Agustín Alomar

Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia.

Background: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon lesion clinically characterized by multiple papules usually located on the face and acral regions of elderly women. Histopathologically, MCAH is characterized by dermal vascular hyperplasia associated with increased number of factor XIIIa‐positive fibrohistiocytic cells and multinucleate cells with scalloped borders.

Abortive or minimal-growth hemangiomas: Immunohistochemical evidence that they represent true infantile hemangiomas

BACKGROUND Infantile hemangiomas have a characteristic natural history of rapid proliferation in the first weeks of life followed by spontaneous involution. At birth, they may be present as a precursor lesion. Sometimes one may see precursor lesions that never undergo a growth phase or that undergo minimal growth. It is unclear the exact nature of these precursor-like lesions. OBJECTIVE We sought to describe the morphology and histopathology of these precursor-like lesions. METHODS We describe 4 patients with macules resembling precursor lesions of hemangiomas that did not show proliferation phase or minimal growth. The histopathologic and immunohistochemical study with glucose transporter-1 was performed in all of these cases. RESULTS The skin biopsy specimen showed superficial ectatic vessels that reacted with anti-glucose transporter-1 antibodies. All skin biopsy specimens exhibited capillary lobules in papillary dermis and, in two of them, in the reticular dermis and subcutis. LIMITATIONS This text is limited by the number of cases reported. CONCLUSIONS Precursor lesions of hemangioma that do not show proliferation phase or minimal growth represent, in the view of glucose transporter-1 immunoreactivity, true hemangiomas of infancy with an aborted or arrested growth cycle.

Treatment of Psoriasis with Anti-TNF Drugs during Pregnancy: Case Report and Review of the Literature

Published experiences of TNF-α inhibition during pregnancy consist of a limited number of case reports, series and ongoing registry data in patients with arthritis and inflammatory bowel disease. A 28-year-old woman – with psoriasis vulgaris since she was 8 years of age and generalized pustular psoriasis during her first pregnancy (partially controlled with ciclosporin, oral prednisone and topical corticosteroids, when lupus anticoagulant was detected at another hospital) – presented 4 months after delivery with severe psoriasis (PASI = 15.4) that did not respond to ciclosporin (3 mg/kg/day). Ten days after the first infusion of infliximab (5 mg/kg), when the patient became aware that she was pregnant again, there was PASI75 response, and the patient wished to continue this treatment after being fully informed. Complete blanching was achieved by week 6 of treatment, and was maintained thereafter until the moment of writing (19 months after the start of treatment). She gave birth by caesarean delivery to a healthy female baby, who was breastfed for 1 month and has developed normally. The current report extends the available evidence on successful infliximab treatment in pregnant women, with the first case of a patient with psoriasis who presented impetigo herpetiformis during her previous pregnancy. No detectable adverse effects were detected in the neonate, despite potential exposure to infliximab throughout gestation and breastfeeding. Even though absolute safety is difficult to prove, available data suggest that women who become pregnant while taking infliximab or other anti-TNFα agents can be reassured regarding the continuation of pregnancy.

Diagnostic, prognostic and pathogenic value of the direct immunofluorescence test in cutaneous leukocytoclastic vasculitis

Background    No precise studies have been performed on cutaneous leukocytoclastic vasculitis (LV) to establish whether it is better to obtain a skin biopsy from lesional or from perilesional skin for direct immunofluorescence (DIF). There is no agreement on the immunoglobulins most frequently detected and the value of DIF for the classification of cutaneous vasculitis.

Carbamazepine-induced drug rashes: diagnostic value of patch tests depends on clinico-pathologic presentation

The campaign of manufacture of CFAP came to an end. The eczema later recurred each time he came in contact with MBF at work but never when on holiday. The patient had previously worked for several years in a plant producing chloro-benzenes and had had no skin problems during that period. Patch tests (Table 1) showed he was allergic to MBF but not to CFAP. 30 controls were patch tested with 0.005% MBF in methyl acetate and toluene and 0.005% CFAP in toluene, with negative results. The chemicals were known to be stable in these diluents, neither of which gave false positive reactions in controls.

Lichen striatus: clinical and epidemiological review of 23 cases

Lichen striatus (LS) is an asymptomatic self-limited skin disease of unknown etiology which was first described by Senear and Caro in 1941 [6]. Although LS is a frequent dermatosis among children, especially in girls between 5 and 15 years of age, there are few reviews in the literature. LS is characterized by erythematous or brownish papules with a flattened surface that are frequently scaly in appearance and occasionally display vesicles. The lesions are usually solitary and unilateral and have a linear distribution following Blaschko’s lines, usually on the extremities. Atypical forms with multiple and bilateral lesions have been described. Onset is usually sudden, with the disease progressing over days or weeks and slowly decreasing spontaneously until the papules resolve within 6–24 months, leaving a transitory residual hypopigmentation, especially in patients with a dark complexion. The inflammatory phase is not always detected, and hypopigmentation may be the first manifestation. The higher incidence during spring and summer, along with the existence of familiar clustering, suggest that viral infections could be an elicitation factor. Other possible precipitating factors may include cutaneous injury, trauma, hypersensitivity, or other as yet unspecified factors. We retrospectively reviewed the pediatric cases handled by the Department of Dermatology, Hospital de la Santa Creu I Sant Pau, in Barcelona between 1987 and 2004 using the photographic files of the Department as a selection criterion. Only patients with available clinical history were included. Diagnosis was based on clinical findings.

