Cutaneous cytomegalovirus infection in a liver transplant patient. Diagnosis by in situ DNA hybridization

A 39-year-old woman who had cryptogenic cirrhosis and who had received two liver transplants developed necrotic skin lesions over the chest, upper arms, and thighs. Biopsy showed enlarged endothelial cells with intranuclear and intracytoplasmic inclusions characteristic of cytomegalovirus (CMV) infection. However, a few multinucleated gaint cells were observed beneath a necrotic epidermis. This, combined with the clinical presentation, suggested to infectious disease consultants an infection with herpes simplex virus. Using the immunoperoxidase technique, inclusions stained positively with antibody to CMV and showed a negative reaction for herpes simplex antigen. In situ hybridization using biotinylated DNA probes on formalin-fixed, paraffin-embedded tissue sections confirmed the diagnosis of CMV infection and failed to substantiate infection with herpes simplex virus. Subsequently, blood cultures became positive for CMV. The early recognition of CMV infection in the skin permitted institution of antiviral therapy with gancyclovir. Specific skin lesions of CMV infection are likely to be encountered with increasing frequency among immunosuppressed patients. Lesions may be vesicular, and epidermal multinucleated giant cells can occasionally be identified. In situ hybridization is a technique that is readily adaptable to surgical pathology laboratories and permits both a rapid, specific diagnosis and the early institution of appropriate therapy.     

Recurrent follicular and lichenoid papules of sarcoidosis

We describe a 62-year-old Japanese woman who exhibited recurrent follicular lichenoid lesions of sarcoidosis. The skin lesions appeared all over the surface of the body without any other organ involvement, and spontaneously regressed within 1 year. Two years later, the lesions recurred on the trunk, especially in the intertriginous areas such as the submammary areas and in fatty folds of the abdomen, and on the thighs and upper arms, with the appearance of uveitis of the right eye. Histological examinations showed that small epithelioid cell granulomas were localized in the perifollicular areas. The present case was characterized by a unique distribution of follicular lichenoid eruptions, which are rare manifestations of sarcoidosis and recurrence of the lesions.     

Familial Occurrence of Eruptive Vellus Hair Cysts

A 15-year-old white male had an extensive skin eruption that had been present since age 2 years. Physical examination revealed flesh-colored, rough-textured papules surrounded by faint erythema. These lesions were present diffusely over the upper trunk, arms, thighs, face, ears, and neck. Evaluation of four immediate family members revealed a similarly affected brother and father. A biopsy of the papules showed the presence of a mid-dermal epithelial cyst containing numerous vellus hairs.     

Anetodermie bei positiver Borrelienserologie

Anetoderma is an uncommon disease characterized by multiple circumscribed atrophic, herniated skin lesions on trunk, thighs and upper arms caused by loss of elastic fibers. Associations with autoimmune diseases or infections, especially spirochetal infections, have been described. We report a case of anetoderma with an increased serum Borrelia burgdorferi IgM-titers. After treatment with doxycycline 200 mg/day for three weeks, the progression of the disease stopped and no new lesions appeared.     

Sofa dermatitis

Furniture components can cause contact allergies. In the last years several cases of eczema after sofa contact have been reported. Typically the skin lesions develop on the back, the buttocks, the dorsal aspects of the thighs and arms and are often very resistant to topical corticoid therapy. Dimethylfumarate (DMF) is postulated to be the causative agent for this Type IV hypersensitivity reaction. DMF is an antimicrobial substance, which is used in asian upholstered furniture industry amongst others. We report the case of a 65‐year old patient with generalised severely itching maculopapular, partly eczematous skin lesions on the buttocks, back, abdomen and arms. The resistance to therapy, several relapses after discharge from hospital as well as the detailed history lead us to the tentative diagnosis. The sofa dermatitis was proven by positive patch testing with furniture material and dimethylfumarate.     

Faint erythema. Another manifestation of cutaneous sarcoidosis?

