Lichen planus pemphigoides presenting with a strikingly unilateral distribution

A 44-year-old Pakistani woman presented with a unilateral eruption of 2 months’ duration. She was in her usual state of health when she awoke one morning with an acute blistering eruption in the right axilla. At that time she also began to experience “rough spots” on the buccal mucosa. Approximately 4 days later, lesions, including small blisters, appeared at multiple other sites, all in a right-sided distribution. The patient had an 8-year history of hypothyroidism and was treated with levothyroxine. Physical examination revealed a widely distributed and strikingly right-sided eruption. Dark brown macules and patches measuring 0.1–1.2 cm, some of which were confluent, were located in the right axillary area at the site of previous blister formation ( Fig. 1a ). Close inspection revealed that some of these lesions had a glistening and violaceous appearance at their periphery. Discrete, small, flat-topped papules with similar qualities were also present at this location. Lichenoid lesions were evident in multiple other

Figure 1 Open in figure viewer PowerPoint (a) Hyperpigmented macules and patches in the right axillary region. Some of these lesions had a glistening, violaceous, and raised quality at the periphery (white arrow) suggesting post-inflammatory hyperpigmentation secondary to lichen planus. (b) Lichenoid lesions in the intergluteal area and the right buttock     

T-cell chronic lymphocytic leukemia mimicking dermatomyositis

A 57‐year‐old man was diagnosed with chronic lymphocytic leukemia (CLL) during routine hematologic evaluation. Two months later, the patient developed a mildly pruritic periorbital eruption and erythematous plaques in a photosensitive distribution. A skin biopsy at an outside institution showed nonspecific histopathologic changes. The patient was diagnosed with amyopathic dermatomyositis and treated with prednisone 1 mg/kg/day with no improvement. The patient was subsequently admitted to our institution for CLL chemotherapy. Physical examination on admission revealed bilateral, violaceous, nonblanching periorbital plaques ( Fig. 1A )and symmetric erythematous plaques with mottled hypo‐ and hyperpigmentation on the shoulders, proximal arms ( Fig. 1B ), and upper chest ( Fig. 1C ). Periungual erythema was noted. Muscle strength was normal. Laboratory examination was significant for a white cell count of 33,700/mm³ with 78% lymphocytes, a hematocrit of 25.8%, and a platelet count of 25,000/mm^3. The lymphocytes were almost exclusively CD4 T cells expressing CD2, CD3, CD5, and CD7. Electrolytes, liver function tests, creatine kinase, aldolase, antinuclear antibodies, and anti‐Jo antibodies were normal or negative. Several biopsies of the plaques on the arms were obtained. Histopathology revealed leukemic infiltration of the skin with monoclonal T lymphocytes positive for CD2, CD3, CD4, CD5, and CD7 ( Fig. 2 ). Steroids were discontinued. The patient died a week later from toxic epidermal necrolysis secondary to allopurinol.

Figure 1 Open in figure viewer PowerPoint Clinical findings included periorbital violaceous plaques mimicking a heliotrope eruption (a) and poikilodermatous‐appearing plaques on the lateral arms (b) and upper chest (c)     

Bullous variant of Sweet's syndrome

A 69-year-old woman presented to our clinic as an emergency with erythematous, well-circumscribed plaques, which were partly vesicular, on her extremities and in her armpits, and additionally hemorrhagic blisters on both her palms and her fingers ( Fig. 1a ), which had developed 2 days after the first appearance of the skin lesions. The rapid onset of the lesions (within a few hours) and the pain associated with them were extremely troublesome to the patient. On admission she complained of fever, tiredness and being easily fatigued. Because of a urinary tract infection 1 month prior to admission, trospiumchloride was given. On clinical examination, body temperature was found to be above 38 °C and infraclavicular lymph nodes were enlarged but not tender.

Figure 1 Open in figure viewer PowerPoint (a) Bullae on the patient's right hand. (b) Multiple partly confluent vesicles with neutrophilic granulocytes intraepidermally and a dense interstitial perivascular infiltration of neutrophilic granulocytes and lymphomononuclear cells (H&E, ×200)     

Carcinoma erysipeloides as a presenting feature of breast carcinoma.

Drug name tamoxifen: Nolvadex An 80-year-old caucasian woman was seen in November 1997 for an asymptomatic raised lesion on the left side of the neck extending to the infraclavicular area of 7 months’ duration. She had been treated with topical steroids by her general practitioner with no relief. On examination, there was a large, irregular, ill-defined, indurated, erythematous plaque, 7 × 4 cm, over the left side of the neck extending to the infraclavicular area ( Fig. 1 ). The diagnoses entertained included tinea incognito, cutaneous mucinoses, and carcinoma erysipeloides.

