Acroqueratoelastoidosis de costa: presentación de un caso con lesiones en pabellón auricular / Coastal acrokeratoelastoidosis: presentation of a case with lesions in both auricular pavilions

La acroqueratoelastoidosis de Costa es una genodermatosis rara, caracterizada por la presencia de múltiples pápulas queratósicas, pequeñas y firmes, en los márgenes laterales de palmas y plantas. Es-tas lesiones estacionarias y asintomáticas aparecen generalmente en la pubertad. Se desconoce su prevalencia. Es de herencia autosómica dominante con expresividad variable. La histología combina hiperqueratosis y acantosis, sin embargo, el hallazgo más importante es la elastorrexis. No se aconseja tratamiento en la mayoría de los pacienes. Se reporta el caso de una paciente de 61 años, sin antecedentes familiares de esta condición, que consulta por aparición en la adolescencia de lesiones hiperqueratósicas asintomáticas en márgenes laterales de ambas manos y lesiones similares en ambos pabellones auriculares.

Acrokeratoelastoidosis is a rare genodermatosis, characterized by the presence of multiple kerato-tic papules, small and firm, on the lateral margins of palms and soles. These stationary and asymptomatic lesions usually appear at puberty. Its pre-valence is unknown. It is of autosomal dominant inheritance with variable expressivity. Histology combines hyperkeratosis and acanthosis, howe-ver, the most important finding is elastorhexis. Treatment is not advised in most patients. We report the case of a 61-year-old female patient, with no family history of this condition, who consulted for appearance in adolescence of as-ymptomatic hyperkeratotic lesions in the lateral margins of both hands and similar lesions in both auricular pavilions.

Unusual size, topography, and surgical resolution of an acquired fibrokeratoma

Abstract: Acquired fibrokeratoma is a rare benign skin tumor that usually manifests as a slow-growing solitary nodular lesion of the digits. We report a case of plantar acquired fibrokeratoma evidencing its atypical size and topography, in addition to the treatment with simple surgical excision followed by healing by second intention. Nondigital fibrokeratomas may occur in 18% of cases and rarely affect the palmoplantar region. These lesions are usually asymptomatic and show ≥ 3cm in size at the time of diagnosis due to delayed diagnosis. Complete surgical excision is the treatment of choice, which is usually curative. In general, primary closure is chosen; however healing by second intention becomes an option in regions of difficult approach.

Piccardi-Lassueur-Graham-Little syndrome associated with frontal fibrosing alopecia

Abstract: Piccardi-Lassueur-Graham-Little syndrome is a rare entity characterized by progressive scarring alopecia of the scalp and keratotic papules on hairless skin, associated with non-scarring alopecia in the axilla and pubic area or lichen planus lesions. We describe the case of a 70-year-old woman who presented a Piccardi-Lasseur-Graham-Little syndrome, along with frontal fibrosing alopecia.

Pellagra affecting a patient with Crohn's disease

Abstract: Pellagra is a nutritional disease caused by a deficiency of niacin. It may lead to death if not identified and treated timely. We review the literature and report a female patient presented with clinical features of pellagra as a complication of Crohn's disease.

"String of pearls pattern": report of three cases of non clear-cell acanthoma

Abstract The coiled and dotted vessels in a serpiginous arrangement or "string of pearls" is considered a classical vascular pattern associated with clear cell acanthoma. We present three cases of epidermal tumors different from clear cell acanthoma that have the same "string of pearls" vascular pattern. Even though most authors keep considering the "string of pearls" vascular pattern an almost pathognomonic sign of clear-cell acanthoma, the cases presented here suggest that some other epidermal tumors can also show this pattern.

Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.

Coexistence of nail lichen planus and lichen planus pigmentosus

Abstract We describe a fifty-six-year old, Afro-descendent female patient showing dystrophy of her twenty nails and hyperchromic, asymptomatic macule on her face. Histopathological examination of the macule showed vacuolization of the basal layer, melanophages in the superficial dermis and lymphoplasmocytic inflammatory infiltrate. Nail biopsy revealed orthokeratotic hyperkeratosis and lichenoid inflammatory infiltrate. Lichen planus pigmentosus is an uncommon variety of lichen planus. It is characterized by typical hyperpigmented macules on the face and neck. Nail changes might be present in 10% of lichen planus cases, but no associations with lichen planus pigmentosus have been described. We report a case of lichen planus in twenty nails associated with lichen planus pigmentosus on the patient's face.

Pitted keratolysis

Abstract Pitted keratolysis is a skin disorder that affects the stratum corneum of the plantar surface and is caused by Gram-positive bacteria. A 30-year-old male presented with small punched-out lesions on the plantar surface. A superficial shaving was carried out for scanning electron microscopy. Hypokeratosis was noted on the plantar skin and in the acrosyringium, where the normal elimination of corneocytes was not seen. At higher magnification (x 3,500) bacteria were easily found on the surface and the described transversal bacterial septation was observed.