A 44-year-old man was seen in the outpatient dermatology clinic with multiple erythematous lesions of 8–12 years' duration. He also had a 23-year history of chest X-ray findings of bilateral hilar adenopathy, and had experienced nasal stuffiness for several years. He had no history of dyspnea, chest pain, cough, fatigue, weight loss, or fever. The patient had also suffered from swelling of the fingers and toes, with associated stiff movement of their joints for several years. He had a past history of diabetes mellitus and hypertension. There was no palpable lymphadenopathy or hepatosplenomegaly. A nasal examination disclosed marked thickening of the mucosa, but a biopsy from the inferior turbinates showed no granulomas. An ophthalmologic evaluation revealed neither vitreous opacity nor uveitis. Pulmonary, cardiac, and neurologic evaluations yielded normal results. A chest roentgenogram revealed a bilateral hilar adenopathy and reticulated infiltrates (Fig. 1). A radiograph of the hands and feet disclosed well-defined phalangeal “punched-out” areas of transradiancy. Gallium scintiscans showed an increased uptake in the nose and the hilar and mediastinal nodes. Laboratory data included normal complete blood cell count and liver and renal function tests. The levels of serum calcium and phosphate were normal. The serum immunoglobulin G (IgG) revealed an increased level of 2370 mg/dL (normal range, 800-1800 mg/dL). The serum angiotensin converting enzyme disclosed an elevated value of 32.7 IU/L (normal range, 7–24 IU/L). The serum lysozyme was also increased to a level of 25.5 mg/L (normal range, 4.2–11.5 mg/L). The erythrocyte sedimentation rate was 3 mm/h. An intradermal skin test to purified protein derivative (PPD) was negative. A Kveim test was not performed. On physical examination, there were widespread multiple erythematous infiltrations at various stages of evolution. The patient had not noticed any prominent change in manifestation of the erythematous lesions over time. Chilblain-like dusky erythematous lesions had affected the acral regions, including the nose, ears, fingers, and toes, for 8 years. The lesions were purple-red or purple with a shining surface, swollen, and moderately well demarcated. The nasal skin also presented a papillomatous appearance (Fig. 2). There were multiple oval or round erythematous plaques of various sizes of 12 years' duration, with moderately well-defined edges, scattered on the back, buttocks, arms, and thighs. They were red or purplish-red, with fine superficial scaling, and were plainly raised above the surrounding normal skin (Fig. 3). In addition to the nodular plaques of this sort, another form of erythema had been present on the trunk for 10 years. The patient stated that this infiltrate had showed no apparent change. The erythematous infiltrate was rather sparse, and the major part presented a faint appearance. There was partial adhesion among the infiltrates. The lesion was soft, flat, pink or red in hue, and had an irregular and ill-demarcated margin. No raised infiltrated rim was seen at the periphery. Scaly desquamation was minimal. Apparent telangiectasia was not observed. The erythematous lesion occupied a large part of the abdomen and lateral sides of the chest (Figs 4 and 5). Skin biopsy specimens from the nasal, toe and abdominal lesions disclosed similar microscopic changes, with islands of granulomatous inflammation. They were composed of numerous epithelioid cells intermingled with Langhans giant cells and chronic inflammatory cell infiltrates. The epithelioid cell granuloma lacked caseation necrosis (Fig. 6). The cumulative findings were thought to be consistent with a diagnosis of sarcoidosis     

Disseminated cutaneous phaeohyphomycosis in immunocompetent child

We present a 6-year-old immunocompetent child with disseminated cutaneous phaeohyphomycosis, affecting his face, arms, thighs, and chest. He was treated with oral itraconazole 100 mg once a day for 5 months and surgical excision of the residual lesions. Disseminated phaeohyphomycosis is very infrequent in immunocompetent children.     

Pruritic skin lesions. A common initial presentation of acquired immunodeficiency syndrome

During July 1983 to December 1984, we observed that 62 (46%) of 134 Haitian patients with acquired immunodeficiency syndrome had intensely pruritic eruptions for which neither specific causative nor categoric diagnoses could be established. These lesions were a presenting manifestation of acquired immunodeficiency syndrome in 79% of the patients and appeared a mean of 8 months before the diagnosis of either Kaposi's sarcoma or opportunistic infection. Lesions included erythematous round macules, papules, or nodules that first appeared on the extensor surface of the arms, but subsequently involved the legs, trunk, and face. Histologically, the lesions were characterized by varying degrees of mixed (predominantly eosinophilic) perivascular and perifollicular inflammatory cell infiltrates of the dermis. The lesions did not respond to any therapeutic regimens used and usually persisted throughout the acquired immunodeficiency syndrome illness. Demographic and laboratory data did not distinguish these patients from those without pruritic skin lesions.     