Figure 1 Open in figure viewer PowerPoint Infiltrated plaque of carcinoma erysipeloides on the left side of the neck     

Ecthyma gangrenosum.

Case 1. An 18-year-old white man with AML-M3 received allogeneic bone marrow transplantation (BMT) in August 1997. On the seventh day of BMT, he developed chills, fever (39.1 °C), anorexia, and perirectal pain. On physical examination, a hemorrhagic bulla and “punched out” ulceration were observed on the perirectal region. Subsequently, the ulcer was surrounded by a red, swollen, tender, cellulitic plaque ( Fig. 1 ).

Figure 1 Open in figure viewer PowerPoint The ulceration on the perianal region of the first patient     

Sympathectomy-induced ichthyosis-like eruption

An 80‐year‐old African–American man, with a past medical history of childhood rheumatic fever, hypertension, coronary artery disease, unstable angina, and asbestosis, underwent cardiac catheterization 37 years ago for unstable angina. The postcatheterization course was complicated by a right brachial arterial thrombosis. A venous brachial bypass graft was placed, with vascular supply to the affected arm restored. Postbypass, the patient recovered full use of the arm, but suffered from persistent arm pain. Surgical sympathectomy was performed 1 year later. The patient has been pain‐free since, but noted soon after sympathectomy, unilateral scaly dry skin, unilateral hypohidrosis of the axilla, and increased temperature sensitivity on the affected limb. In the 37 subsequent years, despite application of emollients, he reported no significant improvements or changes in the skin findings. The patient denied that other family members suffered from similar skin problems. He denied ever having atopy or other skin problems prior to the current episode. On physical examination, dry plate‐like scales were seen on the dorsum of the right hand ( Fig. 1A,B ), right shoulder, and upper back, extending to the midline ( Fig. 1C ), in an area of distribution supplied by the injured nerves. Mild follicular prominence of the affected extensor arm was also seen as compared with the uninvolved arm (not shown).

Figure 1 Open in figure viewer PowerPoint Patient with fish‐like fine scales on the right hand (a) which are absent on the left hand. (b) Close‐up view of the fish‐like scales on the affected hand. (c) Posterior trunk and shoulder demonstrating ichthyosiform change extending to the midline     

Barraquer-Simons syndrome with benign infundibulocystic proliferation

A white man attended our outpatient clinic because of soft papules on the second finger of the left hand. They were livid red‐colored, warty surfaced cysts, 1–2 cm in diameter, arranged in an annular configuration ( Fig. 1 ). The patient showed prominent zygomatic bones and symmetric loss of buccal fat pads (Bichat's fats), leading to sunken cheeks ( Fig. 2 ). The history of the patient could not be taken because of his debility.

Figure 1 Open in figure viewer PowerPoint Soft papules on the second finger of the left hand     

Three cases of cutaneous sparganosis.

Case 1 A 64‐year‐old man visited our clinic complaining of an asymptomatic nodule on his right thigh, detected 10 days previously. Physical examination revealed a solitary subcutaneous nodule, 1.5 cm in diameter on his right thigh. Twenty years previously the patient had undergone surgery to remove a ‘‘worm’' from his leg. The patient stated that he enjoyed eating raw snakes. On performing excisional biopsy of the lesion, the white, flat, shiny sparganum was detected and removed ( Fig. la ). The histopathological study of the biopsy specimen showed several encapsulated, mummified sparganum pieces and dense lymphohistiocytic infiltration along with neutrophils and eosinophils ( Fig. lb) .

Figure 1 Open in figure viewer PowerPoint (a) A flat, band‐like, white glistening sparganum being removed from the right thigh of case 1. (b) An encapsulated, mummified sparganum piece showing degenerated internal organs (hematoxylin and eosin × 40)     

A case of basal cell carcinoma arising in epidermal nevus

A 24‐year‐old man was admitted to our outpatient clinic with lesions in a linear configuration. On dermatologic examination, widespread, dark brown, warty papules and plaques over an erythematous base, following Blaschko"s lines, extended from the middle of the chest to the right arm. These lesions had been present on the chest since birth and had gradually extended during childhood. Recently, a nodular lesion had appeared in the pre‐existing epidermal nevus in the middle part of the chest ( Fig. 1 ). The nodular lesion was totally excised by a plastic surgeon.

