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Perianal fistula is a frequent complication of Crohn's disease. The diagnosis of the later may be difficult if cutaneous disease is dominant, and sometimes an important clinical challenge. The authors report a case of a 34 year old male complaining of painful perianal nodules for the three last years. Cutaneous histopathological examination was inespecific and colonoscopy normal. Pelvic CT scan and fistulography showed an intercommunicated fistular system invading both isquiorectal fossae, without intestinal communication. Repetition of colonoscopy showed normal mucosa, but in two of the biopsy samples noncaseous granulomas were found at the submucosa. The authors explain their main difficulties to reach the diagnosis of Crohn's disease and discuss the investigation and the evolution of the patient.

We reviewed all the cases of Squamous Cell Carcinoma in people under 40 years of age, diagnosed histologically at the Dermatopathology Laboratory of the Santa Maria Hospital, from January 1985 to December 1993. The 19 cases detected were analysed for age, sex, occupation, anatomical site of the tumor, evolution, smoke and alcoholic habits, skin phototypes, immunosuppression, previous viral infections, previous radiotherapy or PUVA therapy and associated diseases. A prevalence of an occupation involving more exposure to the sun was found. Males were more affected than females (2.2/1). In 37% of the patients we detected heavy alcoholic habits. The most frequent anatomical site was the lower lip (42%). Patients with skin phototype I were more affected (53%).

Amatus Lusitanus, of Jewish ancestry as most Portuguese physicians of his days, was born in 1511, in the town of Castelo Branco. He graduated as a physician at the University of Salamanca (1529) and practised in Portugal since then to the end of 1533, when he left to antwerp to escape the Inquisition. He acquired an high reputation as a doctor and botanist and was invited to a medical chair at the University of Ferrara (1541), which he left to go to Ancona (1547). He had to rush out of this city when the Inquisition was established there (1555). After a period at Pesaro and Ragusa he went to Thessalonica where he died of plague in 1568. He was acquainted and treated some of the more eminent personages of his time and published some remarkable medical works as the Index Disocorides, Commentaries on Discorides and the Centuries of Medical Cures which outlived him and were many times edited all over Europe. He referred largely to new diseases and medicines related to the Portuguese and Spanish Discoveries of his time, namely syphilis and drugs as guaiacum and Radix sinarum among others. However he never practiced on the new territorries of Africa, Asia and America as others Portuguese physicians did.

Fourteen cases (9 female, 5 male) of proliferating trichilemmal cysts are reported. The patients age-range was 40-80, 12 being over 60. The cysts wee present for 2-50 years and enlarged in the months or years before the observation. Seven were ulcerated. In 4 with multiple lesions both proliferating and ordinary trichilemmal cysts were present. The histological features were typical in all but one patient (evolution towards squamous cell carcinoma?) with some variation from lesion to lesion and from area to area in the same tumour. The microscopic features suggest that the proliferating trichilemmal cyst must be considered an hamartoma of the terminal hair follicle, not only related to the isthmic segment of the outer root sheath but with some differentiation (smaller and more variable) towards the infundibular and matricial segments. The tumour is likely to be a proliferating trichilemmal cyst from the beginning and not a peculiar evolution of an ordinary, pre-existant, trichilemmal cyst.

A 43 years old male patient with a pruritic, papular and papulovesicular eruption of 8 years duration is reported. The lesions come in crops (4-5 a year) of variable duration (4 days a 2 months) and are not influenced by treatment except, perhaps, systemic corticotherapy. Histologically there is focal acantholysis of limited extension but often involving the whole thickness of the stratum malpighi. The relationships between the persistent and the transient varieties of Greover's disease are discussed as well as the ones with cutaneous diseases in which focal acantholytic dyskeratosis has been observed.