Lichen planopilaris epidemiology: a retrospective study of 80 cases

AbstractBACKGROUND:Lichen planopilaris is a frequent presentation of primary cicatricial alopecia. Scalp distribution characterizes the main clinical presentations: classic lichen planopilaris, frontal fibrosing alopecia and Graham-Little Piccardi-Lassueur Syndrome (GLPLS).OBJECTIVE:Description of the clinical, dermoscopic and histopathological findings of Lichen planopilaris in public and private practices.METHOD:A retrospective observational study was performed by reviewing medical records of patients with lichen planopilaris.RESULTS:Eighty patients were included, 73 (91,25%) were female. Prototype II was seen in 53 (66,25%) patients. Classic lichen planopilaris was seen in 62,5% of the cases. Frontal fibrosing alopecia was seen in 31% of the patients and only one patient presented Graham-Little Piccardi-Lassueur Syndrome (GLPLS). Scalp lesions were scattered throughout the scalp in 47 (58,75%) of the patients, while 24 (30%) presented mainly central scalp lesions, 29 (36,25%) presented marginal lesions and only 4 (5%) patents had vertex lesions.CONCLUSIONS:Clinical presentation of Lichen planopilaris varies. To recognize the heterogeneity of the clinical appearance in lichen planopilaris is important for differential diagnosis.

Multiple minute digitate hyperkeratosis - a peculiar entity

Multiple minute digitate hyperkeratosis is a rare, non-follicular dermatosis, with fewer than 30 cases described worldwide. It can be either acquired or inherited in an autosomal dominant pattern. We describe the case of an 83-year old patient with life-long, multiple, digitate, milimetric lesions, and a positive family history for the same dermatosis.

Linear psoriasis: case report on three year old child

Atypical and unusual locations of psoriasis are very frequent. However, localized linear psoriasis is rare, with few cases described in the literature. It is characterized by a linear distribution of psoriasis lesions along Blaschko lines. We report the case of a three years old child, who presented unilateral erythematous scaly plaques arranged along Blaschko lines in the left hemithorax, with no associated symptoms and no lesions in other parts of the body. The differentiation of linear psoriasis from other linear dermatoses is not easy. The combination of a thorough history, a careful examination of the skin and histopathology are essential to ensure the correct diagnosis and appropriate treatment.

Case for diagnosis

Ostraceous psoriasis is a rare form of psoriasis, characterized by lesions with firmly adhered thick scales, in various colors, with surfaces resembling oysters shells. The protracted course of clinical presentation allied with peculiar lesions and histopathological examination permit the diagnosis. Lesions are usually resistant to topical medications, requiring systemic treatment. It is important that dermatologists are able to diagnose the unusual forms of psoriasis to avoid iatrogeny. We report the case of a patient with ostraceous psoriasis treated with methotrexate.

Syndrome in question

Vulvovaginal-gingival syndrome is characterized by erosions and desquamation of the vulva, vagina, and gingiva. We reported a case of a 32-year-old woman presenting with an 8-year history of damage to the vulval and perianal anatomy and limitation of mouth opening. The patient's symptoms were relieved after treatment with topical tacrolimus cream.

Exuberant clinical picture of Buschke-Fischer-Brauer palmoplantar keratoderma in bedridden patient

Buschke-Fisher-Brauer keratoderma is a rare hereditary autosomal dominant disease of incomplete penetrance. Important differential diagnoses include other palmoplantar keratinization disorders, acquired or hereditary, which is done based on the histopathological findings. This diagnosis alerts especially about the possibility of associated neoplasms. Treatment involves topical keratolytic agents, usually with little efficacy, or with long-term systemic retinoids with follow-up of exuberant collateral effects.

Trichostasis spinulosa of the scalp mimicking Alopecia Areata black dots

Alopecia areata is a common autoimmune disorder that leads to nonscarring hair loss. Black dots, also called comedo-like cadaver hairs, can be found in almost 50% of alopecia areata patients and indicate disease activity. Trichostasis spinulosa is a follicular disorder resulting from the retention of numerous hairs surrounded by a keratinous sheath in dilated follicles. Trichostasis spinulosa is a relatively common but underdiagnosed disorder of hair follicles. Here, we describe a man with alopecia areata of the eyebrows, androgenetic alopecia and trichostasis spinulosa at the vertex and show how dermoscopy can be useful in distinguishing black dots from Trichostasis spinulosa lesions.

Psoriasiform Keratosis - Case report

Psoriasiform Keratosis is a rare clinic entity. The etiopathogenesis remains unknown and the disease is characterized by a solitary, scaly or keratotic papule, or plaque mainly located on the extremities. Histopathological features closely resemble those of psoriasis. We report the case of a 70-year-old woman presenting a solitary and asymptomatic keratotic plaque, located on the back of the left leg, unresponsive to topical corticosteroids. We performed an excisional biopsy and histopathology was consistent with psoriasiform keratosis.