Enzyme‐linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis

Enzyme‐linked immunosorbent assay (ELISA) is an excellent tool for detection of circulating antibodies against the NC16A portion of BP180 antigen. We compared the sensitivity and specificity of a commercially available BP180‐NC16a domain ELISA with that of an indirect immunofluorescence (IIF) testing in the evaluation of bullous pemphigoid (BP) and pemphigoid gestationis (PG), and analyzed the relationship between ELISA results and the presence of IgG deposition, in an epidermal or combined pattern, on direct immunofluorescence (DIF) testing of salt‐split skin. ELISA was performed on serum from 28 patients (24 BP, 4 PG) and 50 controls. IIF testing was performed on serum from 27 patients and 98 controls. For the group of 28 patients with BP or PG, ELISA had a sensitivity of 93% and specificity of 96% (P < 0.001), while sensitivity was 74% and specificity 96% (P < 0.001) for IIF testing. In these patients, ELISA has a higher sensitivity than IIF testing, but similar specificity. Evaluation of controls who had IgG deposition on the dermal side of salt‐split skin on DIF testing showed specificity for the ELISA of 100% (all four cases negative) and 80% for IIF testing (one of five positive). Positive ELISA correlated with a diagnosis of BP or PG only in patients who had IgG at the basement membrane zone (BMZ) by DIF testing. Overall, ELISA appears to have greater sensitivity and specificity for BP or PG than does IIF testing.

Successful Treatment of Eccrine Angiomatous Hamartoma With Botulinum Toxin

A 12-year-old girl was referred to our department for evaluation of a 6-cm erythematous, brownish, indurated plaque that has been present on the sacral area since birth (Figure 1). The gluteal cleft was not deviated, and the lesion was not tender to palpation. The patient complained of profuse sweating that would drench her clothes. These symptoms were distressing and unrelated to emotional stress or physical exercise. A magnetic resonance imaging study revealed no lumbosacral spine abnormalities or cord tethering. A skin biopsy specimen demonstrated benign fibrovascular proliferation with a hyperplasia of the eccrine sweat glands in the dermis (Figure 2), which is consistent with an eccrine angiomatous hamartoma (EAH). The hamartomatous component was 2 mm below the epidermis and extended 4 mm into the dermis. In view of the symptoms, surgical excision was proposed, but the patient and her family preferred a different therapeutic approach.

Cutaneous Granulomatous Lesions in Common Variable Immunodeficiency: Complete Resolution after Intravenous Immunoglobulins

A 64-year-old man with common variable immunodeficiency developed a persistent papulonodular ulcerative eruption on the right leg. Histopathological examination disclosed a chronic inflammatory infiltrate with central necrosis and palisading granuloma. Repeated microbiological (bacteriological, mycological and mycobacteriological) studies failed to isolate any microorganism. After treatment with intravenous immunoglobulins, a progressive resolution of the skin lesions was observed with a complete clearing after 10 months. Clinicopathological features and therapeutic approaches of sterile granulomatous lesions associated with primary immunodeficiencies are reviewed.

Treatment of Acrodermatitis Continua of Hallopeau with TNF-Blocking Agents: Case Report and Review

Acrodermatitis continua of Hallopeau (ACH) is a rare acropustular eruption, characterized by sterile pustules, paronychia and atrophic skin changes, onychodystrophy and osteolysis of the distal phalanges of the fingers and toes. It is considered to be a variant of pustular psoriasis with a chronic relapsing course and frequent refractoriness to many therapeutic modalities, which can be amenable to successful treatment by tumor necrosis factor α antagonists. We report 1 patient with pustular psoriasis and ACH whom we have treated successfully with etanercept (for 30 months) and then adalimumab (for 13 months and ongoing). Blanching was initially achieved with etanercept 50 mg twice a week, but suppression of periungual inflammation then required combination therapy with etanercept 50 mg twice a week and methotrexate 10 mg weekly; lower doses of both drugs did not allow complete control of the disease. Eventually, adalimumab 40 mg every 2 weeks has provided the most cost-effective response in this patient, allowing maintenance of response with partial nail regrowth under monotherapy.