The case of a 48 year old woman is reported with confluent nodules on the base of her nose and adjacent infraorbital areas, isolated ones on her face, and endotracheal masses with severe lumen reduction. Histologically the dermis and chorion of the nasal and tracheal mucosa were infiltrated by normal looking plasma cells. There was an increased ESR and a polyclonal gammapathy but no radiological evidence of skeletal involvement, bone marrow infiltration by atypical plasma cells or Bence Jones proteinuria. Cobalt-therapy was rapidly effective in reducing the endotracheal masses. The skin lesions were unaffected by roentgen-therapy and by several chemotherapeutic schedules. Laboratory values almost reverted to normal and her general condition has been satisfactory for the last two years. The differential diagnosis with multiple myeloma and extramedullary plasmocytoma was discussed and the condition classified as chronic, probably benign, mucocutaneous plasma cell infiltration.

A 20 year old male patient is reported who has, since the childhood, two adjacent, slightly depressed, orifices on the midline of the dorsum of the nose through which a few hairs came out. The histology of the excised lesion revealed a central cystic cavity, open to the surface, in which converge multiple mature hair follicles, either of vellus and terminal types. There are conspicuous, well developed sebaceous glands. No secondary or tertiary hair follicles could be found and hair shafts are often thick, long and pigmented. The differential diagnosis is discussed, particularly with the trichofolliculoma and the fistula of the dorsum of the nose. The lesion shares most of the features of this last lesion and probably is a superficial variety of fistula of the dorsum of the nose. The lesion is quite different from the classical type of trichofolliculoma and the name sebaceous trichofolliculoma is considered to be an inadequate one.

A 50 year old male patient is reported who had an asymptomatic nodule on the left pre-auricular region for about one year. The nodule, clinically diagnosed as an epidermoid cyst, was excised. Histologically it was an intradermal lesion composed of multiple keratinous cysts and solid epithelial nests and masses, malpighian in type. The lesion was imbedded in a well organized stroma, was separated from the overlying epidermis by a superficial dermal band and did not elicit any inflammatory response. No basaloid masses or strands with a lace-like arrangement nor attempts at hair formation could be seen. The differential diagnosis and nosological autonomy of trichoadenoma are discussed and the relevant literature is briefly reviewed.

One case of seborrhoeic keratosis with extensive acantholysis and moderate dyskeratosis is reported. The histological features ressembled those of Darier and Hailey-Hailey diseases. Another seborrhoeic wart simultaneously removed had no microscopic abnormalities. The dermatoses in which focal acantholytic dyskeratosis has been observed are reviewed and similarities and possible relationships to epidermolytic hyperkeratosis are discussed. The relative lack of specificity of these changes is emphasised as well as the occasional coexistence of both types and the variability of histological patterns observed on Grover's transient acantholytic dermatosis. These facts suggest a close relationship between the above mentioned histopathological patterns concerning the mechanisms of its production.

Fifteen patients with clinical and histological features of Crosti's reticulosis are reviewed. Twelve patients had lesions on the upper back and these were the only ones in eight of them. The other ones had extradorsal lesions. The histological picture was always compatible with Crosti's reticulosis. All the patients were treated by superficial roentgen therapy (1,200-1,500 rads) with total regression of the lesions. Four patients were lost for follow up. One progressed to lymphocytic lymphoma and another to Hodgkin's disease. It is possible that Crosti's reticulosis is not an autonomous entity, but a localized stage of more than one lympho-histiocytic neoplasia.

The case of a 21 years old female patient is reported who has had scaly lesions on the scalp since the age of 3, alopecia (later scarring alopecia) since the age of 5, and erythematous and squamous lesions on the trunk and limbs since the age of 14. More recently she developed grouped follicular papules on the trunk, back of the neck and upper limbs. Seven biopsies from the scalp, trunk and sole of foot were performed which showed typical psoriatic changes in all but one, from the scalp, where only scarring alopecia changes could be found. In one lesion from the trunk pseudogranulomatous features were observed in some papillae which were probably due to ectasia, tortuosity and endothelial proliferation of papillary blood vessels. The oral administration of RO 10-9359 was followed by a good remission of the lesions with exception of the scalp alopecia. The differential diagnosis, particularly with pityriasis rubra pilaris and lichen planus, is discussed and the similarities of our case with the Piccardi-Lassueur-Graham Little syndrome emphasised. If one accepts multiple diseases as the cause of the syndrome psoriasis could be admitted as one of them.

