J.M. de Moragas

Pityriasis lichenoides in children : Clinicopathologic review of 22 patients

Abstract: Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with an autoinvolutive course, that can occur in pediatric patients. Traditionally, acute and chronic variants have been described, but other special forms of presentation have been reported. We reviewed the clinical records and histopathologic specimens of all pediatric patients diagnosed with PL in our hospital from 1980 to 1995 to assess the clinicopathologic features of this disorder in our environment. Twenty‐two of the 118 cases reviewed were pediatric patients less than 15 years old (12 males and 10 females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with a mean of 9.3 years. Most of the patients (72%) had the chronic variant of the disease, while the remainder had an acute course. One patient suffered from acute ulceronecrotic PL. Systemic treatments prescribed were erythromycin in eight patients, PUVA in five patients, and methotrexate in one patient. Three patients had a prolonged course with more than two episodes. Acute and chronic PL are polar extremes, but individual cases cannot be classified only on the basis of histopathologic data, since coexistence of lesions in different stages of evolution can lead to sampling bias. Acute ulceronecrotic forms and the presence of a variable degree of cellular atypia in the infiltrate are liable to cause differential diagnostic problems with lymphomatoid papulosis (LP), which cannot be completely resolved on the basis of T‐cell receptor clonal rearrangement detection.

Detection of Mycobacterium tuberculosis complex DNA by the polymerase chain reaction for rapid diagnosis of cutaneous tuberculosis

Summary We assessed the polymerase chain reaction (PCR) technique to detect Mycobacterium tuberculosis complex DNA In 48 paraffin‐embedded specimens from 32 patients with different variants of cuttineous tuberculosis, and compared the resuts with those of culture. A 123 bp product of the 1S6110 insertion sequence specific of M. tuberculosis complex was amplified and confirmed by digestion with Sall restriction endonuclease. The time required for the procedure was 3 days. Thirty‐seven samples (77.1%) were positive for M. tuberculosis complex DNA. No false positive results were obtained in nine negative controls, Of the 20 specimens tested by PCR and culture, the frequency of positivity was 90% for DNA amplification and 65% for culture. In seven cases of lupus vulgaris, the figures were 100% and 57% respectivety. In the 11 specimens culture negative or not microbiologically tesled and PCR negative, evidence for tuberculous infection was provided hy the correlation of various relative fmd absolute criteria. These results show that PCR amplification of the IS6110 insertion fragment is a rapid and accurate means for the detection of M. tuberculosis complex DNA in paraffin‐embedded skin biopsies from patients with cutaneous tuberculosis, especially in paucibacillary lesions.

Generalized acquired cutis laxa associated with coeliac disease: evidence of immunoglobulin A deposits on the dermal elastic fibres

Summary Acquired cutis laxa (ACL) is an uncommon elastolytic disorder of unknown aetiology. In rare instances. ACL has been reported in association with autoimmune diseases and dermal deposit of immunoglobulins, suggesting that destruction of elastic tissue may be immunologically mediated. We report a 35‐year‐old man with generalized acquired cutis laxa (GACL) associated with a persistent papular erythematous eruption that histopathologically showed some resemblance to dermatitis herpetiformis. A marked reduction and degeneration of dermal elastic fibres was noted in biopsies from loose‐hanging skin. Direct immunofluorescence from non‐inflammatory loose skin revealed granular immunoglobulin A (IgA) deposits at the basement membrane zone and fibrillar IgA deposits in the dermal papillae. IgA deposits were also observed on the elastic fibres of the reticular dermis. Electron microscopy of skin from the submammary fold revealed fragmented elastic fibres, partial absence of peripheral microfibrils and abundant neutrophils, some of which were degranulated and adjacent to elastic fibres. Immunoelectron microscopy of an erythematous papule revealed IgA deposits around dermal elastic fibres. Antigliadin, antireticulin and antiendomysium antibodies were present. Jejunal biopsies showed a gluten‐sensitive enteropathy. A possible IgA‐mediated immune mechanism for the development of GACL in our patient is suggested.

Carpal Tunnel Syndrome Presenting as Sclerodactylia, Nail Dystrophy and Acro-Osteolysis in a 60-Year-Old Woman

Skin changes associated with carpal tunnel syndrome have rarely been reported. A 60-year-old patient was referred to our Department of Dermatology for evaluation of an aseptic gangrene of the distal phalanx of the third finger of her left hand, progressive nail dystrophy, sclerodactylia and spontaneous ulcerations. These features were seen on the digits with sensory supply from the median nerve. The patient had hand paresthesias for at least 10 years. No abnormal results were found in biochemical, hematological or immunological determinations. An echo-Doppler study of the upper limbs was normal. An electromyographic study was consistent with a bilateral carpal tunnel syndrome (CTS). Ulcerative and mutilating CTS can occasionally produce sclerodactylia with ulcerative lesions on distal fingers, nail dystrophy and acro-osteolysis as a consequence of mechanical compression of the autonomic fibers of the median nerve. The clinical picture includes Raynauds phenomenon in some patients. Correct diagnosis is important, since ulcerative and mutilating CTS is a marker of a severe neural lesion, which is amenable to treatment by surgical decompression.