A case of Wong‐type dermatomyositis treated with rituximab and IVIG

A 37‐year‐old woman presented to our rheumatology–dermatology clinic with a rash, muscle weakness and fatigue. She has had prior diagnoses of cutaneous lupus and lichen planus based on skin biopsies. She did not respond to topical steroids, hydroxychloroquine and dapsone. Clinically, she had sharply demarcated photo‐distributed erythema over the upper back, chest and upper arms, along with hyperkeratotic follicular papules on bilateral upper arms, shoulders, posterior neck, behind the ears, chest including breasts, abdomen and right buttock. Investigations revealed a high titre ANA, elevated creatinine kinase, aldolase and positive anti‐MJ/nuclear matrix protein 2 (NXP‐2). A skin biopsy showed findings of connective tissue disease. The diagnosis of Wong‐type dermatomyositis was made. She responded to therapy with mycophenolate mofetil, rituximab and IVIG.     

Keratosis lichenoides chronica in an Indian child following erythroderma

A 4-year-old boy presented with mildly itchy, linear, skin lesions over the trunk, arms, and face of 3 months' duration. He had previously been admitted to a private hospital for generalized exfoliation of the skin following drug intake for fever and throat pain. The nature of the drugs was not known. The exfoliative dermatitis was treated with oral prednisolone, 10 mg daily, tapered over 3 weeks. No further topical or oral medication was given. The present skin lesions started 1 month after the cessation of the steroids. There was no family history of skin lesions, voice changes, or systemic complaints. Cutaneous examination showed multiple violaceous, linear, reticulate ridges with adherent scaling over the chest, back, and neck. There were scaly, flat-topped papules over the extensor aspects of both upper arms and the buttocks, and scaly plaques over the cheeks (Figs 1a-d and 2a,b). The scalp showed diffuse greasy scaling. There were no oral, genital, axillary, or eye lesions. The nails were normal. Systemic examination did not reveal any abnormal finding. Routine hematologic investigations, liver and kidney function tests, tests for hepatitis B and C, and enzyme-linked immunosorbent assay (ELISA) for HIV were normal. Histopathology from skin lesions on the back revealed hyperkeratosis, patchy parakeratosis, follicular plugging, alternating irregular acanthosis and epidermal thinning, basal cell degeneration, and a band-like inflammatory infiltrate of lymphocytes, histiocytes, and a few plasma cells (Fig. 3). Based on the classical clinical features and histopathology, keratosis lichenoides chronica was diagnosed, and topical 1% hydrocortisone acetate cream, twice daily, was prescribed. There was slight relief of pruritus at a follow-up visit after 3 weeks; however, the patient was subsequently lost to follow-up.     

The naevus count on the arms as a predictor of the number of melanocytic naevi on the whole body

A study of melanocytic naevi was carried out in southern Spain to examine the relationship between numbers of naevi at different body sites as predictors of whole-body naevus count and to determine whether the naevus count on the arms is valid for identifying the risk factors for total naevi. Subjects were the control group from a case-control study on risk factors for cutaneous melanoma. They were selected from visitors to the University of Granada Hospital (southern Spain) between 1989 and 1993. Of 200 people invited to participate, 146 accepted (73%). Data were collected by personal interview, and melanocytic naevi were counted over the entire body surface by clinical skin examination performed by one dermatologist. Partial correlation coefficients (R) estimated by multiple linear regression were calculated. Comparisons between whole-body naevi and naevi on the arms, and their relationship with risk factors, were assessed by analysis of variance and covariance. Arms in men (adjusted R = 0.88) and thighs in women (adjusted R = 0.82) were the best predictors of total naevi after adjusting for age and sun exposure. Age, occupational and leisure sun exposure, and sunburns showed significant correlations with the total number of naevi. Similar results were found for the naevus count on the arms. In conclusion, the prediction of whole-body numbers of naevi by a naevus count on specific sites differs between men and women: arms in men and thighs in women are the best predictors. Nevertheless, naevus counts on the arms allowed us to study the risk factors for total naevi as well as whole-body naevus count: age and occupational sun exposure were the strongest determinants.     