Figure 1 Open in figure viewer PowerPoint Basal cell carcinoma (white arrow) appearing on the epidermal nevus in the middle part of the chest     

Case of generalized lichen amyloidosis

A 23-year-old white female patient presented with a 3-year history of a pruritic rash on the trunk, extensor surfaces of the extremities, back, abdomen, and the glutea. There was no family history of any similar dermatological disease. Dermatological examination revealed generalized rough, mildly keratotic, symmetrical dome-shaped papules, involving dorsal and extensor surfaces of both the lower and upper extremities, abdomen, back and the glutea ( Fig. 1 ). Physical examination was normal.

Figure 1 Open in figure viewer PowerPoint Dome-shaped, lichenified papules, 4–5 mm in diameter, on the right lower extremity     

Marginal acrokeratoderma and psoriasis: is there an association?

Case 1 A 70‐year‐old housewife presented to us with multiple, asymptomatic, depressed, crateriform plaque lesions over the radial margin of the index finger and thumb and thenar and hypothenar eminences of both hands of 25 years' duration ( Fig. 1 ). There was no history of prolonged sun exposure or repeated trauma to the hands and there were no such lesions on the soles. None of her other family members were affected. We made a provisional diagnosis of keratoelastoidosis marginalis. Skin biopsy revealed an unremarkable epidermis, with minimal lymphocytic infiltration in the upper dermis and hyalinization in the reticular dermis, extending to the deeper dermis. Two years later she returned with well‐defined, erythematous, scaly, mildly itchy plaque lesions over the instep of both feet and the center of both palms. A clinical diagnosis of palmoplantar psoriasis was made.

Figure 1 Open in figure viewer PowerPoint Case 1. Crateriform plaque lesions on the hands. Note the palmoplantar psoriasis     

Bilateral segmental neurofibromatosis: a case report and review

A 65‐year‐old white woman presented with complaints of ‘‘moles’' on the left side of her trunk present for an undetermined period of time. She denied associated pain and pruritus. Her past medical history was significant for hypothyroidism and depression, for which she was taking levothyroxine and venlafaxine, respectively. In addition, she was on hormone replacement therapy. Reportedly, no other family members were affected by similar skin lesions. Physical examination revealed soft, dome‐shaped, skin‐colored papules grouped in a T11–12 dermatomal distribution on her trunk bilaterally. The lesions were more numerous and densely grouped on the right side of her trunk ( Figs 1 and 2 ). No axillary freckling or café‐au‐lait macules were noted. No Lisch nodules were observed upon ophthalmologic examination.

Figure 1 Open in figure viewer PowerPoint Dermatomal distribution of neurofibromas on the right flank     

Cutaneous sinus tract of dental origin.

A 47‐year‐old woman presented with a nodular lesion on her face that had been discharging pus intermittently for 7 months. She could not recall any previous trauma to the site. Dermatologic examination revealed a nontender, erythematous, nodular, draining lesion on the patient's chin ( Fig. 1 ). Dental examination showed poor oral hygiene and severe chronic periodontitis, but no acute infection. Radiography demonstrated diffuse periapical radiolucency associated with the right central mandibular incisor ( Fig. 2 ). The patient was diagnosed with chronic apical periodontitis. Because it was too late for her oral condition to be corrected with endodontic treatment, the tooth was extracted and the scar on her chin was revised later.

Figure 1 Open in figure viewer PowerPoint A cutaneous draining sinus of dental origin presenting as an erythematous nodule on the patient's chin     

Prolidase deficiency

A 32‐year‐old mentally retarded woman was admitted to hospital with recurrent ulcers on her legs, which appeared for the first time at 8 years of age. Apart from recurrent lower respiratory tract infections and chronic otitis media, her personal history was unremarkable. Her parents were second‐degree relatives. The family history showed no similar disease or mental retardation. Physical examination revealed that the patient had an unusual facial appearance, with a high‐arched palate; she had multiple tooth caries ( Fig. 1 ).

Figure Figure 1  Open in figure viewer PowerPoint Facial appearance of the patient     

Alopecia: a rare manifestation of lupus vulgaris

A 14‐year‐old girl presented with a 2‐year history of gradually developing, asymptomatic, reddish, raised lesions over the scalp, face, arms and trunk. The family history was significant for tuberculosis. Her grandmother was found to have pulmonary tuberculosis. Clinical examination revealed reddish‐brown, soft papules forming two large plaques by coalescence over the scalp and back ( Figs 1 and 2 ).

