Isabel Colmenero

Deep morphea after vaccination in two young children.

Abstract:u2002 A number of dermatoses have been reported to appear in close temporal or spatial relationship to the intramuscular injection of vaccines. We describe two young children who had morphea consistent with the deep morphea type (morphea profunda) that appeared at the site of a previous vaccination. Trauma has been implicated as an important trigger of morphea, both in children and in adults. Furthermore, vaccines might induce an immune response, which could lead to morphea in predisposed individuals.

Nevus psiloliparus and aplasia cutis: a further possible example of didymosis.

Abstract:u2002 Nevus psiloliparus is a distinct type of mesodermal nevus of the scalp characterized by absence or paucity of hair, and presence of an excessive amount of fatty tissue. It is considered a hallmark of encephalocraniocutaneous lipomatosis, a rare disorder comprising a variety of cutaneous, ophthalmologic, and neurologic defects. We report two infants with encephalocraniocutaneous lipomatosis with nevus psiloliparus on the scalp in close association with aplasia cutis congenita. This unusual association may be considered a further example of didymosis, for which we propose the term, didymosis aplasticopsilolipara.

Pyoderma gangrenosum in an infant.

Abstract:u2002 Pyoderma gangrenosum is rare in children and very rare in infants less than 1u2003year of age. We report Pyoderma gangrenosum in a 6‐month‐old girl without any associated disorders, which was well controlled with oral prednisone. This entity in infants usually presents with multiple lesions, mainly located on the face, buttocks, thighs, and extremities, which in some instances are associated with pathergy. Pyoderma gangrenosum in infants has a good response to therapy, and healing is usually achieved.

Postvaccination Bullous Pemphigoid in Infancy: Report of Three New Cases and Literature Review

Bullous pemphigoid (BP) is an acquired autoimmune blistering disorder of unknown etiology uncommon in childhood. Unlike adult BP, infantile BP shows acral distribution and resolves rapidly with systemic steroids. We report three infants with infantile BP presenting shortly after vaccination for diphtheria, pertussis, tetanus, poliomyelitis, hepatitis B, Haemophilus influenzae B, and meningococcus C. Our cases further reinforce the causal association between childhood BP and vaccination.

Systemic Juvenile Xanthogranuloma with Fatal Outcome

Abstract:u2002 Juvenile xanthogranuloma is a benign and self‐limited disease which usually appears in the skin of children. Visceral involvement has been rarely reported, as has fatal outcome in some affected individuals. We report a case of systemic juvenile xanthogranuloma in a female newborn with mainly skin, bone marrow, and liver involvement, leading to death at the age of 2u2003months.

Epidermolysis bullosa simplex with mottled pigmentation: a family report and review.

Abstract:u2002 Epidermolysis bullosa simplex with mottled hyperpigmentation (EBS‐MP) is an uncommon subtype of EBS. Its clinical features depend on the age of diagnosis, and clinical variations have been described even within family members. We present six cases from two unrelated Spanish families each with several affected members with EBS‐MP and review the clinical and genetic findings in all reported patients. We highlight the changing clinical features of the disease throughout life.

Malignant atrophic papulosis: a case report with severe visual and neurological impairment.

Abstract:u2002 Malignant atrophic papulosis, or Degos’ disease, is a severe systemic vasculopathy extremely rare in children. The skin, gastrointestinal tract, and central nervous system are most frequently affected. We report a 5‐year‐old girl with malignant atrophic papulosis who presented with widespread skin lesions from early infancy and severe visual and neurological impairment.

Papular Epidermal Nevus with “Skyline” Basal Cell Layer (PENS) with Extracutaneous Findings

Abstract:u2002 Papular epidermal nevus with “skyline” basal cell layer (PENS), a variant of epidermal nevus, was recently described in otherwise normal children. We describe herein a patient with multiple, typical PENS lesions associated with peculiar facies, bilateral Achilles tendon shortening, and mild psychomotor delay. The association of PENS with extracutaneous manifestations suggests the possibility of a new type of epidermal nevus syndrome, for which we propose the term PENS syndrome.

Persistent Agmination of Lymphomatoid Papulosis

Abstract:u2003 A 12‐year‐old boy had lesions clinically and histopathologically consistent with lymphomatoid papulosis (LP) which were persistent and appeared grouped in a circumscribed area of normal looking background skin on the right abdomen. Staging work‐up did not disclose systemic disease. Persistent agmination of lymphomatoid papulosis (PALP) is somewhat different from classical LP because it involves a circumscribed, patch‐sized area of the skin and the papules within never resolve completely despite some waxing and waning. PALP might be considered a localized or regional form of LP or a distinct lymphoproliferative disorder; in any case, at least a cautious long‐term follow up is recommended, with attention to the possible development of true lymphoma.

Multiple Lichenoid Juvenile Xanthogranuloma

Abstract:u2003 A 2‐year‐old boy had hundreds of discrete, small, flat yellow to brown colored papules distributed over the face, neck, and trunk. Biopsy revealed a well‐demarcated infiltrate within the papillary dermis composed of histiocytes and some Touton giant cells and eosinophils. The cells were negative for protein S‐100 and CD1a but positive for CD68 staining. No systemic lesions of juvenile xanthogranuloma were identified. Lichenoid juvenile xanthogranuloma is a very infrequent variant of juvenile xanthogranuloma.