Papulonecrotic tuberculid on the scalp

A 39‐year‐old woman was referred with a 1‐year history of a 4‐cm mass of the left axilla, compatible with a lymph node. Itchy red papules that evolved to crusty lesions and subsequently showed varioliform atrophic scarring appeared over the face, extensor surface of the arms, back, thighs, and scalp ( Figs 1 and 2 ). Her history included weight loss of 8 kg, but no other associated symptoms or contact with tuberculosis patients. The blood count showed a normochromic–normocytic anemia with an erythrocyte sedimentation rate (ESR) slightly elevated at 28 mm in the first hour (normal, < 20 mm). Tuberculin skin test was positive (13 mm with necrotic reaction) and anti‐human immunodeficiency virus serology was negative. Four lesional skin biopsies and one lymph node biopsy were performed and sent for histopathology and culture. The skin biopsy showed small‐vessel lumen occlusion by tiny thrombi, endothelial hyperplasia, and intense perivascular inflammatory reaction throughout the dermis, together with partial degeneration of collagen, compatible with tuberculid. Lymph node biopsy showed chronic, necrotizing, granulomatous lymphadenitis, consistent with a diagnosis of lymph node tuberculosis. Mycobacteria were absent on Ziehl–Neelsen staining and culture in all specimens.

Figure 1 Open in figure viewer PowerPoint Varioliform atrophic scarring over the extensor surface of the arms, typical of papulonecrotic tuberculid (PNT)     

Reactive angioendotheliomatosis in the setting of antiphospholipid syndrome

A 31-year-old man with systemic lupus erythematosus and antiphospholipid syndrome developed erythematous purpuric plaques distributed over the lower chest, abdomen and upper thighs. Biopsy of lesional skin revealed intravascular proliferation of endothelial cells with associated microthrombi formation. The histological pattern was consistent with reactive angioendotheliomatosis, a rare reactive pattern seen associated with disparate medical conditions. The pathogenesis of the reactive angioendotheliomatosis in our patient was suspected to be related to his procoagulant state; thrombi formed despite a therapeutic international normalized ratio while on warfarin. His lesions began to resolve with the cessation of warfarin and commencement of subcutaneous enoxaparin, oral clopidogrel and oral aspirin. The skin biopsy findings were pivotal in influencing the change of therapy in this patient and decreasing his immunosuppression.     

Pustular reactions to hexafluorosilicate in foam rubber

A case is reported of a foam rubber carrier with pustular lesions on the arms, wrists, thighs and trunk. Scratch and patch testing with the foam rubber components was negative. Animal testing revealed sodium hexafluorosilicate (Na2SiF6), one of the ingredients of the foam rubber, to be a pustulogen on previously damaged skin.     

Buschke-Ollendorff syndrome

Background Buschke–Ollendorff syndrome is a rare autosomal dominant disease featuring osteopoikilosis and skin lesions. It is caused by genetic mutations in a protein deeply involved in bone and connective tissue morphogenesis. Methods We describe a 39‐year‐old woman with Buschke–Ollendorff syndrome. Results After a minor trauma, radiologic examination of the left ankle of a 39‐year‐old woman revealed features of osteopoikilosis. Physical examination of the patient showed multiple asymptomatic nodules on both thighs, present since the age of 20 years, which had increased in size and number. Recently, a linear, string‐like lesion had appeared on the right thigh. Conclusion The correct diagnosis of Buschke–Ollendorff syndrome may require a high index of suspicion.     

Terra firma-forme dermatosis in a child

We present a case of reticulate hyperpigmented patches symmetrically distributed on the arms of a 13- year-old boy that appeared after a summer seaside vacation. The lesions were easily wiped off with isopropyl alcohol, confirming the diagnosis of terra firma-forme dermatosis. Dermatologists should be aware of this relatively common skin condition.     