Figure 1 Open in figure viewer PowerPoint Lupus vulgaris, plaque form manifesting as alopecia     

Waardenburg syndrome: case report

A 15-year-old Arab boy presented with congenital deafness, heterochromia iridis (right, brown; left, blue), poliosis (white forelock), with an area of leukoderma beneath the forelock, broad nasal root, and lateral displacement of the medial canthi ( Fig. 1 ). Further investigation revealed that one of his siblings was similarly affected. The child was diagnosed with Waardenburg syndrome.

Figure 1 Open in figure viewer PowerPoint Waardenburg syndrome: white forelock, heterochromia iridis, broad nasal root, leukoderma and hypertrichosis of medial eyebrows     

Systemic cryptococcosis with solitary cutaneous lesion in an immunocompetent patient

A 55-year-old white farmer from north-east Brazil presented with a 1-month history of headache and a solitary nodular lesion on his face. He was a smoker and had reported contact with pigeons. He did not complain of any other systemic symptom such as cough or pain. Physical examination of the skin lesion revealed a nodule measuring approximately 3 cm in diameter with central ulceration and overlying crust ( Fig. 1 ). The lesion clinically resembled a keratoacanthoma.

Figure 1 Open in figure viewer PowerPoint Nodular, single, keratoacanthoma-like lesion with a necrotic crust on the face     

Cutaneous and neurologic manifestations of biotinidase deficiency

A male newborn with no obstetric or familial antecedents, except that his parents were cousins, developed hypotonia, lethargy, and feeding problems from birth. Analysis revealed a marked metabolic acidosis and hyperammonemia. Three weeks later, he was admitted to hospital in order to receive parenteral nutrition and to undertake a study for metabolic diseases. The boy did not improve in spite of the use of parenteral nutrition and began to present with inspiratory stridor and tachypnea. One week later, he presented with an erythematous scaling eruption, which was especially intense in the lumbosacral region ( Fig. 1a,b ).The scalp was only slightly affected.

Figure 1 Open in figure viewer PowerPoint Erythematous scaling eruption, more intense in the lumbosacral region     

Bluish patches on the ears and axillae with dark urine: ochronosis and alkaptonuria

A 45‐year‐old white woman presented with a 3‐year history of bluish discoloration of her ears ( Fig. 1 ). The patient had noted a bluish‐black staining of the armpit areas of her clothing for the prior 6 months. A review of systems and past medical history were significant for a history of constant right lower back pain and bilateral knee pain for approximately 1 year. A review of medications revealed daily use of conjugated estrogens and multivitamins and intermittent use of ibuprofen. A review of her family history revealed that a sister was affected with the same disorder, but the spouse and children were not. The patient's parents were not known to be consanguineous.

Figure 1 Open in figure viewer PowerPoint Bluish patches are present on the ear overlying the cartilage     

Lichen planus pemphigoides: report of two cases

Case 1 A 73‐year‐old woman with a 5‐month history of widespread lichenoid eruption developed disseminated blisters. She had insulin‐dependent diabetes mellitus and renal tuberculosis treated with isoniazide (300 mg/day), rifampin (600 mg/day), ethambutol (1.5 g/day) and pyrazinamide (2 g/day) following right nephrectomy. Antituberculous therapy which had been begun 2 months before the onset of lichenoid eruption was stopped by the patient following the appearence of blisters. Examination revealed lichenoid plaques on the face, neck, trunk and extremities and tense bullae on the neck and extremities ( Fig. 1a ). The bullae were either on lichenoid plaques or normal appearing skin. Some of the blisters were hemorrhagic. Two skin biopsies were performed. The first biopsy from a blister with a lichenoid base on the arm, showed typical changes of lichen planus (LP) with subepidermal cleft formation. The second biopsy from a blister on normal appearing skin on the trunk showed subepidermal bulla with perivascular inflammatory cell infiltrate of eosinophils and lymphocytes. Direct immunofluorescence (DIF) of perilesional skin showed linear deposition of IgG and C3 along basal membrane zone (BMZ). Direct immunofluorescence on salt‐split skin revealed epidermal binding of immunoreactants. Indirect immunofluorescence showed a circulating IgG autoantibody binding to the BMZ at a titre of 1/200. Immunoblot analysis of her serum recognized minor 180 Kd BP antigen. An initial trial of dapsone (100 mg/day) for 2 weeks was not effective. She was then treated with oral methylprednisolone (32 mg/day), which induced rapid improvement in her skin lesions ( Fig. 1b ). After 2 months of treatment, corticosteroid was stopped and the patient stayed in remission for 1 year till she died due to myocard infarction.

Figure 1 Open in figure viewer PowerPoint (a) Lichenoid papules and hemorrhagic blisters on the face and the neck. (b) After 2 months of oral methylprednisolone therapy