The case of a 60 year old woman with an umbilical nodule of sudden onset, followed one year later by cholestatic jaundice and weight loss, is reported. The histology of the nodule suggested a metastatic adenocarcinoma of undetermined origin. Laboratory and radiological investigations, abdominal echography and laparotomy disclosed carcinoma of the gallbladder with invasion of the main biliary duct and peritoneal dissemination. A biliodigestive derivation was found technically impossible at the surgical operation and the neoplastic disease far too advanced tu justify chemotherapy. Only eleven cases of biliary tract carcinoma with umbilical metastases could be found in the literature. The relative incidence of the primary tumors giving umbilical metastases, the different ways in which they can reach the umbilicus and the ominous meaning of these metastases are discussed and the relevant literature is reviewed.

Out of 210 cases of ulcerative colitis 41 had skin and mucosal lesions: skin lesions in 23 (11%), oral ones in 11 (5.2%) and perianal in 16 (7.6%). Cutaneous lesions were: maculopapular rashes (5.2%), erythema nodosum (2.9%), pyoderma gangrenosum (1.4%), papulopustular lesions, vasculitis and erythema multiforme (1% each). Aphthae were the commonest oral complication (4.3%) and fissures and fistula the more prevalent perianal ones (4.3% and 3.4%). More than one mucocutaneous complication occurred in 17.1% of the patients and in 18 patients there were associated articular (36.6%), ocular (9.8%) and hepatic (2.4%) changes. The colitic type of arthritis and keratoconjunctivitis sicca were the commoner joint and eye manifestations.

Dyskeratosis follicularis like changes are reported on a pigmented intradermal naevus (follicular infundibulum) from the nose of a 23 years old woman with no other stigmata of Darier's disease. These findings are discussed at the light of Ackerman's concept of focal acantholytic dyskeratosis and the relevant literature is reviewed.

A 17 years old male patient with acne vulgaris since the age of 13 is reported in whom a sudden exacerbation occurred with large, inflammatory, exquisitely tender, nodular lesions on the back and shoulders. Some were ulcerated and covered by hemorrhagic crusts. The lesions healed with large, unsightly, atrophic scars. With the cutaneous flare there was fever, arthralgia, loss of weight, leucocytosis and increased ESR. Systemic antibiotics (tetracycline, dicloxacillin, erythromycin) were unable to prevent or control the general and cutaneous symptoms that rapidly subsided on oral steroids. The initial relatively low dose could not be significantly reduced without further relapses. Attempts to replace prednisone by dapsone or clofazimine were unsuccessful. The relevant literature is reviewed and commented upon.

Out of 3,000 consecutive basal cell carcinoma 81 (2.7%) were located on the scalp of 77 patients (41 males, 36 females). The age at diagnosis was 50.8 +/- 14 years. Seventy five patients had a single lesion, one had 2 and another 4. Significant etiological factors were organoid nevi (11), syringocystadenoma papilliferum (1), androgenic alopecia (5), wound scar (1) and radiodermatitis (1). Out of 29 patients who answered a specific enquiry 10 admitted to having been treated by X-ray epilation for tinea capitis in childhood and 4 to have taken arsenic containing medicines. The evolution of the tumors varied from several months to more than 10 years. They were mostly nodular, pigmented and, less commonly, ulcerated with a pearly border, pendunculated or sclerosing in type. Histologically they were mainly solid or adenoid, with 2 sclerosing and 1 keratotic in type. In two cases there was underlying bone involvement. Treatment was by conventional surgery (54), Mohs' surgery (24) and cryotherapy (3). Four lesions recurred but the follow-up was below 5 years in most cases. We cannot confirm the impression of a more aggressive behaviour often suggested in the literature.