Granuloma multiforme

CASE 1: A 51-year-old woman presented with skin-colored annular lesions on the upper chest, neck, and dorsa of the hands of 1-year duration. The lesions initially started as small papular lesions, which gradually evolved to form large annular and polycyclic lesions. Initially, the lesions were associated with a burning sensation and pruritus. She had no other systemic complaints. Examination revealed 5-6 annular, polycyclic lesions distributed over the upper chest, neck, and dorsa of both hands, varying in size from 1 to 5 cm. All the lesions had a prominent erythematous to skin-colored, irregular, papular border which was firm in consistency (Figs 1 and 2). Central clearing and minimal atrophy were evident in larger lesions. There was no sensory loss or peripheral nerve thickening. Clinical possibilities entertained were granuloma annulare, granuloma multiforme, and annular sarcoid. Routine investigations, including hemogram, renal and liver functions, blood sugar levels, chest X-ray, and urine examination, were within normal limits. A skin biopsy taken from the edge of a lesion revealed foci of collagen degeneration surrounded by an inflammatory infiltrate composed of many histiocytes and multinucleated giant cells. In addition, there were perivascular and periadnexal lymphocytic aggregates. No acid-fast bacilli (AFB) were detected. These findings were consistent with a diagnosis of granuloma multiforme (Fig. 3). CASE 2: A 47-year-old man presented with annular skin-colored lesions associated with a mild burning sensation of 8 months' duration. On examination, 2-3 annular, arciform lesions were distributed over the upper chest and neck. The lesions ranged in size from 2 to 7 cm in diameter, were irregular in shape, and were rimmed by a well-defined raised papular border. Again, there was no sensory loss or peripheral nerve thickening. Histopathology of the skin from the edge of the lesion showed multiple areas of histiocytic granulomas with focal necrobiosis and prominent multinucleated giant cells, findings consistent with granuloma multiforme.     

Reactions to Penicillamine: A Case of Cutis Laxa,Elastosis Perforans Serpiginosa and “Pseudo” Pseudoxanthoma

This patient was a 61‐year‐old white female who received several years of penicillamine therapy for the treatment of cystinuria. She subsequently developed penicillamine induced cutis laxa, elastosis perforans serpiginosa, and pseudoxanthoma elasticum like skin lesions. In addition, she suffered from numerous chronic bilateral lower extremity skin ulcerations. Her past medical history was also significant for end stage renal disease requiring hemodialysis and pulmonary fibrosis. She presented to the University of Miami Wound Care Center in 1/04 for treatment of her chronic ulcerations. On physical examination, the patient had multiple large hyperpigmented plaques with central ulcerations on her lower extremities. Some of the ulcers had overlying crust and others were covered with yellow fibrinous tissue. She also had generalized thickened, lax skin with multiple folds. On her neck, thighs, back and arms were violaceous, atrophic, serpiginous plaques with peripheral crusted erosions. A biopsy taken from the patients left thigh revealed dermal elastosis and the features of pseudo‐pseudoxanthoma. Two additional biopsies taken from the left thigh demonstrated elastosis perforans serpiginosa. This case highlights multiple skin manifestations of penicillamine therapy.     

Chronic actinic dermatitis

A 52-year-old man experienced a pruritic and erythematous eruption on his face, neck, upper chest, and arms. On examination his lesions consisted of edematous, erythematous plaques with scale and lichenified plaques. His clinical presentation, a skin biopsy specimen with a spongiotic dermatitis, positive patch and photopatch tests, and low MEDB and MEDA tests provided a diagnosis of chronic actinic dermatitis.     

Bullous localization of a lupus erythematosus induced by radiotherapy

INTRODUCTION: Connective diseases induced or exacerbated by radiotherapy are not frequent. We report a particular erosive erythematosus lupus with lesions in the precise distribution of radiation therapy given for a breast cancer. OBSERVATION: An 80 year-old woman presented with painful erosive skin lesions of her breast which had been treated by irradiation 9 years before. The patient also had rheumatoid arthritis. A skin biopsy showed keratinocyte necrosis, acantholysis and a dermal lymphocytic infiltrate under the basal cell layer. Direct immunofluorescence showed granular deposition of IgG, IgM and C3 along the basal cell layer. Antinuclear antibodies were positive at a titre of 1: 1,000. Erythematosus lupus diagnosis was established and annular lesions secondarily appeared on her neck, back, and arms. A treatment with hydroxychloroquine and topical corticostero?ds was effective in 3 months. DISCUSSION: The unusual erosive lesions and their localization on a previously irradiated site suggest the role of X-rays in our observation, despite their late appearance after radiotherapy. Radiotherapy and erythematosus lupus affect the same target structures (basal cells and small dermal capillaries) and could have additive effects. The presence of rheumatoid arthritis in the past medical history may have exaggerated this complication.