 3 x 2 cm maximum diameter, with no erythematous ring, on the upper and outer gluteal quadrants (Fig. 1). The results of the rest of the physical examination were unremarkable and clinical signs of systemic sclerosis were not found. Histopathologic examination of a skin biopsy showed a normal epidermis, sclerosis of the reticular dermis, and a lymphocytic perivascular and periadnexial infiltrate (Fig. 2). The diagnosis of a cutaneous morphea-like reaction to vitamin Ki was made and no treatment was administered because spontaneous cure can be expected in most cases.

Linear rheumatoid nodule.

A 42-year-old man, witbout antecedents of interest, was admitted to tbe hospital with migratory artbralgias lasting several years and Raynauds phenomenon. Occasional nodules appeared on the interphalangic joints, followed by spontaneous disappearance. Exploration revealed a nodule on tbe elbow, 3 cm in diameter. Tbe nodule was mobile and painless and showed no signs of inflammatory lesions. A linear lesion (Fig. 1) was also observed extending some 15 cm from tbe posterior edge of the axilla along the ribcage. The linear lesion was approximately 4 mm thick; location was subcutaneous, witb a rope-like consistency and absence of inflamation or atrophy. Tbe overlying skin was slightly pigmented. Tbe nodule was painless and did not affect arm mobility. The rest of exploration was negative. No abnormalities were found in tbe bemogram chemistries, urinalysis, HBsAg, Mantoux test, immunoelectrophoresis, ANAs, complement level, rheumatoid factor, ASLO, joint and chest radiography, and electrocardiogram. Biopsies were performed on tbe elbow and axillary band lesions. The histologic findings were similar in both cases and revealed deep dermal and subcutaneous cell tissue lesions under an intact epidermis. Tbe inflammatory infiltrate consisted predominantly of histiocytes and was arranged around a more eosinopbilic zone, consisting of homogeneous and fragmented collagen together with collagen fibrinoid degeneration (Fig. 2). The in-depth infiltration showed a granulomatous pattern. The superficial dermis presented mononuclear cells and isolated fibroblasts infiltrating the collagen bands.

Progression of Meyerson's naevus to Sutton's naevus.

Melanocytic naevi can suffer two types of perilesional inflammation, a depigmentation and a dermatitis [1, 2]. The former seems to be a form of cell-mediated, auto-immune reaction directed towards the destruction of the naevus and related to vitiligo. The dermatitis reaction is thought to be immune mediated [3, 4]. Meyerson’s phenomenon has not only been described with melanocytic naevi but also with other entities such as dermatofibroma. Although both processes involve the immune system, the mechanisms are different [5, 6]. Some authors consider these two as the opposite ends of the same immunological processes [3, 4]. On the other hand, other authors support the idea that they are different processes [5, 6] because the clinical, histological and evolutional data of both entities are very different [5]. The clinical findings which suggest that they are separate processes are that the eczematous halo does not show depigmentation and is pruriginous [1, 6]. The histology favours the latter hypothesis: the eczematous halo does not show the regression changes which are characteristic of the halo naevus [5]. Finally, the evolution of these two entities is often quite different, since in previously reported cases the eczematous halo did not evolve towards depigmentation and regression of the naevus [3–5]. A 28-year-old man developed itchy areas around the melanocytic naevi on both legs, of 1 month’s duration. Three months previously, he had experienced a similar episode that resolved spontaneously. Fig. 1. One of the naevi on the leg showing an eczematous halo. Fig. 2. Biopsy of one of the Meyerson’s naevi. The dermo-epidermal melanocytic naevus shows signs of regression in the papillary dermis. There is a lymphohistiocytic infiltrate, with numerous eosinophils in the upper dermis. Haematoxylin-eosin. Original magnification !200.