Multiple Fibrofolliculomas and Trichodiscomas

A late-onset, sporadic case of multiple fibrofolliculomas and trichodiscomas is reported. This case and other reports in the literature suggest to the authors that benign neoplasms derived from the mesodermal component of the pilar apparatus may present in various combinations and may also be associated with other hamartomatous skin proliferations. The wide spectrum of published combinations may be due to similarities in clinical presentation and biopsy-sampling uncertainties.

Kaposi’s Sarcoma in a Patient with Erythroblastopenia and Thymoma: Reactivation after Topical Corticosteroids

We report a 69-year-old female with erythroblastopenia and thymoma who developed lesions of Kaposi’s sarcoma (KS) after thymectomy, 2 months after the initiation of therapy with methylprednisolone. Control of mucocutaneous KS lesions was obtained with radiotherapy, interferon alfa-2b and withdrawal of systemic immunosuppressive therapy. Erosive oral lichen planus appeared later, and after therapy with topical corticosteroids a new lesion of KS developed that regressed after withdrawal of topical corticosteroids. The detection of HHV-8 only in lesional skin supports the hypothesis that this virus can trigger the development of KS lesions.

Disseminated Kaposi's sarcoma not associated with HIV infection in a bisexual man.

We report a 42-year-old white bisexual man with disseminated Kaposis sarcoma limited to the skin and gastrointestinal tract. Results of several serum tests for human immunodeficiency virus (HIV) antibodies have been negative. The CD4/CD8 ratio has remained normal, and his Kaposis sarcoma has had a benign clinical course during 30 months of follow-up. Similar reports of disseminated Kaposis sarcoma with a benign clinical course in homosexual or bisexual men without demonstrable HIV infection are reviewed. Some cellular immune impairment that might be more prevalent in the homosexual population may be implicated in the pathogenesis of this type of Kaposis sarcoma.

Malignant Hidroacanthoma simplex

2 cases of malignant hidroacanthoma simplex are reported. Prominent ultrastructural findings were sparse tonofilaments and villous-like, interdigitating cytoplasmic processes connected by small desmos

Treatment of Meralgia paraesthetica with Topical Capsaicin

Lluis Puig, Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Avda. San Antonio M. Claret 167, E-08025 Barcelona (Spain) Meralgia paraesthetica is an uncommon neurologic disorder usually caused by compression of the lateral femoral cutaneous nerve. This nerve leaves the pelvic cavity at the anterosuperior iliac spine below the inguinal ligament and superficial to the sar-torius muscle. In its course it is vulnerable to entrapment, generally related with obesity, pregnancy or unknown causes [1]. Meralgia paraesthetica has also been described to appear as a result of neuromas [2], malignant tumours of the psoas muscle [3], metastatic carcinomas in the second lumbar vertebra [4] or following coronary bypass surgery [5], due to compression arising from prolonged supine position on the operating table. Meralgia paraesthetica is clinically characterized by a disturbed sensation at the an-terolateral side of the thigh. Patients can complain of pain, numbness, itching ordysaesthe-sia, and the perception of pinprick and touch is often diminished or lost. In some cases they Lluís Puig Department of Dermatology Hospital de la Santa Creu i Sant Pau Avda. San Antonio M. Claret 167 E-08025 Barcelona (Spain) refer a burning pain that can be unbearable. The femoral cutaneous nerve is only sensitive so the motor function remains uninvolved and the reflexes are normal. Electrophysio-logic tests and somatosensory evoked potentials can be useful as diagnostic procedures [6]. Many treatments have been advocated for meralgia paraesthetica [2]. Analgesia can be provided by nerve block or local infiltrations. Surgical procedures include neurolysis, transposition or decompression of the nerve and mobilization of the supra-inguinal ligament. The possible efficacy of topical capsaicin in the treatment of meralgia paraesthetica has not been previously reported, to our knowledge. A 54-year-old man was referred for evaluation of dysaesthesia in the anterolat-eral right thigh. His medical history included partial epileptic crisis due to a congenital arachnoid cyst in the left parietotemporo-occipital area which had been treated with carbamazepine for the last 3 years. He also suffered from aquagenic pruritus which was well controlled with the administration of hydroxyzine before showers. He complained of paraesthesias, numbness, itching and burning sensations localized on the anterolateral aspect of the right thigh of 2 months’ evolution. Physical examination did not reveal any cutaneous abnormality, and the results of complete blood cell counts and biochemical parameters were normal. The sensory response of both femorocutaneous nerves could not be evocated on electrophys-iological examination. The patient was instructed to apply topical 0.025% capsaicin cream five times daily on the lateral aspect of

Cheilitis granulomatosa Treated with Metronidazole

Cheilitis graunulomatosa (CG) is a chronic inflammatory disorder of unknown etiology. We report the case of a patient with CG who has successfully been treated with metronidazole.