A 12-year-old diabetic girl is reported who developed faintly violaceous nodules on the back of the right thigh. These spread to the posterior aspect of the left thigh and to both legs. She complained of some muscular weakness. A diagnosis of cutaneous polyarteritis nodosa was considered. No blood eosinophilia was detected. Skin biopsy disclosed scattered dermal foci of eosinophilic and granular degeneration of collagen with a palisade of histiocytes and eosinophils in a flame configuration. The lesions did not correspond to the sites of insulin injections. Eight childhood cases of eosinophilic cellulitis were found in the literature, all of them in males. The specificity and meaning of the flame configuration are discussed.

Erythema nodosum (EN) has been reported in patients with ulcerative colitis (UC) since 1909. The reported incidence varies from 0.9 p. 100 to 18.9 p. 100. A wide range of incidence of EN has also been found in Crohn's disease (CD), with figures varying from 0.7 p. 100 to 8 p. 100 (fig. 1). Different criteria for the diagnosis of EN, UC and CD probably account for the scattering of values. A review of 324 cases of inflammatory bowel diseases revealed 21 cases with one or more episodes of EN: 6 out of 195 cases of UC (3.1 p. 100) and 15 out of 129 cases of CD (11.6 p. 100). The incidence was higher in females (5.7 p. 100 in UC, 17.5 p. 100 in CD) than in males (0 in UC, 6.9 p. 100 in CD). At the time of the eruption, patients with CD were younger (mean 24.3 years) then those with UC (mean 37.5 years). The interval between the onset of the intestinal symptoms and the nodular eruption was shorter in patients with CD (2.6 years) than in those with UC (8.2 years). The inflammatory bowel disease (whether UC or CD) started earlier in patients with EN than in EN-free patients. EN antedated the onset of intestinal manifestations in one patient with UC. In the remaining 20 patients the intestinal symptoms came first. EN was often recurrent, and the 21 patients suffered 32 episodes at varying intervals. One patient had 4 and another 3 episodes; six patients had 2 and 13 had one single episode. Three patients with UC had 2 episodes and 5 patients with CD had 2 or more episodes. The morphology and distribution of the lesions was fairly classical, except in 2 patients who had only 1 and 2 nodules respectively on one leg. The eruption subsided in all cases within 2 to 5 weeks, and no atrophy, suppuration or ulceration was observed. Most episodes of EN occurred during active phases of the intestinal disease. This was not so, however, in 2 cases in which the eruption followed an acute streptococcal throat infection. EN was more often found in total UC than in the less extensive distal and rectal types (Table I). It was also more frequent in the ileocolic form than in the exclusively ileal and colonic forms (Table II).(ABSTRACT TRUNCATED AT 400 WORDS)

A 54 year old woman with an asymptomatic small cutaneous horn on her chin for several years is reported. Histologically the lesion had the combined features of an inverted follicular keratosis on its upper part and a nodular intradermal trichilemmoma with central cystic degenerative changes underlying the inverted follicular keratosis exophytic one. The relationships between the two lesions is discussed as well as the main controversial histogenetic hypothesis.

A 52 year old housewife was vaccinated against smallpox at the age of 18, on her right deltoid area. At the age of 50 she noticed erythema and scaling on the vaccination scar and 2 years later a nodule appear that enlarged during the following 3 months. There was no history nor skin changes suggestive of significant sun exposure. The histological examination of an initial biopsy and of the subsequently excised lesion revealed a basal cell carcinoma of the solid type. The relevant literature was reviewed and discussed with emphasis on sex and age incidence, age and site of vaccination, free interval between inoculation and tumor appearance, coexistence or not of other sun induced neoplasias and precancerous lesions and other possibly relevant clinical and etiopathogenetic aspects.