NEUTROPHILIC ECCRINE HIDRADENITIS IN AN HIV‐INFECTED PATIENT

A 30-year-old man who is an intravenous drug addict (not known to be Hiv-positive) was admitted to hospital complaining of fever and general malaise. The last dose of heroin had been administered 4 days previously and he had been taking dextropropoxyphenone since then. On admission, the leukocyte count was 9,200 per mL (neutrophils 8,100, lymphocytes 250), hemoglobin 12.4 g per dL, and the platelet count was 183,000 per mL. After a radiologic diagnosis of pneumonia was made, cloxacillin (1 g every 8 h iv) and gentamicin (80 mg every 8 h iv) were started, together with fluconazole (100 mg per day orally) for oropharyngeal candidiasis. Methadone, lorazepam, and flunitrazepam were given to prevent an opiate withdrawal syndrome. Seven days after admission, 12 erythematous maculae, of 5 mm diameter, were seen on the anterior thorax, together with pancytopenia (leukocyte count 700 per mL, neutrophils 600 per mL, lymphocytes 0 per mL, hemoglobin 10.9 g per dL, platelets 16,000 per mL) and persistent fever. Two days later, the patient died of acute respiratory insufficiency. Pneumocystis carinii and acid/alcohol-resistant bacilli were later confirmed by stain and culture, in the sputum, as was the positive HIV serology. A skin biopsy specimen showed a neutrophilic inflammatory infiltrate, together with necrosis of the epithelial cells of the eccrine coils (Fig. 1). The epidermis and the eccrine ducts in the dermis were intact. Neither mucin deposits nor squamous metaplasia of the eccrine ductal epithelium were seen. Special staining for bacteria, fungi, and acid-fast bacilli was negative.

Bronchogenic Cyst Presenting as a Nodular Lesion

Abstract: We report a subcutaneous bronchogenic cyst in a 7‐year‐old girl with unusual presentation as a nodular lesion. The cyst was excised and no connection with deeper structures was found. Histologic examination showed the typical features of a bronchogenic cyst. The characteristic clinical and histologic features of bronchogenic cysts are reviewed. The problems of differential diagnosis are also discussed.

Erythema Annular Centrifugum in Q Fever

• A 24-year-old man was admitted to the hospital because of fever, arthralgias, myalgias, and rash. He was well until 1 month earlier, when he developed fever, frontal headache, nausea, and vomiting that continued for 10 days. He improved spontaneously and was stable until 2 days before admission. He was living in a rural area and had had previous contact with horses. On physical examination, he was found to have multiple annular skin lesions on the forearms, hands, and legs. These lesions had a variable size of 5 to 8 cm, and showed an erythematous and palpable edge with a flattened and pale center. The rings enlarged slowly, and some of them showed a polycyclic pattern. There were no other remarkable findings. The Mantoux test was negative. Diagnostic laboratory and radiologic studies were unremarkable except for two slightly elevated liver function enzyme tests: GOT, GPT. Several serologic tests ruled out syphilis, brucellosis, Lyme disease, and HIV infection. A complement fixation test for antiphase 2 antigen of Coxiella burnetii showed a significant rise in titer, from negative to 1/256, between two separate samples. The Weil-Felix test was negative. A skin biopsy disclosed a perivascular lymphocytic infiltrate around the vessels ofthe upper and mid-cutis. The epidermis and the dermoepidermal interface were preserved. Direct immunofluorescence gave negative results. Therapy with doxycycline (200 mg per day) was started. After 1 week, the patient became afebrile, and cutaneous improvement was observed.

Livedo reticularis and thrombotic purpura related to the use of diphenhydramine associated with pyrithyldione.

We describe the case of a young woman who habitually took large doses of a combination of diphenhydramine and pyrithyldione. She complained of arthralgia, painful recurring plaques and nodules, together with persistent reticular purpuric mottling and areas of necrosis on her legs. There were positive antinuclear antibodies and rheumatoid factor and histologically massive thrombosis of the dermal vessels without signs of vasculitis. Other cases of similar adverse reactions attributed to this drug combination used as a hypnotic have been described.

TRICOADENOMA OF NIKOLOWSKI

The patient, aged 50 years, with no relevant clinical history, complained of a symptomless, soft tumor of the right buttock that had been present for many years. On examination, it was found to be pediculate, oval, somewhat erythematous, firm on palpation, and with a central keratin plug. The greatest diameter of the lesion was 1.2 cm (Fig. 1). On surgical removal of the lesion, it was seen to be composed of a symmetric intradermal proliferation of cystic formations, uniformly distributed throughout and made up of flat, pluristratified epithelium with laminated keratin. There was little difference in the thickness of the walls of the cysts (Fig. 2). Some cysts were attached to a short tadpole-shaped, epithelial cord and surrounded by a scanty fibroblastic stroma. No hairmatrix-like differentiation nor basaloid structures were seen, nor were atypical cells present. There was hardly any inflammatory infiltration.